Masato Mizuashi, Ryuhei Okuyama, Noriko Matsumura, Kentaro Sugisaki, Setsuya Aiba
DOI: 10.2340/00015555-0700

A 56-year-old Japanese man was treated with systemic corticosteroids in a local hospital for a 6-year history of purpuric eruptions affecting the upper and lower eyelids, neck and upper chest. He had frequent relapses of the eruptions, together with fever and knee joint pain. One year previously, he had presented with haemorrhagic diverticula in his colon, which were surgically resected. Two months previously, he had presented with haematuria and proteinuria. Examination revealed a purpuric maculopapular rash with prominent oedema on bilateral upper and lower eyelids, and palpable purpuric macules, 2–5 mm in diameter, scattered on the neck and upper chest (Fig. 1a). Laboratory examinations showed an increased white blood cell count of 9250/mm3 and elevated anti-streptolysin-O titre of 316 mg/dl (normal range 160 mg/dl). Serum IgA was increased (541 mg/dl; normal range 91–391 mg/dl). Liver and renal function tests showed normal values except blood urea nitrogen: 22 mg/dl (normal range 15 mg/dl). Skin biopsy from the upper eyelid revealed massive perivascular cell infiltrate composed of neutrophils in the upper and mid-dermis, which was associated with nuclear dust, necrosis of endothelial cells and fibrinoid degeneration (Fig. 1b). IgA deposition was observed along the small blood vessel walls in the upper dermis by direct immunofluorescence staining. He was treated with infusion of polyclonal immunoglobulin (20 g/day for 5 days) together with tonsillectomy, which led to improvement of his symptoms. However, 4 months after the treatment, oedematous swelling and tenderness developed in the scrotum together with fever.What is your diagnosis? See next page for answer.