Unusual presentation of cutaneous vasculitis

Pakistan A 12‐year‐old boy from a village near Sukkur presented to the Civil Hospital, Karachi, Pakistan, in January 1992, with widespread black discoloration and ulcers of the lower extremities and focal areas of involvement of the upper extremities and face. The patient was previously healthy, when he suddenly developed low grade fever and severe pain on the left side of the chest for which he was given two unknown white tablets. His fever gradually became high, and next morning he was unable to walk and had erythematous patches on his lower extremities, nose, and posterolateral aspects of the upper extremities. He also developed watery diarrhea without blood or mucus and was incontinent. He was treated with a single dose of cotrimoxazole, furazolidone, and mefenamic acid. Oral rehydration salts were also given. His diarrhea improved, but the erythematous patches started to become black. Other symptoms included frequency and difficulty in passing urine, with only a few drops passed each time. He was hospitalized in Sukkur where oral antimicrobials (erythromycin, clindamycin) and parenteral steroids (dexamethasone, 4 mg, six hourly) were administered. The latter was gradually tapered over 15 days. The urinary symptoms improved after 1 day of hospitalization and treatment. Focal areas of the affected skin of the ears, face, and upper extremities became dry and sloughed off. The lesions were tender. There was no past history of sore throat, jaundice, joint pains, or blood transfusion. He had an episode of left‐sided chest pain 1 year ago for which he was given medication. Five years ago he had a traumatic fracture of the lower leg. On examination, patchy but extensive black discoloration of the feet, lower legs, knees, and posterolateral aspects of the thighs was seen. Large ulcers were present on the buttocks and upper extremities. Ulcers were also seen on the nose, cheeks, lateral aspects of the pinna, upper arms, and elbows ( Figs 1 and 2). His temperature was 98.6 °F orally, pulse rate was 90/min (regular), respiratory rate was 20/min, and blood pressure was 90/50 mmHg. The patient was non‐toxic, with no apparent systemic involvement on clinical examination. 1Patchy, extensive ulceration and black discoloration of the buttocks, posterolateral aspect of the thighs, knees, lower legs, and feet2Focal ulcers on the face and pinna. Ulceration of the elbow and posterior upper arm with focal black discoloration of the skin In the initial investigations, hemoglobin was 11 g%, erythrocyte sedimentation rate (ESR) was 47 mm (first hour), and leukocytosis was 36,250/mm, –2 3 reverting to normal on clinical improvement. The ESR after rising to 56 mm (first hour) decreased to 15 mm (first hour) with control of the infection. Total serum proteins were 5.1 g/dL with albumin 2.5 g/dL. Pus oozing from the ulcers revealed Pseudomonas aeruginosa and Staphylococcus aureus (coagulase positive) on culture. The anti‐streptolysin (ASO) titer was between 100 and 333 Todd units. C3 was 1.11 g/dL (N 0.7–1.80) and C4 0.64 g/dL (N 1.15–0.7). Studies with normal or negative results included several urinalyses, blood cultures, liver function tests, blood urea nitrogen, serum creatinine, antinuclear antibodies (ANA), anti smooth muscle antibodies, anti mitochondrial antibodies, anti double‐stranded DNA antibodies, cryoglobulins, hepatitis B surface antigen, Coombs test, and chest X‐ray. Gram stains of skin sections were negative for any organisms. Skin biopsy showed ulceration and pustules in the epidermis, with the underlying dermis having multiple areas of severe vasculitis with fibrinoid necrosis in the vessel walls and neutrophil polymorph infiltration. Small‐ and medium‐sized vessels were involved, with no evidence of granuloma. Direct immunofluorescence showed immunoglobulin M (IgM) (focal) and IgG in the vessel walls and at the dermo–epidermal junction and C3 (++) at the dermo–epidermal junction. IgA and fibrin were negative. The patient was given prednisolone, 35 mg orally daily, which was gradually reduced to 20 mg daily. At this dose, new vesicles appeared on the healing skin of the lower legs, but disappeared when the steroid was increased to 35 mg again. Antimicrobials (aminoglycosides, cephalosporins, penicillins, and monobactams) were administered according to microbial culture and sensitivity results for the cutaneous infection with Pseudomonas aeruginosa and Staphylococcus aureus. Healing occurred with auto‐amputation of several digits of the feet. The patient was discharged on azathioprine, 25 mg, and prednisolone, 25 mg, daily. The latter was reduced by 2.5 mg per week to 10 mg (after 6 weeks) on alternate days. It was noted that, on reduction of immunosuppressives, vesicles typically appeared around the ankles. Immunosuppressives were tapered off and completely stopped after 2 years (March 1994). The patient was examined again in February 1997 ( Figs 3 and 4). There was complete remission (for 3 years). 3Focal scarring of face and pinna4Loss of digits, atrophy, and scarring associated with hyperpigmentation and patchy depigmentation