Background: Sneddon-Wilkinson disease (SW) is chronic and benign disease belonging to the heterogeneous spectrum of neutrophilic diseases. It is characterised by flask, superficial, aseptic pustules, mainly involving the trunk, axilla and inguinal folds, favourably responsive to sulfones.
Aim: We report an original case with an atypical clinical presentation and course.
Case report: A 25-year old woman presented with an erythematous pustular eruption that had appeared one week before. Similar flare-ups were reported for 3 years. No drug intake history nor recent infection were reported. Physical examination showed superficial pustules mainly located on the face and limbs, lying on an erythematous skin and sometimes grouped in large plaques with a serpiginous disposition having a characteristic aspect of hypopyon pustule. A specimen taken from the pustules did not show bacterial or fungal infection. Histological examination of a biopsy specimen showed subcorneal pustules with a dermal peri-capillary neutrophilic infiltrate. Direct immuno-fluorescence was negative. No associated diseases were found. The patient was resistant to a 1 month treatment with dapsone (100 mg/day). Improvement was obtained with acitretin (25 mg/day) within 3 months.
Conclusion: Our case has some specificities: cephalic involvement, resistance to dapsone and favourable outcome to a low dose of acitretin.