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Pyoderma gangrenosum and leucocytoclastic vasculitis

Mr J.H. was a 40-year-oId man who presented in 1978 with a 3-year history of a widespread eruption of purpura and stnall ulcerating lesions (0-5-1 cm diameter) on his buttocks and lower limbs (Fig. i). The histology showed an extensive dermal infiltrate with much leucocytoclasis. Red cell extravasation and neutrophils within vessel walls were also evident (Fig. 2). Immunofiuorescence revealed deposition of IgM, A, C3 and fibrin within the vessels. A diagnosis of leucocytoclastic vasculitis was made. Investigations for an underlying cause of his vasculitis were normal. These included immunoglobulins, complement, cryoglobulins, antinuclear factor, rheumatoid factor, protein electrophoresis and chest X-ray. He responded to dapsone (100 mg o.d.) and remained relatively free of lesions for several years. In 19B3 he developed large weeping ulcers (3-15 cm) on his lower limbs. These ulcers were on an oedematous and erythematous base with serpiginous borders and undermined edge (Fig. 3). A clinical diagnosis of pyoderma gangrenosum was made. The biopsy from the edge of an ulcer showed a dermal abscess. Leucocytoclasis, fibrinoid necrosis and vessel thrombosis were clearly seen (Fig. 4). Investigations revealed an IgA kappa band with a serum level of 11 g/ml. There was no Bence-Jones proteinuria and bone marrow examination was normal. In vitro tests of neutrophil and monocyte function were normal. Intradermal tests with saline, trafuril and Candida antigen were negative but tuberculin (purified protein derivative, PPD; i: 1000 dilution) produced an indurated erythema.