I have my doubts between 1 and 2; exceptionally C. botulinum can infect traumatic or puncture wounds in IVDUs using brown heroin or cause sinusitis in cocaine addicts. On the other hand, the case may refer to a severe sepsis with poor initial manifestation at first sight, with incipient rhabdomyolysis and muscle pain, in this case it may be a necrotizing fasciitis, where the most frequently implicated germ is S. pyogenes.

A 46-year-old male parenteral drug user came to the emergency department with fever with chills, mental confusion, diffuse myalgias and intense pain in the left hand of 24 hours of evolution without clear local phlogosis signs. She does not refer any traumatic history. Examination revealed: temperature 38.9°C, 120 beats per minute, respiratory rate 30/min, blood pressure 90/54 mm Hg. Laboratory tests showed leukocytosis with left deviation (25,000 leukocytes/mm3, 80% neutrophils); increased creatinine (1.6 mg/dL) and CK (138 U/L). Among the following, which is the most likely diagnosis:

[Fatal necrotizing fasciitis due to Serratia marcescens].

An 81 year old female patient with chronic heart failure and atrial fibrillation receiving anticoagulant therapy, was admitted with progressive pain on her right leg for the past 24 hours, associated to local erythema, edema and warmth. The lesion evolved at the same site where she presented a chronic ulcer for the previous 5 months managed only with local care. At admission a necrotic plaque on the affected site was perceived; there was no hypotension or mental confusion but signs of a deep venous thrombosis on the involved leg were found. She was febrile (37.8 degrees C) and with tachychardia (126 per minute). Laboratory evaluation revealed normal white blood cell count and a subtherapheutic anticoagulant INR value. A chest x-ray showed infiltrates on the left lower lung lobe. On the following hours the lesion evolved with increasing pain, haemorrhagic bullae and a purulent discharge through the ulcer, with the patient developing mental deterioration, hypotension, respiratory failure and shock. The patient received intravenous ciprofloxacin and clindamycin and was operated 15 hours after admission performing an over-the knee amputation. A cardiac catheterization demonstrated a low cardiac output (2.3 L/min), and both a high systemic vascular resistance (2888 din.s.cm(-5)) and pulmonary capillary wedge pressure (17 cm H(2)0), results compatible with cardiogenic shock. Evolution was progressively worse and she died of multiple organic failure 36 hours after admission. Two blood culture samples grew Serratia marcescens. No necropsy was performed and cultures taken from the leg remained negative.

Dyspnoe, cough and fever in 80-year old patient with chronic heart failure.

The prevalence of heart failure (HF) in developed countries exceeds 10% in adults over 70 year old. At the following report we aim to present a case of HF worsening complicated by gout attack. 80-year old patient was admitted to the hospital, with the suspicion of pneumonia, because of 3-day history of dyspnoe, cough and fever. Patient reported redness, swelling and pain in the area of left elbow. Prior to admission patient was diagnosed with bursitis and treated with antibiotic without symptoms resolution. There was past medical history of atrial fibrillation, hypertension, intermittent claudication, COPD. One month before, patient was hospitalized due to HF worsening. Diuretics' dosage was increased at that time and symptoms resolved. On admission: blood pressure 145/88 mm Hg, HR 96/min irregular, saturation O2 88% without oxygen therapy. On physical examination, bilateral pulmonary crackles, redness, tenderness of left elbow were found. Laboratory tests demonstrated elevated parameters of inflammation - leukocytosis 13.4G/L, neutrophilia 11G/L, CRP 142.5 mg/L, but normal procalcitonin 0.27 ng/ml. Moreover, high NTproBNP 8573 pg/ml and hyperuricemia 13.1 mg/dl were detected. Chest X-ray indicated pulmonary venous congestion. ECG revealed atrial fibrillation with QRS rate of 100/min, left axis deviation. Therefore, gout attack was diagnosed and after colchicine administration symptoms resolved quickly. Clinical signs including fever, elevated parameters of inflammation and dyspnoe justify pneumonia consideration in differential diagnosis. Importantly, non-infectious causes of inflammatory conditions, like gout must be also considered. Patients with HF often develop hyperuricemia due to diuretic treatment, aggravated catabolism and often co-prevalent chronic kidney disease.

Community-acquired pneumonia

Hospitalization Some CAP patients require intensive care, with clinical prediction rules such as the pneumonia severity index and CURB-65 guiding the decision whether or not to hospitalize. Factors increasing the need for hospitalization include: Age greater than 65 Underlying chronic illnesses Respiratory rate greater than 30 per minute Systolic blood pressure less than 90 mmHg Heart rate greater than 125 per minute Temperature below 35 or over 40 °C Confusion Evidence of infection outside the lung Laboratory results indicating hospitalization include: Arterial oxygen tension less than 60 mm Hg Carbon dioxide over 50 mmHg or pH under 7.35 while breathing room air Hematocrit under 30 percent Creatinine over 1.2 mg/dl or blood urea nitrogen over 20 mg/dl White-blood-cell count under 4 × 10^9/L or over 30 × 10^9/L Neutrophil count under 1 x 10^9/L

Case 294.

History A 50-year-old woman presented to the emergency department of our hospital with a 2-day history of lower limb pain associated with unusual asthenia and diffuse arthralgia over the past 3 weeks. She was a native of Guinea and had lived in France for most of her life, working as a personal care assistant. Her only medical history of note was an occurrence of fetal death at 12 weeks gestation when she was 35 years old. She had bilateral lower limb swelling, without changes in skin temperature or color. All proximal and distal arterial pulses were felt. General physical examination findings were otherwise unremarkable. Her laboratory tests showed a decreased hemoglobin concentration of 8.9 g/dL (normal range, 12-16 g/dL), a decreased platelet count of 45 × 10<sup9</sup/L (normal range, 150-400 × 10<sup9</sup/L), a C-reactive protein level of 158 mg/L (normal range, <5 mg/L) and a d-dimer level of 2000 mg/L (normal range, <500 mg/L). Compression US of the lower limbs revealed bilateral calf vein thrombosis involving the fibular and posterior tibial veins. Curative anticoagulation using low-molecular-weight heparin (enoxaparin, subcutaneous injection of 100 units per kilogram of body weight twice a day) was started. The day after the start of anticoagulation therapy, the patient reported dyspnea and acute chest and abdominal pain. Her vital signs were assessed, and she had elevated blood pressure and increased heart rate and respiratory rate, but she remained afebrile. Her cardiac auscultation was unremarkable, besides tachycardia. Skin examination revealed small areas of necrosis on the fingertips of her right hand. Laboratory studies were repeated and showed an increase in serum creatinine level from a baseline value of 0.49 mg/dL to a new value of 1.01 mg/dL (normal range, 0.6-1.1 mg/dL), an apparition of low-grade proteinuria of 0.43 g per day (normal range, <0.3 g/day), and a high serum troponin level of 1066 ng/L (normal range, <14 ng/L), whereas electrocardiography showed no ST segment modification and echocardiography revealed a moderately altered left ventricular ejection fraction (45%). There was no coronary occlusion seen at emergency coronarography. Contrast-enhanced CT of the chest, abdomen, and pelvis was performed (Figs 1, 2) together with cardiac MRI (Figs 3, 4).

[A 62-year-old man with an acute onset of consciousness disturbances].

We report a 62-year-old man who developed coma and died in a fulminant course. The patient was well until May 1, 1996 when he noted chillness, tenderness in his shoulders, and he went to bed without having his lunch and dinner. In the early morning of May 2, his families found him unresponsive and snoring; he was brought into the ER of our hospital. He had histories of hypertension, gout, and hyperlipidemia since 42 years of the age. On admission, his blood pressure was 120/70, heart rate 102 and regular, and body temperature 36.3 degrees C. His respiration was regular and he was not cyanotic. Low pitch rhonchi was heard in his right lower lung field. Otherwise general physical examination was unremarkable. Neurologic examination revealed that he was somnolent and he was only able to respond to simple questions such as opening eyes and grasping the examiner's hand, but he was unable to respond verbally. The optic discs were flat; the right pupil was slightly larger than the left, but both reacted to light. He showed ptosis on the left side, conjugate deviation of eyes to the left, and right facial paresis. The oculocephalic response and the corneal reflex were present. His right extremities were paralyzed and did not respond to pain Deep tendon reflexes were exaggerated on the right side and the plantar response was extensor on the right. No meningeal signs were present. Laboratory examination revealed the following abnormalities; WBC 18,400/ml, GOT 131 IU/l GPT 50 IU/l, CK616 IU/l, BUN 30 mg/dl, Cr 2.1 mg/ dl, glucose 339 mg/dl, and CRP 27.4 mg/dl. ECG showed sinus tachycardia and ST elevation in II, III and a VF leads and abnormal q waves in I, V5, and V6 leads. Chest X-ray revealed cardiac enlargement but the lung fields were clear. Cranial CT scan revealed low density areas in the left middle cerebral and left posterior cerebral artery territories. The patient was treated with intravenous glycerol infusion and other supportive measures. At 2: 10 AM on May 3, he developed sudden hypotension and cardiopulmonary arrest. He was pronounced dead at 3:45 AM. The patient was discussed in a neurological CPC, and the chief discussant arrived at the conclusion that the patient had acute myocardial infarction involving the inferior and the true posterior walls and left internal carotid embolism from a mural thrombus. Post mortem examination revealed occlusion of the circumflex branch of the left coronary artery due to atherom plaque rupture and myocardial infarction involving the posterior and the lateral wall with a rupture in the postero-lateral wall. Marked atheromatous changes were seen in the left internal carotid, the middle cerebral and the basilar arteries; the left internal carotid and the middle cerebral arteries were almost occluded by thrombi and blood coagulate. The territories of the left middle cerebral and the occipital arteries were infarcted; but the left thalamic area was spared. The neuropathologist concluded that the infarction was thrombotic origin not an embolic one as the atherosclerotic changes were severe. Cardiac rupture appeared to be the cause of terminal sudden hypotension and cardiopulmonary arrest. It appears likely that a vegetation which had been attached to the aortic valve induced thromboembolic occlusion of the left internal carotid artery which had already been markedly sclerotic by atherosclerosis. It is also possible that the vegetations in the aortic valve came from mural thrombi at the site of acute myocardial infarction, as no bacteria were found in those vegetations.

When Coke Is Not Hydrating: Cocaine-Induced Acute Interstitial Nephritis.

A 47-year-old African American man was admitted with 4 days of back pain, nausea and vomiting, and low urine output. There was no history of fever, dysuria, frequency, hesitancy, viral symptoms, trauma, rash, or constipation. Despite his past medical history of hypertension, diabetes mellitus, and hyperlipidemia he denied taking any medications for 18 months, including nonsteroidal anti-inflammatory drugs, acetaminophen, or antacids. He denied smoking and alcohol but admitted to cocaine use. No significant FH. Physical examination results were as follows: BP 235/125 mm Hg, heart rate 90 beats/min, temperature 98°F, O2 saturation normal; lungs and heart normal, abdomen soft but bilateral costovertebral angle tenderness. Neurological examination was normal. Laboratory tests yielded the following results: creatinine (Cr) 10.5 mg/dL (1.2 mg/dL in 2010), blood urea nitrogen 63 mg/dL, glucose 151 mg/dL, Ca 9.4 mg/dL, PO4 6.1 mg/dL, Hgb 15 g/dL, white blood cells (WBC) 9100, platelets 167 000, amylase/lipase normal, aspartate aminotransferase/alanine aminotransferase (AST/ALT) normal, bilirubin 1.4 mg/dL, alkaline phosphatase 39 IU/L, creatine phosphokinase 127 µg/L. Hepatic panel, C- and P-ANCA (cytoplasmic- and perinuclear-antineutrophil cytoplasm antibodies, respectively), anti-GBM (anti-glomerular basement membrane), antimyeloperoxidase, antinuclear antibody, and Helicobacter pylori were all negative. C3, C4 normal, urinalysis: 2+ blood, no white blood cells or eosinophils, no casts, no albumin, negative for nitrate/leukocyte esterase and bacteria. Imaging: chest radiograph, abdominal radiograph, computed tomography of the abdomen, electrocardiography, and transthoracic echocardiography were all normal. Course. The patient's urine output declined from 700 to 400 cm(3)/d and the on third day he required hemodialysis with Cr 14 mg/dL. Renal biopsy showed typical findings of interstitial nephritis. The patient was dialyzed for 10 days and responded to steroids and went home with an improving Cr of 3.5 mg/dL, back to baseline of 1.5 in 8 weeks. Discussion. Internists encounter patients with acute kidney injury (AKI) on a daily basis, most of which can be explained by prerenal azotemia, acute tubular necrosis (ATN), obstruction, or rhabdomyolysis among other etiologies. Cocaine is only rarely implicated as an etiology of AKI and if it is, usually the injury is due to ATN or pigment effects. Acute interstitial nephritis (AIN) caused by cocaine, on the other hand, has only been described in a handful of cases. AIN is a renal lesion that causes a decline in creatinine clearance and is characterized by an inflammatory infiltrate in the kidney interstitium and is most often associated with drug therapy. AIN can also be seen in autoimmune disorders like systemic lupus erythematosus, Sjögren's syndrome, or sarcoidosis; or with infections remote to the kidney like Legionella, leptospirosis, and streptococcal disease. Our case was very similar to the other reported cases of AIN due to cocaine in that all have occurred in middle-aged African American males and all have responded to steroids. This case reminds clinicians to consider AIN in patients with AKI and a history of cocaine abuse.

A 30-Day-Old Infant with Necrotizing Fasciitis of the Perineal Region Involving the Scrotum Due to Methicillin-Resistant Staphylococcus aureus (MRSA) and Extended-Spectrum β-Lactamase (ESBL)-Producing Klebsiella pneumoniae: A Case Report.

BACKGROUND Fournier's gangrene is an idiopathic form of necrotizing fasciitis involving the genital and perineal regions; it is associated with high complication and mortality rates. Rarely, perineal infection may be caused by hospital-acquired antimicrobial-resistant bacteria. This report is of a 30-day-old infant with methicillin-resistant Staphylococcus aureus (MRSA) and extended-spectrum ß-lactamase (ESBL)-producing Klebsiella pneumoniae necrotizing fasciitis involving the perineal region. CASE REPORT A 30-day-old male infant presented to the Emergency Department with rapidly progressive white discoloration of scrotal skin since 3 days prior to admission, progressing from 2-3 white spots to covering two-thirds of the scrotal skin. Pain upon urination was noted, with normal appetite and bowel movements. He had a history of diaper rash 6 days earlier accompanied by fever, and the rash was treated with topical antifungal and corticosteroid ointment. He was born at term by caesarean delivery, with birth weight 2900 g. Laboratory examinations revealed leukocyte count 23 000/µL and CRP 26.8 mg/dL. Hemoglobin was 10.6 g/dL, serum sodium was 134 mEq/L, blood glucose was 80 mg/dL, serum urea was 15 mg/dl, and creatinine was 0.27 mg/dL. Chest and abdominal X-rays were normal. He received broad-spectrum antibiotics and underwent surgical debridement, and necrotic tissue was obtained for biopsy and culture. Histology examination showed non-specific granulation tissue consistent with Fournier gangrene. Soft- tissue culture isolated MRSA and ESBL-K. Antibiotics were changed according to the sensitivity report. Blood and urine cultures were negative. CONCLUSIONS Immediate surgery and antibiotics are essential in treating Fournier gangrene to avoid life-threatening complications. Initial symptoms are non-specific. Diagnosis remains primarily clinical, confirmed by intraoperative macroscopic findings.

Mystery in the basement.

56 year-old male presented to the University of Mississippi Medical Center emergency department (ED) with complaints of progressive shortness of breath, productive cough, fever, and malaise. His past medical history was significant for hypertension as well as a 60 pack-year history of smoking. Upon arrival to the ED he had a temperature of 103.6 degrees F, blood pressure of 80/40 mm Hg, a pulse of 110 beats per minute, respirations of 28 per minute, and an oxygen saturation of 50% on room air. He appeared to be in significant respiratory distress. Lung examination revealed diffuse bilateral rhonchi and wheezes in all lung fields. He was emergently intubated. Chest radiograph demonstrated a miliary pattern scattered throughout all lung fields in addition to parenchymal opacities. A complete blood count revealed a white blood cell count of 33,500 10(3)/microL, hematocrit of 37%, and platelets of 906,000 10(3)/uL. Blood urea nitrogen and creatinine were 27 mg/dL and 1.0 mg/dL, respectively. Initial ABG on 100% oxygen showed pH 7.15, pCO2 82 mm Hg, and pO2 62 mm Hg. Troponin I was negative. An electrocardiogram demonstrated sinus tachycardia. Blood and urine cultures were obtained.

Case 294: Catastrophic Antiphospholipid Syndrome.

History A 50-year-old woman presented to the emergency department of our hospital with a 2-day history of lower limb pain associated with unusual asthenia and diffuse arthralgia over the past 3 weeks. She was a native of Guinea and had lived in France for most of her life, working as a personal care assistant. Her only medical history of note was an occurrence of fetal death at 12 weeks gestation when she was 35 years old. She had bilateral lower limb swelling, without changes in skin temperature or color. All proximal and distal arterial pulses were felt. General physical examination findings were otherwise unremarkable. Her laboratory tests showed a decreased hemoglobin concentration of 8.9 g/dL (normal range, 12-16 g/dL), a decreased platelet count of 45 × 10<sup9</sup/L (normal range, [150-400] × 10<sup9</sup/L), a C-reactive protein level of 158 mg/L (normal range, <5 mg/L), and a d-dimer level of 2000 mg/L (normal range, <500 mg/L]). Compression US of the lower limbs revealed bilateral calf vein thrombosis involving the fibular and posterior tibial veins. Curative anticoagulation using low-molecular-weight heparin (enoxaparin, subcutaneous injection of 100 units per kilogram of body weight twice a day) was started. The day after the start of anticoagulation therapy, the patient reported dyspnea and acute chest and abdominal pain. Her vital signs were assessed, and she had elevated blood pressure and increased heart rate and respiratory rate, but she remained afebrile. Her cardiac auscultation was unremarkable, besides tachycardia. Skin examination revealed small areas of necrosis on the fingertips of her right hand. Laboratory studies were repeated and showed an increase in serum creatinine level from a baseline value of 0.49 mg/dL to a new value of 1.01 mg/dL (normal range, 0.6-1.1 mg/dL), an apparition of low-grade proteinuria of 0.43 g per day (normal range, <0.3 g/ day), and a high serum troponin level of 1066 ng/L (normal range, <14 ng/L), whereas electrocardiography showed no ST segment modification and echocardiography revealed a moderately altered left ventricular ejection fraction (45%). There was no coronary occlusion seen at emergency coronarography. Contrast-enhanced CT of the chest, abdomen, and pelvis was performed (Figs 1, 2) together with cardiac MRI (Figs 3, 4).

Cases from the Osler Medical Service at Johns Hopkins University.

PRESENTING FEATURES: A 70-year-old African American man was admitted with a history of fever, chills, and malaise of several days' duration. His past medical history was notable for end-stage renal disease requiring hemodialysis, coronary artery disease, and aortic stenosis requiring a bioprosthetic aortic valve replacement. On the day of admission, the patient was noted to have a shaking chill while undergoing dialysis through his catheter and was admitted to the hospital. He complained of pain at the catheter insertion site, shortness of breath, and dyspnea on exertion, but denied chest pain. On physical examination, the patient had a temperature of 100.4 degrees F, with a heart rate of 64 beats per minute, blood pressure of 127/72 mm Hg, and an oxygen saturation of 97% on room air. He was a mildly obese man in no apparent distress. He had shotty cervical lymphadenopathy and a right subclavian dialysis catheter in place, with erythema and pus at the entry site. His jugular venous pressure was 10 cm H(2)O. Lung examination showed bibasilar rales. Heart sounds were normal, with no rub or gallop. He had a 2/6 systolic ejection murmur best heart at the left sternal border as well as a 3/6 holosystolic murmur at the apex that radiated to his left axilla. Examination of the abdomen and extremities was unremarkable. The patient's neurological examination was unremarkable, and he was alert and oriented to person, place, and time. Laboratory studies showed an elevated white blood cell count of 16,700 cells/microL. His blood urea nitrogen level was 43 mg/dL and his serum creatinine level was 4.9 mg/dL. Multiple blood cultures grew methicillin-resistant Staphylococcus aureus. An admission, chest radiograph showed no infiltrate. An admission electrocardiogram showed normal sinus rhythm with first degree atrioventricular block, left anterior fascicular block, and left ventricular hypertrophy. shows rhythm strips from lead II electrocardiograms 5 months before admission (top), on admission (middle) and 5 days after admission (bottom). What is the diagnosis?

[Rhabdomyolysis, hyponatremia and fever in a patient with Schmidt's syndrome].

A 38-year-old man had fever (40 degrees C) and a swollen right leg. Two weeks before admission he had received non-steroid anti-inflammatory drugs (paracetamol and ibuprofen) after a tooth extraction. Some weeks before he had noticed a rough voice and a dry skin. The patient had a sinus tachycardia of 145/min (blood pressure of 130/80 mm Hg). Laboratory data revealed a CK of 16,650 U/l (< 80 U/l), myoglobin of 2420 U/l (< 90 microg/l) and LDH of 1250 U/l (<240 U/l), leukocyte count of 12,000 / microl and C-reactive protein of 3.0 mg/dl (<0.5 mg/dl). Sodium was markedly decreased (110 mmol/l (135 - 145 mmol/l)). Determination of thyroid hormone showed primary hypothyroidism with an elevated TSH of 62.6 mU/l (0.3 - 4.0 mU/l); T4 of 38 nmol/l (58 - 154 nmol/l), T3 1.17 of nmol/l (1.23 - 3.08 nmol/l) and fT4 of 6 pmol/l (10 - 25 pmol/l). The thyroid autoantibodies were increased (thyroxine peroxidase antibodies of 684 U/l (<35 U/l) and thyroglobulin antibodies of 173 U/l (<40 U/l)). Ultrasound of the thyroid revealed an nonhomogeneous structure. Cortisol at 8.00 a. m. was reduced by 63 mmol/l (180 - 640 mmol/l) and did not increase after administration of ACTH (60 min. cortisol at 90 mmol/l (>550 mmol/l)). ACTH was increased (141 pg/ml; normal range 17 - 52 pg/ml). The initial therapy consisted of hydrocortisone (100 mg i.v as bolus and 100 mg during the next 24 hours) and levothyroxine replacement (200 micro g) was initiated. During the following 8 days clinical symptoms regressed. Values of sodium, myoglobin and LDH decreased. After therapy with cephalosporin (Ceftriaxon) and penicillin (Flucloxacillin) fever and inflammation parameters decreased. This is a rare case of a rhabdomyolysis and hyponatriaemia due to hypothyroidism and Addison's disease (Schmidt's syndrome).

Heroin addict with gangrene of the extremities, rhabdomyolysis and severe hyperkalemia.

Long-time consumption of narcotics leads to altered mental status of the addict. It is also connected to damages of different organic systems and it often leads to appearance of multiple organ failure. Excessive narcotics consumption or abuse in a long time period can lead to various consequences, such as atraumatic rhabdomyolysis, acute renal failure and electrolytic disorders. Rhabdomyolysis is characterized by injury of skeletal muscle with subsequent release of intracellular contents, such as myoglobin, potassium and creatine phosphokinase. In heroin addicts, rhabdomyolysis is a consequence of the development of a compartment syndrome due to immobilization of patients in the state of unconsciousness and prolonged compression of extremities, direct heroin toxicity or extremities ischemia caused by intraluminal occlusion of blood vessels after intraarterial injection of heroin. Severe hyperkalemia and the development of acute renal failure require urgent therapeutic measures, which imply the application of either conventional treatment or a form of dialysis. We presented a male patient, aged 50, hospitalized in the Emergency Center Kragujevac due to altered mental status (Glasgow Coma Score 11), partial respiratory insufficiency (pO2 7.5 kPa, pCO2 4.3 kPa, SpO2 89%), weakness of lower extremities and atypical electrocardiographic changes. Laboratory analyses, carried out immediately after the patient's admission to the Emergency Center, registered the following disturbances: high hyperkalemia level (K+ 9.9 m mol/L), increased levels of urea (30.1 mmol/L), creatinine (400 micromol/L), creatine phosphokinase--CK (12,0350 IU/L), CK-MB (2500 IU/L) and myoglobin (57000 microg/L), with normal levels of troponin I (< 0.01 microg/L), as well as signs of anemia (Hgb 92 g/L, Er 3.61 x 10(12)/L), infection (C-reactive proteine 184 microg/mL, Le 16.1 x 10(9)/L) and acidosis (base excess--18.4 mmol/L, pH 7.26. Initial examination of the patient revealed swelling and paleness of the right lower leg, signs of gangrene of the right foot and the 1st and the 4th toes of the left foot. The patient had normal values of arterial pressure (130/80 mmHg) and heart rate (64/min(-1)); roentgenographic lungs examination and computerized tomography (CT) brain examination did not reveal pathological changes in lung and brain parenchyma; toxicological analyses confirmed the presence of heroin in patient's organism. The patient was treated by intensive conventional treatment (infusion of crystalloid solutions, 8.4% solution of sodium bicarbonate, i.v. infusion of diuretics, calcium gluconate and short-acting insulin), and also by antibiotics and anticoagulants. Normalization of kalemia and fast regression of electrocardiographic changes were registered. The patient refused the suggested surgical treatment (fasciotomy, foot amputation). After stabilization of kidney function and improvement of his mental state, the patient agreed to undergo surgical procedure. Therefore, on the day 30 of hospitalization the above-knee amputation of the right leg was performed, and on the day 38 the transmetatarsal amputation of the left foot was carried out. After 46 days of hospital treatment, the patient was released and sent to home treatment. The routine laboratory diagnostics, which implies determining of the levels of potassium, urea, creatinine and CK in the serum of all hospitalized heroin addicts can contribute to timely detection of hyperkalemia and acute kidney weakness and undertaking of appropriate therapeutic measures.

Necrotizing fasciitis

Scoring system A white blood cell count greater than 15,000 cells/mm3 and serum sodium level less than 135 mmol/l have a sensitivity of 90% in detecting the necrotizing soft tissue infection. It also has a 99% chance of ruling out necrotizing changes if the values have shown otherwise. Various scoring systems are being developed to determine the likelihood of getting necrotizing fasciitis, but a scoring system developed by Wong and colleagues in 2004 is the most commonly used. It is the laboratory risk indicator for necrotizing fasciitis (LRINEC) score, which can be used to stratify by risk those people having signs of severe cellulitis or abscess to determine the likelihood of necrotizing fasciitis being present. It uses six laboratory values: C-reactive protein, total white blood cell count, hemoglobin, sodium, creatinine, and blood glucose. A score of 6 or more indicates that necrotizing fasciitis should be seriously considered. The scoring criteria are: CRP (mg/L) ≥150: 4 points

Necrotizing fasciitis

CRP (mg/L) ≥150: 4 points WBC count (×103/mm3) <15: 0 points 15–25: 1 point >25: 2 points Hemoglobin (g/dl) >13.5: 0 points 11–13.5: 1 point <11: 2 points Sodium (mmol/l) <135: 2 points Creatinine (umol/l) >141: 2 points Glucose (mmol/l) >10: 1 point

Pharmacology_Katzung

A 65-year-old man undergoes cystoscopy because of the presence of microscopic hematuria in order to rule out urologic malignancy. The patient has mild dysuria and pyuria and empirically receives oral therapy with cip-rofloxacin for presumed urinary tract infection prior to the procedure and tolerates the procedure well. Approxi-mately 48 hours after the procedure, the patient presents to the emergency department with confusion, dysuria and chills. Physical exam reveals a blood pressure of 90/50, pulse of 120, temperature of 38.5° C and respira-tory rate of 24. The patient is disoriented but the physical exam is otherwise unremarkable. Laboratory test shows WBC 24,000/mm3 and elevated serum lactate; urinalysis shows 300 WBC per high power field and 4+ bacteria. What possible organisms are likely to be responsible for the patient’s symptoms? At this point, what antibiotic(s) would you choose for initial therapy of this potentially life-threatening infection?

Bornholm disease

Physical exam findings In a studied case of Bornholm disease the chest pain was unable to be reproduced on palpation and failed to improve with changes in position. The pain was made worse during deep inhalation. A pleural rub was present, however lung auscultation was clear and rashes were absent. Laboratory findings and imaging In a prior case of Bornholm disease the laboratory results showed the white blood cell count, hemoglobin, hematocrit, creatinine, liver function test (LFT), troponin, and creatine kinase (CK) were all within normal limits. The chest x-ray showed bilateral pleural effusions which resolved after infection. The erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) levels were found to be elevated. The electrocardiogram (EKG) did not show any abnormalities related to ischemia.

[Septicemic melioidosis in Southern Taiwan: a case report].

The patient was a 56 year-old man, a resident of Hen-Tsueng Township in Ping-Tung County. He worked as a ranger at Ken-Ting Farm in southern Taiwan, and had been to Thailand for sight-seeing 5 years ago. He came to our outpatient department about one month prior to hospitalization for intermittent fever of one week duration. At that time, complete blood count was within normal limits and a chest roentgenogram was unremarkable. He was given erythromycin without showing any clinical improvement. Two days prior to admission, he noted pain in the left hip. The next day, severe dyspnea developed suddenly and chest x-ray film revealed bilateral nodular lesions. Physical examination on hospitalization revealed an acutely ill and jaundiced male with a temperature of 37.4 degrees C, blood pressure: 110/47 mmHg, pulse rate: 137/min, and respiratory rate: 26/min. There were rales in both lungs, but no lymphadenopathy or organ enlargement. Laboratory study showed WBC: 1,470/mm(3), platelet count: 47,000/mm(3), blood sugar: 226 mg/dL, mildly elevated transaminases and bilirubin, and BUN: 69 mg/dL, Cr: 4.3 mg/dL. Arterial blood gas analysis indicated an acute metabolic acidosis with PaO2 of 32 mmHg. Despite the initial impression of melioidosis and administration of ceftazidime plus gentamicin, his condition rapidly deteriorated and expired 18 hours after admission. Two sets of blood cultures grew Burkholderia pseudomallei. Melioidosis has been called a great imitator of diseases and culture results are frequently misinterpreted. The mortality is high even with suggested therapy with ceftazidime, cotrimoxazole, amoxicillin-clavulanate, chloramphenicol, and/or tetracycline. There has been a total of 10 cases reported in southern Taiwan and 2 of them were clearly indigenous. Melioidosis should be included in the reportable diseases, and its prevalence in Taiwan also should be investigated.

First_Aid_Step2

■Signs and symptoms are similar to those of cystitis but show evidence of upper urinary tract disease. ■ Symptoms include f ank pain, fever/chills, and nausea/vomiting. Dysuria, frequency, and urgency are also possible. ■UA and culture: Results are similar to those of cystitis, but with WBC casts. Send blood cultures to rule out urosepsis. Pyelonephritis is the most common serious medical complication of pregnancy. Twenty to thirty percent of patients with untreated bacteriuria will develop pyelonephritis. When in doubt, admit a patient with pyelonephritis and administer IV antibiotics. Urosepsis must be considered in any elderly patient with altered mental status. SIRS = two or more of the following: 1. Temperature: Either < 36°C or > 38°C (i.e., hypothermia or fever). 2. Tachypnea: > 20 breaths per minute or PaCO2 < 32 mmHg on ABG. 3. Tachycardia: HR > 90 bpm. 4. Leukocytosis/leukopenia: WBC < 4000 cells/mm3 or > 12,000 cells/mm3. CBC: Reveals leukocytosis.

[A case of microscopic polyangiitis with severe cardiac and respiratory muscle involvement].

A 66-year-old female was admitted to our hospital in January, 1998, complaining of low grade fever and muscle weakness of her legs. Physical examination revealed muscle weakness of her neck (4/5) and proximal skeletal muscles of her bilateral legs (3/5-4/5). She showed proteinuria and microhematuria. Her serum levels of ureanitrogen, creatinine, aspartate aminotransferase, alanine aminotransferase, creatinekinase, aldolase and myoglobin were all within the normal ranges. Antinuclear antibodies were negative, but her serum levels of pANCA (743 EU) and C reactive protein (18.0 mg/dl) were elevated. Neuroconduction velocity of her left common peroneal nerve was decreased to 40.8 m/sec and electric myograph showed neurogenic changes. Magnetic resonance images (MRI) of her bilateral thigh depicted high signal intensity in quadriceps by T 2 weighed images, but the signals were not enhanced by gadolinium injection. Muscle and renal biopsies revealed necrotizing vasculitis of the small arteries. Crescentic glomerulonephritis was also observed by renal biopsy. These findings supported the diagnosis of microscopic PN. On 16 th admission day, she developed acute cardiac and respiratory failures due to cardiac and respiratory muscle involvements with PN, and was assisted by mechanical ventilation. She was treated with methylprednisolone pulse therapy (500 mg/day, three consecutive days) on 18 th admission day, followed by 40 mg of oral prednisolone daily. However, her symptoms deteriorated, and herserum creatinine levels increased to 2.4 mg/dl. On 24 th admission day, intravenous cyclophosphamide pulse therapy (500 mg/day) was instituted. Her cardiac wall motion on echocardiography and serum creatinine levels gradually improved, but her skeletal and respiratory muscle weakness did not improve. On 38 th admission day, she was complicated with respiratory infection by methicillin resistant Staphylococcus aures. On 62 th admission day, she died of endotoxic shock. This is the first report describing respiratory muscle involvement with PN, and the second report describing MRI findings of muscle involvement by PN. Therefore, our case provides important clinical information for the diagnosis and treatment of the disease.

Myxedema coma associated with combination aripiprazole and sertraline therapy.

To describe a case of myxedema coma (MC) associated with combination aripiprazole and sertraline therapy. A 41-year-old male presented to the emergency department with confusion, right-sided numbness and tingling, slurred speech, dizziness, and facial edema. His blood pressure was 160/113 mm Hg, with a pulse of 56 beats/min and temperature of 35.4 degrees C. Initial abnormal laboratory values included creatine kinase (CK) 439 U/L; serum creatinine 1.6 mg/dL; aspartate aminotransferase 85 U/L; and alanine aminotransferase 35 U/L. Repeat cardiac markers revealed an elevated CK level of 3573 U/L with a CK-MB of 24 ng/mL. Thyroid function tests showed thyroid-stimulating hormone 126.4 microIU/mL and free thyroxine 0.29 ng/dL. Home medications of unknown duration were sertraline 200 mg and aripiprazole 20 mg daily. He was admitted to the intensive care unit and initially treated with intravenous levothyroxine and dexamethasone. By hospital day 4, the patient was clinically stable and discharged to home. Myxedema coma, the most significant form of hypothyroidism (HT), is a rare but potentially fatal condition. The known precipitating causes of MC were ruled out in this patient, which left his home medications as the likely cause. Cases of HT caused by certain atypical antipsychotics and antidepressants are found in the literature, but none was reported with aripiprazole therapy. There are also no reported cases of sertraline or aripiprazole inducing MC. Use of the Naranjo probability scale indicates that the combination of aripiprazole and sertraline was a probable inducer of MC in this patient. Due to the widespread use of psychotropic medications, clinicians should be reminded of the rare, yet life-threatening, occurrence of MC when treating patients, especially with combination therapies such as sertraline and aripiprazole.

Case 282.

HistoryA 63-year-old woman with a history of left mastectomy for breast cancer and partial gastrectomy with Roux-en-Y reconstruction for nonhealing peptic ulcer presented to the emergency department and reported a 1-month history of abdominal distention, fevers, chills, and flu-like symptoms. She was initially suspected of having flu, and she completed a course of oseltamivir; however, she had continued fatigue, fever, chills, abdominal bloating, and loss of appetite. She reported no contact with a sick person or recent travel. At admission, laboratory studies revealed leukocytosis, with a white blood cell count of 15.1 × 10<sup3</sup/µL (15.1 × 10<sup9</sup/L) (normal range, 4.0-10.0 × 10<sup3</sup/µL [4.0-10.0 × 10<sup9</sup/L]), an elevated sedimentation rate of 100 mm per hour (normal range, 0-30 mm per hour), and a C-reactive protein level of 203.8 mg/L (1940.9 nmol/L) (normal range, ≤10 mg/L [≤95.2 nmol/L]). Liver enzyme levels were elevated, with an alanine aminotransferase level of 48 U/L (0.80 µkat/L) (normal range, 0-29 U/L [0-0.48 µkat/L]), an aspartate aminotransferase level of 98 U/L (1.6 µkat/L) (normal range, 10-37 U/L [0.16-0.62 µkat/L]), an alkaline phosphatase level of 682 U/L (11.4 µkat/L) (normal range, 65-195 U/L [1.1-3.3 µkat/L]), and a total bilirubin level of 1.5 mg/dL (25.7 µmol/L) (normal range, 0.3-1.0 mg/dL [5.1-17.1 µmol/L]). Abdominopelvic CT was performed (Figs 1-3).

[Status epilepticus induced by prolonged immersion in hot herb bath: report of one case].

A 6-year-old girl with cerebral palsy developed conscious disturbance and generalized convulsion after one-hour hot herb drug bath. Physical examination on admission revealed rectal temperature 41 degrees C, hot skin, respiration 46/min, regular heart beat 98/min, BP 130/60 mmHg, Glascow coma scale 4 (E2M1V1), soft and flat abdomen, no hepatosplenomegaly, no skin rash, no focal neurological sign, increased generalized muscle ton. Laboratory data showed CBC: WBC 20400 cumm (Neutrophils 31%, Lymphocytes 69%), Hb 11.6gm%, ESR 11 mm/hr, arterial blood gas: PH 7.077, PO2 43mmHg, PCO2 57.1mmHg, HCO3- 16 mEq/L, BE-11.5mEq/L, serum sodium 143 mEq./L, potassium 5.2 mEq/L, chloride 101 mEq/L, free calcium ion 3.8mg%, GOT 63IU/L, GPT 263 IU/L, amylase 193 IU/L, alkaline phosphatase 388 IU/L, LDH 1245 IU/L, CPK 677 IU/L, total bilirubin 0.8 mg/dl, direct type 0.1 mg/dl, BUN 18 mg/dl, Glucose 35 mg/dl. Urinalysis revealed proteinuria( ) trace hematuria and pyuria, but no cast. Lumbar puncture is within normal limits. Bacteriology including blood and CSF are normal. Multiple organ failure was noted at that time. Intensive cooling methods were performed including central and peripheral cooling. We used luminal and valium to control the seizure. Condition didn't improve. Afterwards cardiopulmonary arrest developed. Patient expired 8 hours after admission despite of resuscitation. Heat stroke in infancy and childhood is different from that in adulthood. The predisposing factors are high ambient temperature, dehydration, very young baby, sweat gland dysfunction, or ectodermal dysplasia. Definition of heat stroke includes 1) rectal temperature above 41 degrees C, 2) behavioral change, 3) warm skin, wet or dry.(ABSTRACT TRUNCATED AT 250 WORDS)

Case 282: Fishbone Pylephlebitis.

HistoryA 63-year-old woman with a history of left mastectomy for breast cancer and partial gastrectomy with Roux-en-Y reconstruction for nonhealing peptic ulcer presented to the emergency department and reported a 1-month history of abdominal distention, fevers, chills, and flu-like symptoms. She was initially suspected of having flu, and she completed a course of oseltamivir; however, she had continued to experience fatigue, fever, chills, abdominal bloating, and loss of appetite. She reported no contact with a sick person or recent travel. At admission, laboratory studies revealed leukocytosis, with a white blood cell count of 15.1 × 103/μL (15.1 × 109/L) (normal range, 4.0-10.0 × 103/μL [4.0-10.0 × 109/L]), an elevated sedimentation rate of 100 mm per hour (normal range, 0-30 mm per hour), and a C-reactive protein level of 203.8 mg/L (1940.9 nmol/L) (normal range, ≤10 mg/L [≤95.2 nmol/L]). Liver enzyme levels were elevated, with an alanine aminotransferase level of 48 U/L (0.80 µkat/L) (normal range, 0-29 U/L [0-0.48 µkat/L]), an aspartate aminotransferase level of 98 U/L (1.6 µkat/L) (normal range, 10-37 U/L [0.16-0.62 µkat/L]), an alkaline phosphatase level of 682 U/L (11.4 μkat/L) (normal range, 65-195 U/L [1.1-3.3 μkat/L]), and a total bilirubin level of 1.5 mg/dL (25.7 µmol/L) (normal range, 0.3-1.0 mg/dL [5.1-17.1 μmol/L]). Abdominopelvic CT was performed.

InternalMed_Harrison

Fever >38.3°C (101°F) on at least two occasions 2. Illness duration of ≥3 weeks 3. 4. Diagnosis that remains uncertain after a thorough history-taking, physical examination, and the following obligatory investigations: determination of erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) level; platelet count; leukocyte count and differential; measurement of levels of hemoglobin, electrolytes, creatinine, total protein, alkaline phosphatase, alanine aminotransferase, aspartate aminotransferase, lactate dehydrogenase, creatine kinase, ferritin, antinuclear antibodies, and rheumatoid factor; protein electrophoresis; urinalysis; blood cultures (n = 3); urine culture; chest x-ray; abdominal ultrasonography; and tuberculin skin test (TST). The range of FUO etiologies has evolved over time as a result of changes in the spectrum of diseases causing FUO, the widespread Percentage of Cases Due to Indicated Cause

Case 312.

A 29-year-old woman was referred for US of bilateral breasts during evaluation for noncyclical mastalgia predominantly in the left breast of 8 months duration. She had been taking selective serotonin receptor inhibitors for the past 6 months for a clinical diagnosis of generalized anxiety disorder. A detailed medical history revealed breast cancer in the patient's mother and grandmother. There was no history of weight loss or appetite loss, nor was there a history of any altered bowel or bladder habits. The patient was overweight, with a body mass index of 26.8 kg/m<sup2</sup, and appeared anxious during the general physical examination, with an increased pulse (102 beats per minute) and normal blood pressure (118/82 mm Hg). Local examination revealed multiple small mobile painful lesions that were palpable in all quadrants of the bilateral breasts, anterior abdominal wall, and forearm. On further questioning, the patient reported similar painful lesions were present in her mother and one brother. Laboratory investigations showed a normal hemoglobin level (12.4 g/dL; normal range, 12-15 g/dL), a total leukocyte count of 9000 cells per microliter (9 cells × 10<sup9</sup/L) (normal range, 4500-11 000 cells per microliter [4.5-11 cells × 10<sup9</sup/L]), a normal differential leukocyte count (74% neutrophils [normal range, 40%-80%], 24% lymphocytes [normal range, 20%-40%], and 2% eosinophils [normal range, 1%-4%]), and an erythrocyte sedimentation rate of 5 mm per hour (normal range, 0-29 mm per hour). High-frequency US of bilateral breasts (Figs 1, 2) was performed in conjunction with Color Doppler US (Fig 3) and shear-wave elastography of representative lesions in the breasts (Fig 4). Similar lesions were also found in the subcutaneous plane of the right forearm (Fig 5) and the anterior abdominal wall (Fig 6).

[Legionnaire's pneumonia with rhabdomyolysis and acute renal failure. A case report].

Legionella pneumophyla is the agent responsible of Legionnaire's disease. It appears as a severe pneumonia and often requires admission in Intensive Care Unit. In literature, renal failure is reported to occur in 15 percent of Legionnaire disease and this event induce a mortality over 50% of these cases. The authors describe a case of Legionnaire's pneumonia with respiratory failure, rhabdomyolysis and acute renal failure. Patient was a female, 61 yrs old, admitted to our hospital because of fever (38 degrees-38.5 degrees C), severe respiratory failure (pH = 7.49, PaCO2 = 23.1 mmHg, PaO2 = 56.7 mmHg), oliguria (< 200 ml/24 h); chest x-rays and computed tomography (TC) showed a pneumonia at right lower lobe. Among other things, in blood analysis was noted the following values: BUN = 47 mg/dl, creatinine = 2.1 mg/dl, Na+ = 133 mmol/L, Cl- = 97 mmol/L, Ca+ = 7.2 mg/dl, K+ = 5.8 mmol/L, AST = 213 U/L, ALT = 45 U/L, LDH = 1817 U/L, CPK = 16738 U/L, CPK-MB = 229 U/L, myoglobin > 4300 ng/ml., leucocyte count = 17,500/mmc (N = 92%, L = 3%, M = 5%), positive anti Legionella IgG and IgM (IgG > 1:64, IgM > 1:96), evidence of Legionella soluble antigen in the urine analysis. Therapy with clarytromicyne (500 mg b.i.d i.v.) and rifampicin (600 mg/die i.v.) was begun; computed tomography showed after six days an improvement of pulmonary lesion but, in the following days, health status and blood analysis got worse. Patient went on antibiotics and underwent haemotherapy (Hb: 8 gr/dl), haemodialysis because of acute renal failure but healthy status worse furthermore and she died on 18th days after admission. This case point out rhabdomyolysis with acute renal failure is suggestive for Legionnaire's disease and is associated with high rate of mortality.

InternalMed_Harrison

Fever ˜38.3° C (101° F) and illness lasting ˜3 weeks and no known immunocompromised state History and physical examination Stop antibiotic treatment and glucocorticoids Obligatory investigations: ESR and CRP, hemoglobin, platelet count, leukocyte count and differential, electrolytes, creatinine, total protein, protein electrophoresis, alkaline phosphatase, AST, ALT, LDH, creatine kinase, antinuclear antibodies, rheumatoid factor, urinalysis, blood cultures (n=3), urine culture, chest x-ray, abdominal ultrasonography, and tuberculin skin test

A Rare Case of MRSA Pericarditis with Expanding, Purulent Pericardial Effusion Leading to Uremic Kidney Failure from a Right, Necrotic Toe.

Purulent pericarditis is an extremely rare entity with only a few reported cases so far. This condition deserves prompt diagnosis because of its significant mortality rate if left untreated. A 76-year-old man with a past medical history of coronary artery disease (CAD) with percutaneous coronary intervention (PCI) to the left anterior descending artery (LAD) and right circumflex artery (RCA), ischemic cardiomyopathy with moderately reduced ejection fraction (EF 45-50%), peripheral artery disease (PAD), COVID-19 pneumonia complicated by fibrotic lung disease (on 3 liters of home oxygen), type-2 diabetes mellitus (T2DM), hypertension (HTN), hyperlipidemia (HLD), and chronic kidney disease (CKD) stage III presented with complaints of pleuritic chest pain and shortness of breath. On hospital day 1, he was afebrile and hemodynamically stable with physical exam remarkable for bibasilar crackles and dry gangrene of his right first toe. He developed progressive altered mental status, hypotension, oliguric renal failure, and respiratory distress on hospital day 6. On exam at this time, he had an elevated jugular venous distension (JVD) of 12-14 cm water, pericardial friction rub with decreased heart sounds, and orthopnea; all were consistent with cardiac tamponade clinically. An electrocardiogram (EKG) showed new ST elevations in leads I, II, and aVL with ST depression in aVR and V1 with only mild elevation in troponin I to 0.07 ng/mL. A transthoracic echocardiogram (TTE) was done on hospital day 7 and showed a moderate sized pericardial effusion with inferior vena cava (IVC) enlargement but no atrial collapse, ventricular collapse, IVC collapse, or respiratory variation in the mitral and tricuspid inflow velocities. Blood cultures grew methicillin-resistant <iStaphylococcus aureus</i (MRSA) on hospital day 6, and he was started on intravenous (IV) vancomycin. The differential diagnosis for his enlarging pericardial effusion included purulent pericarditis, uremic pericarditis, or hemorrhagic effusion. He had urgent diagnostic and therapeutic pericardiocentesis with removal of 350 milliliters of fluid. The pericardial fluid was cloudy, tan-brown with a gram stain showing gram-positive cocci in clusters and cultures growing MRSA, which confirmed the diagnosis of purulent pericarditis secondary to MRSA infection. After the pericardiocentesis, his blood pressure, respiratory distress, and renal failure improved. The source of the bacteremia was from osteomyelitis of his gangrenous, right toe with bone biopsy growing both MRSA and <iStreptococcus anginosus</i. He underwent toe amputation for definitive source control. He was discharged on hospital day 24 with a plan to complete 6 weeks of IV vancomycin.

Normalization of creatine kinase values in a case of rhabdomyolysis during daptomycin treatment.

A 41-year-old woman presented in the Emergency Department with suspected compartment syndrome of lower left leg (creatine kinase [CK]: 12,502 IU/L, Cr: 4.31 mg/dL). Fasciotomy of the four limb compartments was conducted. By day 2, the patient presented oliguria during previous 24 h, so daily intermittent dialysis was carried out. On day 12, the patient presented an episode of bacteremia due to Staphylococcus hominis. Treatment with vancomycin was initiated and was changed after 4 days to daptomycin due to unsatisfactory clinical progression (6 mg/kg every 48 h, according to renal function and patient's weight) (CK: 2,972 IU/L). After 15 days of treatment, the dose of daptomycin was increased to 6 mg/kg every 24 h (CrCL: 46 mL/min, CK: 83 IU/L). The antibiotic was continued for another 4 days. Fourteen days later, the patient was discharged (CK: 26 IU/L). Daptomycin could be prescribed in some patients with elevated CK values. A cut-off value of baseline CK for use of daptomycin needs to be determined.

Diagnosis and management of carbon monoxide poisoning in the emergency department.

An 89-year-old female is found by her family, lying unconscious on her kitchen floor after they had been unable to reach her by phone for several hours. EMS is activated and when the paramedics arrive, they note that the gas oven is on, and there is thin, gray smoke coming from around the door. The house gas supply is turned off, windows are opened, and the family and the patient are immediately evacuated from the home. En route to the hospital, the patient is placed on high-flow oxygen at 15 liters per minute by non-rebreather mask. Her bedside glucose determination is 229 mg/dL. Vital signs are within normal limits during transport. She opens her eyes to sternal rub, and makes spontaneous movements of all extremities. Upon arrival to the ED, the patient becomes more alert and is able to respond to your questions. She tells you that she remembers putting a tray of calzones into the oven, after which she has no recall of the day's events. She has a past medical history of "well-controlled" hypertension, hyperlipidemia, and non-insulin-dependent diabetes. Her medications include hydrochlorothiazide 25 mg daily, lisinopril 10 mg daily, simvastatin 20 mg daily, and metformin 1000 mg twice daily. On physical examination, weight is 65 kg, blood pressure is 97/50 mm Hg, heart rate is 113 beats per minute, respiratory rate is 22 breaths per minute, temperature is 37.1 degrees C (98.8 degrees F), and oxygen saturation is 99% on 15 liters per minute via non-rebreather mask. She appears her stated age. Cardiopulmonary examination is remarkable only for tachycardia. Her abdomen is soft and non-tender with normal bowel sounds. Her skin is warm and dry, and there is no peripheral edema. Her cranial nerves are intact, with briskly reactive, symmetric pupils. Motor and sensory examination is non-focal, and cerebellar testing is notable only for an intention tremor on finger-nose-finger test. Gait is normal and speech is fluent and without errors. Laboratory testing shows a hemoglobin of 10.3 g/dL and a leukocyte count of 11.7 x 10(9)/L. Electrolyte results fall within the normal range, and her serum creatinine is 1.7 mg/dL. Qualitative CK-MB and troponin I tests are positive, and the sample has been sent to the STAT lab for quantitative testing. Serum carboxyhemoglobin level is 15% with normal serum pH on an arterial blood gas. An ECG reveals deep, down-sloping inferior and lateral ST-segment depressions which were not present on a routine cardiogram 1 month prior. You have many questions about this patient's care. What symptoms and physical signs need to be addressed and treated? What additional diagnostic testing should be performed? What treatment regimen is appropriate and what should be avoided? What are the risks or delayed complications from her illness? Are there special considerations for this or other patient populations?

[Efficacy and safety of levofloxacin treatment of community--acquired pneumonia in hospitalized patients].

The aim of this prospective study was to determine the efficacy and safety of levofloxacin in the treatment of community-aquired pneumonia (CAP) in outpatient with ineffective antibiotic management, requiring hospitalization. The examined group included 25 patients (11 M, 14 F) of mean age 70+/-17,5 years with abnormalities in X-ray on admission to hospital. Risk factors for pneumonia and previous antibacterial therapy were analyzed. In the hospital they were treated for 7 days with levofloxacin 500 mg twice a day administred intravenously. Body temperature, blood cell count, ESR, CRP, AST, ALT, LDH, CPK, creatine, urea, potassium, sodium, ABG, and ECG were measured on admission and in the 3-rd and 7- th day of therapy. The chest X-rays were performed and analyzed on hospital discharge. 18 patients were aged > 65 yrs, cardiovascular diseases co-existed in 14, COPD in 9, smoking habit in 12, renal failure in 3, diabetes in 3 and alkohol addiction in 1 cases. On admission 4 patients had respiratory failure, 10 hypoxaemia. During therapy a decrease of body temperature (p<0,001), concentration of CRP (p<0,004) and LDH (p<0,03), CPK (p<0,04) and increase of PaO2 (p<0,012) were observed. The changes of other parameters were not statistically significant. We did not observe any changes in ECG. On discharge from the hospital in 16 patients complete regression and in 6 patients partial regression of lesions in chest X-ray examination were observed. In 3 patients levofloxacin therapy was noneffective: in 2 because of persistent high body temperature after 3 days of treatment and in 1 patients because of recurrent of fever. Adverse events were mild. Transient exacerbation of renal failure was observed in 3 patients. Our study demonstrates that levofloxacine ni dose 2x500 mg given intravenously for 7 days is effective and safe in treatment of CAP in patients with previously ineffective antibacterial therapy.

Toxic shock syndrome

Diagnosis For staphylococcal toxic shock syndrome, the diagnosis is based upon CDC criteria defined in 2011, as follows: Body temperature > 38.9 °C (102.02 °F) Systolic blood pressure < 90 mmHg Diffuse macular erythroderma Desquamation (especially of the palms and soles) 1–2 weeks after onset Involvement of three or more organ systems: Gastrointestinal (vomiting, diarrhea) Muscular: severe myalgia or creatine phosphokinase level at least twice the upper limit of normal for laboratory Mucous membrane hyperemia (vaginal, oral, conjunctival) Kidney failure (serum creatinine > 2 times normal) Liver inflammation (bilirubin, AST, or ALT > 2 times normal) Low platelet count (platelet count < 100,000 / mm3) Central nervous system involvement (confusion without any focal neurological findings) Negative results of: Blood, throat, and CSF cultures for other bacteria (besides S. aureus) Negative serology for Rickettsia infection, leptospirosis, and measles


Porphyria Cutanea Tarda: 60% of patients with PCT are male, many of them drink alcohol in excess, women who develop it are usually treated with drugs containing estrogens. Most are males with signs of iron overload, this overload reduces the activity of the enzyme uroporphyrinogen decarboxylase, which leads to the elevation of uroporphyrins. HCV and HIV infections have been implicated in the precipitation of acquired PCT. There is a hereditary form with AD pattern. Patients with PCT present with blistering of photoexposed skin, most frequently on the dorsum of the hands and scalp. In addition to fragility, they may develop hypertrichosis, hyperpigmentation, cicatricial alopecia and sclerodermal induration.

A 62-year-old man with a history of significant alcohol abuse, carrier of hepatitis C virus, treated with Ibuprofen for tendinitis of the right shoulder, goes to his dermatologist because after spending two weeks on vacation at the beach he notices the appearance of tense blisters on the dorsum of his hands. On examination, in addition to localization and slight malar hypertrichosis.  The most likely diagnosis is:

Relationship between porphyria cutanea tarda (PCT) and viral hepatitis.

Recent reports have revealed the high prevalence of serological markers of viral hepatitis in porphyria cutanea tarda (PCT). We present two cases of PCT associated with hepatitis C and discuss the relationship between PCT and viral hepatitis. Case 1: A 50-year-old Japanese male noticed blisters, erosions, and fragility on sun-exposed areas of his skin in November of 1990. He had no history of excessive alcohol intake. He had been taking analgesics for eighteen years. Case 2: A 64-year-old Japanese male was referred in October of 1989 because of pigmentation on sun-exposed areas of his skin. He had been drinking alcohol excessively for 43 years. The hepatitis C virus (HCV) antibody was present in each case. Tests for the HCV antibody and hepatitis B serological markers were run in 5 other patients. HCV antibody was present in 3 of them. The two cases negative for the HCV antibody exhibited the hepatitis B antibody. We speculated that viral hepatitis infection may play an important role in precipitating PCT in cases with a history of a long term excessive intake of alcohol or chemicals.

Familial and sporadic porphyria cutanea tarda: clinical and biochemical features and risk factors in 152 patients.

Porphyria cutanea tarda is the most frequent porphyria and occurs in both sporadic and familial forms. We conducted the current study in a series of 152 consecutive patients with porphyria cutanea tarda attending the Porphyria Unit of the Hospital Clinic of Barcelona, Spain, to update the clinical manifestations of the disease and to study the sex differences, the proportion of familial forms, and the role of different risk factors in this population. Patients were classified as familial and sporadic cases according to erythrocyte uroporphyrinogen-decarboxylase activity and uroporphyrinogen-decarboxylase genotyping. In our cohort, skin fragility and blisters on the hands were the most frequent clinical manifestations. Women more frequently had facial hypertrichosis (84.8%; p = 0.004), affected areas other than the hands and face (33.3%; p = 0.008), and pruritus (27.3%; p = 0.041) compared with men. Of our patients, 11.8% did not present the typical clinical onset of the disease, with facial hypertrichosis and hyperpigmentation the more frequent complaints in these cases. Analysis of risk factors showed a high prevalence of hepatitis C virus infection (65.8%) and alcohol abuse (59.9%), both being more frequent in men (p < 0.001). Hepatitis C virus infection was the only risk factor that showed differences between the sporadic and familial forms in the logistic regression model (odds ratio, 0.05; 95% confidence interval, 0.006-0.46). In conclusion, atypical forms of presentation of porphyria cutanea tarda should be considered in order to prevent delayed diagnosis. We note the sustained role of hepatitis C virus infection in the precipitation of sporadic porphyria cutanea tarda. Therefore, in countries with a high prevalence of hepatitis C virus infection, the absence of such infection in a patient with porphyria cutanea tarda may suggest a possible familial case.

Hepatitis C- and HIV-induced porphyria cutanea tarda.

Male, 47 FINAL DIAGNOSIS: Porphyria cutanea tarda Symptoms: Chills • cough dry • thumb swelling - Clinical Procedure: - Specialty: Metabolic Disorders and Diabetics. Challenging differential diagnosis. Porphyria cutanea tarda (PCT) is the most common type of the porphyria. It occurs due to the deficiency of enzyme uroporphyrinogen decarboxylase (UROD), which is the fifth enzyme in the biosynthesis of heme and catalyzes the conversion of uroporphyrinogen to coproporphyrinogen. The risk factors for PCT include hereditary hemochromatosis, hepatitis C infection, ethanol abuse, estrogen use, HIV, smoking, chlorinated polycyclic aromatic hydrocarbons, and hemodialysis. A 47-year-old Hispanic man presented with right thumb swelling, redness, and pain for approximately 1 week. Past medical history included HIV/AIDS, hepatitis C infection, alcohol abuse, heroin abuse, and CMV retinitis. Skin examination revealed blistering and hypo/hyper pigmented lesions over the dorsal aspects of the hands and other sun-exposed areas. Serum porphyrins were discovered to be elevated. The quantitative urine porphyrins revealed elevation of uroporphyrins, heptacarboxyl-porphyrins, hexacarboxy-porphyrins, pentacarboxyl-porphyrins and coproporphyrin. Genetic mutation of UROD was not detected. Due to the classic cutaneous lesions, laboratory findings, and associated risk factors, we were able to confirm our suspicion of the sporadic (type 1) form of PCT. A strong correlation has been demonstrated between the sporadic (type 1) form of PCT and hepatitis C virus (HCV) infection in multiple studies. The mechanism through which HCV infection may cause or trigger PCT is unknown. PCT has been described for many years, but still eludes the differential diagnosis in a patient with cutaneous findings. The uniqueness of our case is the possibility that combined risk factors have an effect on PCT.

Porphyria cutanea tarda. Don't forget to look at the urine.

Diagnosis of porphyria cutanea tarda is usually fairly straightforward because of the characteristic clinical findings. Blisters and erosions develop acutely on sun-exposed skin, sometimes accompanied by hypertrichosis, abnormal pigmentation, and milia formation. Iron stores are usually elevated, and liver transaminases and blood glucose levels are often above normal as well. Gross examination of the urine can provide a valuable clue, since urine of porphyria cutanea tarda patients is red to brown in natural light and pink to red in fluorescent light. Biopsy of a bullous lesion is useful to rule out other diseases. Confirmation of porphyria cutanea tarda requires measurement of porphyrin levels in a 24-hour urine collection. Once the diagnosis is confirmed, it appears reasonable to screen all patients with porphyria cutanea tarda for hepatitis C and possibly B, but especially those less than 30 years old who have extremely high liver transaminase levels. Therapeutic measures for porphyria cutanea tarda include avoidance of exacerbating factors, especially ultraviolet light, ethanol, and certain medications. Phlebotomy or chloroquine therapy is reserved for patients in whom conservative measures fail.

[Sclerodermatous changes in porphyria cutanea tarda: six cases].

The clinical features of porphyria cutanea tarda (PCT) are usually distinctive and include blistering on sun-exposed areas, fragile skin, hypertrichosis and hyperpigmentation. Sclerodermatous changes are much less common, and may either reveal PCT or else appear later. We carried out a retrospective study of the files of six female patients presenting such lesions. Six women (age: 45 to 72 years) were referred for sclerodermatous lesions on sun-exposed areas of the upper body. In four patients, these lesions revealed PCT and in the remaining two patients they were indicative of previously treated but relapsing PCT. Four had sclerodermatous skin changes mimicking morphea of the neck and neckline, the top of the back and the face, while one presented more diffuse facial and cervical sclerosis. Associated alopecia was seen in three patients. The last patient presented isolated sclerodermiform alopecia. Associated malar hypertrichosis was seen in five cases and facial hyperpigmentation was noted in three cases. Four exhibited no blisters, cutaneous fragility, milia or photosensitivity. Histological findings were consistent with morphea or scleroderma in all cases. All patients presented abnormal liver tests: cirrhosis was present in four cases (primitive biliary cirrhosis, alcoholic cirrhosis and hepatitis C) and fatty liver in two cases. In four cases, there was excessive alcohol intake. Uroporphyrin levels were above the normal range in all cases. Local corticosteroid therapy associated with phlebotomy and/or low-dose hydroxychloroquine resulted in complete normalisation of porphyrin levels in four patients, with complete resolution of the cutaneous lesions in two patients and partial improvement in the other two. Sclerodermatous changes are uncommon in PCT. They are not always late and secondary to the process of healing of blisters but can in fact constitute the first cutaneous symptom of the disease while revealing the underlying liver disease. Even in the absence of blisters, photosensitivity or cutaneous fragility, a diagnosis of PCT must be suspected in a setting of sclerodermatous changes distributed on the neck and face, or the neckline, or scarring alopecia, if associated with abnormal liver tests. Skin biopsy to confirm the diagnosis of scleroderma may delay the diagnosis, which is in fact based on porphyrin level. Normalization of the latter parameter under treatment allows regression of lesions.

Biochemistry_Lippinco

1.2. A 50-year-old man presented with painful blisters on the backs of his hands. He was a golf instructor and indicated that the blisters had erupted shortly after the golfing season began. He did not have recent exposure to common skin irritants. He had partial complex seizure disorder that had begun ~3 years earlier after a head injury. The patient had been taking phenytoin (his only medication) since the onset of the seizure disorder. He admitted to an average weekly ethanol intake of ~18 12-oz cans of beer. The patient’s urine was reddish orange. Cultures obtained from skin lesions failed to grow organisms. A 24-hour urine collection showed elevated uroporphyrin (1,000 mg; normal, <27 mg). The most likely diagnosis is: A. acute intermittent porphyria. B. congenital erythropoietic porphyria. C. erythropoietic protoporphyria. D. hereditary coproporphyria. E. porphyria cutanea tarda.

Total Corneal Melt in Patient with Porphyria Cutanea Tarda in Presence of Another Risk Factor.

<iPurpose</i: To report a case of two siblings affected by porphyria cutanea tarda (PCT) presenting with varying severity of ocular disease and evaluation of difference in the presence of risk factors. <iMethods</i: Case report <iResult</i: A 42-year-old Indian male diagnosed with PCT and history of daily intake of alcohol, presented with sudden decrease in vision of right eye. Examination showed perforated cornea surrounded by rim of 360-degree avascular necrotic sclera, with no evidence of infection. Similar systemic history was present in his elder brother. He did not consume alcohol and presented with mere localized scleral thinning. Since the patient's sibling acted as a natural control for the evaluation of alcohol as an independent risk factor, the difference in severity of disease can be explained on the basis of difference in alcohol intake. <iConclusion</i: In the presence of progression of the disease, other risk factors like intake of alcohol should be evaluated.

Porphyria Cutanea Tarda Associated With Acute Hemorrhagic Pancreatitis.

Porphyria cutanea tarda (PCT) is a condition of dysregulated heme synthesis that leads to accumulation of photosensitizing precursors with resultant fragility and blistering of the skin. It can be hereditary or acquired and has been known to be associated with hepatic C virus, alcohol, HIV, and estrogen. In this article, we report an unusual presentation of PCT associated with acute hemorrhagic pancreatitis in a 57-year-old man. He presented initially to a community hospital with acute onset of epigastric abdominal pain and new-onset ascites. Lipase was elevated. Diagnostic paracentesis was grossly bloody. He was then transferred to our institution for concern for acute hemorrhagic pancreatitis. On arrival, physical examination demonstrated vesicles and bullae with erythematous bases, in different stages of healing seen over the dorsal aspects of both hands with scaling, scarring, and hypopigmentation and hyperpigmentation of the skin. Laboratory evaluation and skin biopsy confirmed the diagnosis of PCT. Search for an underlying etiology failed to reveal typical predisposing factors. This report illustrates that acute hemorrhagic pancreatitis may be an underlying etiology for PCT.

Porphyria cutanea tarda in a human immunodeficiency virus-infected patient: A rare scenario in India.

A 30-year-old unmarried, heterosexual male presented with an 8-month history of tense blistering skin lesions over the hands. Physical examination revealed facial hypertrichosis and multiple erosions with crusts and scars over the dorsum of both hands. Woods lamp examination of the urine, histopathology and urinary porphyrin levels were suggestive of porphyria cutanea tarda (PCT). The patient responded well to hydroxychloroquine and antiretroviral drugs. This case report calls for a detailed evaluation and HIV testing in every patient with PCT.

A case of cutaneous squamous cell carcinoma associated with sporadic porphyria cutanea tarda due to liver disorder after Schistosoma japonicum infection.

A 56-year-old man suffered from cutaneous squamous cell carcinoma (cutaneous SCC) which occurred on a cutaneous lesion of sporadic porphyria cutanea tarda (sporadic PCT). His liver function decreased from the time he was infected with Schistosoma japonicum at the age of 10. He drank a little alcohol. Erythematous maculae with blisters or erosions occurred on sun-exposed areas of his skin when he was 52. His urine continued to be red. After detailed examinations including liver biopsy, he was diagnosed as having sporadic PCT due to liver disorder after infection with Schistosoma japonicum. At the age of 56, a small red papule arose on his right earlobe at the site of a sporadic PCT lesion. The papule rapidly enlarged with ulceration; this completely destroyed his right earlobe, which was covered with odoriferous yellow-white necrotic tissue. The tumor then extended to his right preauricular area with ulceration. A skin biopsy confirmed well-differentiated cutaneous SCC. The association of cutaneous SCC with sporadic PCT has not been previously reported; we think that the association is significant. Such an occurrence may have been induced by either direct or indirect effects of ultraviolet light or a scar formed by the sporadic PCT.

Porphyria cutanea tarda in human immunodeficiency virus-seropositive men: case report and literature review.

Porphyria cutanea tarda (PCT) is a disorder of heme synthesis characterized by (a) a diminished activity of uroporphyrinogen decarboxylase biochemically and (b) cutaneous lesions secondary to a delayed type of photosensitivity clinically. A human immunodeficiency virus (HIV)-infected patient with PCT is reported and the world literature is reviewed. To date, 17 HIV-seropositive men with PCT have been described. The initial appearance of PCT occurred before or concurrent with the diagnosis of HIV infection in 71% of these individuals (12 men). The median age at onset of PCT was 36 years (range of 20 to 69 years); the median age for the detection of HIV infection was 35 years (range of less than 20 to 71 years). All of these patients had elevated levels of urine porphyrins and blisters on their dorsal hands. Abnormal liver function tests, erosions, hyperpigmentation, hypertrichosis, and skin fragility were also present in some of the men. Polycythemia, serologic evidence of increased iron stores, scarring, milia, and sclerodermoid changes were rarely observed. Successful therapeutic approaches for PCT in men with HIV infection included (a) elimination of PCT-precipitating agents, (b) avoidance of sun exposure, and (c) periodic phlebotomy. Multiple hypotheses for an etiologic role of the HIV and/or an HIV-associated infection, directly or indirectly, in the pathogenesis of PCT in HIV-seropositive men have been suggested.

Photosensitivity: results of investigation in 250 patients.

Between January 1972 and December 1974, 250 patients were referred for investigation of possible photosensitivity to a university-associated clinical research unit for photobiology. In addition to an appropriate history and clinical examination, phototesting was carried out with a solar simulator, monochromatic light and fluorescent light directed to patch-tested areas of skin. Photosensitivity was not demonstrated in 110 patients (44%). Among the laboratory-confirmed photosensitivities, diagnoses included polymorphous light eruption (4.4%), erythropoietic protoporphyria (3.6%), porphyria cutanea tarda (2%), photoallergic contact dermatitis (5.6%), persistent light reaction (4%),  systemic drug phototoxicity (1.2%), phototoxic contact dermatitis (2%), solar urticaria (0.8%) and photoaggravated dermatoses (7.6%). It is important to establish a precise etiologic diagnosis in patients with photosensitivity in order to treat the disorder specifically or effectively or both.

Diet and alcohol effects on the manifestation of hepatic porphyrias.

Porphyria cutanea tarda (PCT) is the most frequently reported type of porphyria. The average patient is male more than 40 years old with a history of alcohol consumption. In women the incidence of PCT has increased with use of estrogens for birth control. The cutaneous features are those of chronic porphyrin photosensitivity on the light-exposed area of the skin: pigmentation, hirsuitism and fragility, and vesiculobullae, which has prompted the expression bullosa actinica et mechanica. One-third of the patients have glucose intolerance. PCT has been reported frequently among the Bantu people in South Africa as resulting from combinations of alcohol and cooking in ironware. The average patient has a higher than normal hematocrit, which is used as a guide to treatment by phlebotomy ranging from 8 to 14 units removed every 2-4 wk. Chemically induced PCT has been reported with chlorinated hydrocarbons, the best-known of which is hexachlorobenzene (HCB). Porphyria was noted in more than 3,000 patients in southeast Turkey between 1955 and 1961, because of consumption of seed wheat treated with HCB. In addition, more than 1,000 children under the age of 1 year died because HCB was transferred from the mother, either via the placenta or through breast milk.

[Porphyria cutanea tarda revealed by voriconazole].

Voriconazole is a systemic antifungal drug that can induce phototoxic reactions suggestive of porphyria cutanea tarda (PCT); however, porphyrin levels in urine, blood and stool remain within the normal range. Superficial cheilitis is frequently associated with this clinical picture; it is believed to be related to drug-induced impairment of endogenous retinoid metabolism. We report a case of true PCT associated with cheilitis, both occurring soon after the introduction of voriconazole and partially disappearing after withdrawal of this drug. A 65-year-old man with a past history of excessive alcohol consumption presented with typical features of PCT associated with a mild superficial desquamating cheilitis. Both symptoms had appeared 12 days after initiation of oral voriconazole for a cavitary aspergillosis. Laboratory tests confirmed a sporadic case of PCT. Withdrawal of voriconazole (replaced by itraconazole) resulted in complete disappearance of the cheilitis but incomplete remission of the PCT. Ultimately, the patient was successfully treated by venous puncture. This patient had both voriconazole-induced superficial cheilitis and a true PCT which seemed related to the same drug. The mechanism by which voriconazole may have revealed PCT remains elusive and could possibly have involved decreased uroporphyrinogen decarboxylase activity in the liver or potentiation of the phototoxic effects of porphyrins by the cutaneous toxicity of voriconazole. On presentation of a clinical picture of PCT-like photosensitivity in a patient on voriconazole, laboratory investigations should be performed routinely to rule out true PCT, even in cases of associated cheilitis.

Porphyria cutanea tarda associated with human immunodeficiency virus infection.

Since 1987, about 60 cases of porphyria cutanea tarda (PCT) associated with human immunodeficiency virus (HIV) have been reported. The respective roles of HIV and toxic hepatic factor in PCT remain unclear. We report 10 new cases and analyse the following toxic hepatic factors: hepatitis C and B, alcoholism, drugs. The route of HIV transmission to these 10 men were: IV drugs abuse (3), homo/bisexuality (4), heterosexuality (1), and unknown (2). When PCT was diagnosed, their average age was 38 years (29-54) and the HIV-infection had been established for 4.8 years (0.33-9). Seven men had HIV-related symptoms and a CD4+ lymphocyte count below 200/mm3. Cutaneous signs and urinary porphyrin count were characteristic. Alcohol abuse was present in 8/10 patients. AST, ALT and/or gamma GT were high in 9/10 patients; 5/10 patients had HCV antibodies (4 were HCV-PCR positive). HBs antigenemia was negative among the 5/8 patients with HBV antibodies; 10/10 patients took prescribed hepatotoxic drugs. Our series confirms the presence of toxic hepatic factors in PCT of HIV-positive patients. Hepatitis C, alcoholism and hepatotoxic drug consumption seem to be triggers for the appearance of PCT in HIV-positive patients.

Naproxen-induced pseudoporphyria presenting a diagnostic dilemma.

A case of clinical porphyria cutanea tarda posed a diagnostic dilemma when screening tests for porphyrinuria gave negative results and two skin biopsy specimens failed to show the histologic picture of porphyria cutanea tarda, pseudoporphyria, or epidermolysis bullosa acquisita. The dilemma was resolved when the eruption cleared following elimination of naproxen from the patient's treatment. Subsequent naproxen challenge resulted in new bullae on the dorsum of the hands.

Hypertrichosis -- History and Physical

Porphyrias such as porphyria cutanea tarda (PCT) and hepatoerythropoietic porphyria (HEP) can present as hypertrichosis within sun-exposed areas. Patients will present with other symptoms of porphyria. The hypertrichosis associated with PCT often favors the lateral face. Patients will present with other stigmata of PCT including a blistering photosensitive eruption. [41]

Prince William of Gloucester

Shortly before transferring to Tokyo in August 1968, Prince William was examined by a Royal Air Force doctor, Headly Bellringer, at the request of the prince's mother. William told the doctor that he had suffered from jaundice, beginning in December 1965 and lasting several months. He had subsequently noticed that his skin was prone to a blistering rash, particularly on exposure to sunshine. Bellringer tentatively diagnosed porphyria, prescribed sunblock cream and gave him a medical warning card regarding the need to avoid certain medications. Although he was aware of the theory of the royal family's history of porphyria then being proposed by Ida Macalpine and Richard Hunter, he stated he "tried not to let it influence him...with all the symptoms, I was left with little option but to diagnose the Prince's condition as porphyria." William was later examined by haematologists at Addenbrooke's Hospital in Cambridge and also by a Professor Ishihara in Tokyo, both of whom also concluded

Clinically, type I and type II PCT are similar, and the need for differentiating the two arises only during the management and patient counseling. The patients should be asked in detail about any of the susceptibility factors discussed above, like alcohol, smoking, chemical, or estrogen exposures. Any evidence of neurovisceral symptoms can help point to other porphyrias. Family history is usually not helpful, and a detailed medication history might be beneficial to rule out any drug-related eruptions. Since the porphyrin accumulation in the PCT can change the urine color to reddish-brown, a complete history will also include questions about the patient's urine color.

Porphyria cutanea tarda

Porphyria cutanea tarda is the disease that both Dabney Pratt and Brother Rush suffer from in Virginia Hamilton's children's novel Sweet Whispers, Brother Rush. References External links Alcohol and health Autosomal dominant disorders Hepatitis C virus-associated diseases Porphyrias Skin conditions resulting from errors in metabolism

Porphyria cutanea tarda affecting lower lip.

In porphyria cutanea tarda, the most common type of porphyria, blisters of the skin occur because of high sensitivity to sunlight. This case report describes porphyria cutanea tarda of the lower lip mimicking an actinic cheilitis in a 62-year-old man with a history of Hodgkin's lymphoma.

HAART: a risk factor for development of porphyria cutanea tarda?

Porphyria cutanea tarda (PCT) is caused by inherited or acquired partial deficiency of the uroporphyrinogen-decarboxylase (Uro-D) enzyme activity. It is the most common form of porphyria. The main triggering factors to the development of porphyria cutanea tarda are alcohol, hepatitis C virus and human immunodeficiency virus. There are several reports of PCT associated with drugs, among them, antiretroviral therapy. We describe three HIV-positive patients, which showed photosensitivity as well as the emergence of tense blisters on sun-exposed areas during the use of highly active antiretroviral therapy (HAART) and discuss the possibility of PCT after the use of these drugs by those patients.

[Sporadic porphyria cutanea tarda: a case report in a Moroccan man].

This report describes a case of sporadic porphyria cutanea tarda involving a 38-year-old Moroccan man. Clinical diagnosis was based on characteristic features, i.e., facial hypertrichosis and bullous lesions lasting four months during the summer of 2000 followed by macular scarring on the dorsal surfaces on the hands. Three well-known precipitating factors were noted, i.e., sun, ethanol and hepatitis C virus infection. Laboratory diagnosis was based on dark red urine and elevated serum and urine uroporphyrin levels. Enhanced uroporphyrin production was due to urodecarboxylase deficiency in the liver. Urodecarboxylase activity in red blood cells and serum ferritin level were normal. The patient is heterozygous for the His63Asp HFE gene mutation associated with hereditary hemochromatosis. The photoprotective effect of melanin in this dark-skinned patient failed to offset uroporphyrin-induced photosensitivity. Avoidance of sun, ethanol and phlebotomy have prevented recurrences.

Porphyria Cutanea Tarda -- Pearls and Other Issues

Porphyria cutanea tarda is the most common porphyria caused by decreased activity in the uroporphyrinogen decarboxylase enzyme, leading to the accumulation of porphyrins. Alcohol, estrogen use, viral infections, smoking, and iron overload are susceptibility factors that are commonly associated with the development of porphyria cutanea tarda. Excessive circulating porphyrins react with light causing a delayed skin reaction manifesting as blisters, hypertrichosis, hyperpigmentation, and scleroderma-like changes on sun-exposed surfaces. Porphyria cutanea tarda may be treated by simply avoiding the precipitating factors. However, phlebotomy and hydroxychloroquine are both effective in achieving biochemical and clinical remission.

Porphyria cutanea tarda

Epidemiology PCT prevalence is estimated at 1 in 10,000. An estimated 80% of porphyria cutanea tarda cases are sporadic. The exact frequency is not clear because many people with the condition never experience symptoms and those that do are often misdiagnosed with anything ranging from idiopathic photodermatitis and seasonal allergies to hives. Society and culture Porphyria cutanea tarda is implicated in the origin of vampire myths. This is because people with the disease tend to avoid the sun due to photosensitivity and may develop disfigurement that eats away their noses, eyelids, lips, and gums giving their teeth a fang-like appearance. It has also been suggested they may have developed a craving for healthy blood to replace their own in a self medicated treatment in prior centuries.

Porphyria Cutanea Tarda -- History and Physical -- Cutaneous Manifestations

Photosensitivity, along with the formation of skin blisters, vesicles, bullae, and increased fragility of the skin, is seen on sun-exposed parts of the body. This most commonly involves the back of the hands, forearms, neck, face, and feet. These repeated insults to the skin later resolve, leaving behind hyper- and hypopigmented scars and milia, which are small yellowish-white keratin filled papules. [10] Since PCT is delayed blistering photosensitivity, most patients do not associate sun exposure with their skin lesions. [4] These skin lesions arise from minor trauma and sometimes may get infected, causing intense pain and inflammation. [18] The scarring may lead to contractures mimicking the skin findings of a patient with scleroderma, thus labeling this phenomenon as "pseudoscleroderma." [19] Scarring alopecia on the scalp can lead to a diffuse hair loss.

The management of porphyria cutanea tarda.

Porphyria cutanea tarda (PCT), the commonest of all porphyrias, is usually characterized by blisters and fragility of skin in light-exposed areas. It can be clinically indistinguishable from other disorders including variegate porphyria and the diagnosis can only be made by rigorous biochemical analysis. PCT does not cause acute attacks of porphyria. It is usually an acquired condition caused by inhibition of the uroporphyrinogen decarboxylase enzyme in the liver. Hereditary haemochromatosis, hepatitis C virus infection, alcohol, oestrogens and a family history of PCT are the major risk factors for the condition and should be searched for specifically in all patients. Liver disease, including hepatocellular carcinoma, is common in patients with PCT, and should be investigated for at presentation by means of a liver biopsy where possible. Patients with severe hepatic pathology or longstanding untreated PCT need to be monitored for the development of hepatocellular carcinoma in the long term. Low dose twice weekly chloroquine is the mainstay of treatment, but venesection should be used in patients with severe iron overload or hepatitis C-related liver disease. Subsequently, long-term follow-up is needed in all patients to monitor for relapse.

The sensitivity and specificity of "caterpillar bodies" in the differential diagnosis of subepidermal blistering disorders.

Caterpillar bodies are eosinophilic, elongated, segmented bodies located within the roofs of blisters and are considered to represent a specific histopathologic feature of porphyric bullous eruptions, including porphyria cutanea tarda and erythropoietic protoporphyria. The possibility that similar or identical bodies may be present in other disorders prompted further study exploring the specificity of caterpillar bodies in the differential diagnosis of subepidermal vesiculobullous disorders. Seventy-six cases exhibiting subepidermal clefting were reviewed by light microscopy. "Classic" caterpillar bodies were present in porphyria cutanea tarda (6 of 14) and 1 case representing either venous stasis-associated bulla or possibly bullosis diabeticorum. Caterpillar body-like clusters were present in porphyria cutanea tarda (5 of 14), erythropoietic protoporphyria (1 of 3), bullous pemphigoid (7 of 24), and junctional or dystrophic epidermolysis bullosa (3 of 5). In some cases, classic caterpillar body and caterpillar body-like clusters coexisted in a blister roof. Caterpillar bodies or caterpillar body-like clusters were not identified in any cases of dermatitis herpetiformis, linear IgA bullous dermatosis, bullous erythema multiforme, epidermolysis bullosa acquisita, or wound-healing reactions. In contrast to caterpillar bodies, caterpillar body-like clusters were negative for PAS or Type IV collagen stains. In conclusion, caterpillar bodies appear to represent a specific feature of porphyria cutanea tarda (specificity, 98%) but were present in less than half of those cases in this study (sensitivity, 43%). Caterpillar body-like clusters are less specific and may be present in porphyria cutanea tarda, erythropoietic protoporphyria, pseudoporphyria, bullous pemphigoid, epidermolysis bullosa, and epidermolysis bullosa acquisita.

List of MeSH codes (C16)

– skin diseases, genetic – albinism – albinism, ocular – albinism, oculocutaneous – Hermansky–Pudlak syndrome – piebaldism – cutis laxa – dermatitis, atopic – dyskeratosis congenita – ectodermal dysplasia – Ellis–van Creveld syndrome – focal dermal hypoplasia – neurocutaneous syndromes – Ehlers–Danlos syndrome – epidermolysis bullosa – epidermolysis bullosa dystrophica – epidermolysis bullosa, junctional – epidermolysis bullosa simplex – ichthyosiform erythroderma, congenital – hyperkeratosis, epidermolytic – ichthyosis, lamellar – ichthyosis vulgaris – ichthyosis, x-linked – incontinentia pigmenti – keratoderma, palmoplantar – keratoderma, palmoplantar, diffuse – Papillon–Lefèvre disease – keratosis follicularis – pemphigus, benign familial – porokeratosis – porphyria, erythropoietic – porphyrias, hepatic – coproporphyria, hereditary – porphyria, acute intermittent – porphyria cutanea tarda

1599: first iconographic description of hepatoerythropoietic porphyria.

The author's aim was to contribute information to the history of porphyrias through the analysis of a 16th century portrait. The subject drawn by physician Aldrovandi is a 20-year-old girl showing remarkable facial hypertrichosis, while her body is described as hairless. After a brief excursus through the history of porphyrias, the author revisited a previous diagnosis of hypertrichosis lanuginosa with the aid of current clinical findings. Accurate research of the drawings by the 16th century physician together with the study of our case's family history have supplied information on the young woman affected by hirsutism, such as the absence of hair on her body surface. Careful observation together with updated knowledge resulted in a diagnosis of hepatoerythropoietic porphyria; a disease characterized by severe facial hypertrichosis, a hairless body, and very early onset and heredity.

Porphyria Cutanea Tarda -- Deterrence and Patient Education

Patients should be educated about the entire disease process and reassured about the non-fatality of this condition. Phlebotomy should be explained along with the need for regular appointments to check porphyrin levels in the blood. Avoidance of susceptibility factors like cessation of smoking, alcohol, estrogen, and iron therapies along with the importance of sun-protective measures like sunscreens, hats, and protective clothing should be emphasized. Any prior history of HCV or HIV should be disclosed, and the patient should strictly adhere to the treatment regimens advised by the treating physician. Testing for ferritin levels, infections, and genetic testing are all part of the treatment strategy for PCT, and the patient should be made aware of this.

Hepatocellular carcinoma

Acute and chronic hepatic porphyrias (acute intermittent porphyria, porphyria cutanea tarda, hereditary coproporphyria, variegate porphyria) and tyrosinemia type I are risk factors for hepatocellular carcinoma. The diagnosis of an acute hepatic porphyria (AIP, HCP, VP) should be sought in patients with HCC without typical risk factors of hepatitis B or C, alcoholic liver cirrhosis, or hemochromatosis. Both active and latent genetic carriers of acute hepatic porphyrias are at risk for this cancer, although latent genetic carriers have developed the cancer at a later age than those with classic symptoms. Patients with acute hepatic porphyrias should be monitored for HCC.