A 48 year old woman presented with a 10 day history of painful red nodules over her face, trunk, and limbs which rapidly enlarged before developing central ulceration. She was otherwise well with no complaints. She had received four days of oral flucloxacillin but otherwise was on no medication. There was no past medical history of note. Examination revealed deep cutaneous ulcers with purplish undermined edges and slough at the ulcer bases (fig 1). Ulcer swabs and blood cultures failed to grow any organisms. Chest radiography, full blood count, and biochemical profile were normal, but the C reactive protein was markedly raised at 380 mg/l. Complement C3 and C4 levels and IgG, IgA, and IgM titres were normal. Antinuclear antibodies, DNA antibodies (single and double strand), and neutrophil cytoplasmic antibodies were negative. Neutrophil function tests were normal (as assessed by respiratory burst chemiluminescence). A skin biopsy showed a predominantly neutrophilic infiltrate of the dermis with a leucocytoclastic vasculitis. She was treated with 14 days of flucloxacillin and the ulcers healed over the next month.

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Figure 1

Typical cutaneous lesion (reproduced with permission).

Two years later she developed diarrhoea after a chicken meal. After one week she again developed cutaneous ulcers over her face, trunk, and limbs. She presented six weeks later with weight loss, abdominal pains, multiple skin ulcers, and passing faeces through her vagina. On examination the skin lesions were as before, there was tenderness in the left iliac fossa, and a rectovaginal fistula was present. Inflammatory markers were raised but ulcer, stool, and blood cultures were sterile.