Ofuji's papuloerythroderma: two cases treated with azathioprine

BACKGROUND: Ofuji's papuloerythroderma is a rare disorder, characterized by a generalized pruriginous eruption, sparing the folds. It predominates in the elderly. The pathology is still unknown but associations with lymphoma have been described. Various therapeutic approaches have been tried, most often including local and general corticosteroids and PUVA. OBSERVATION: Two patients aged 71 and 84 years presented red pruriginous macular rash sparing the abdominal folds. Eosinophilia and lymphopenia were observed. Cutaneous biopsies showed dermal lymphocytic and plasmocytic infiltrates with, in one case, eosinophil and neutrophil exocytosis. Clinical, biological and morphological investigations showed no association with other diseases such as cancer or lymphoma. Azathioprine permitted clinical and biological remission in both patients but had to be interrupted because of minor side effects (infection, gastroenterologic disorders) and corticosteroids were introduced in one case. DISCUSSION: We suggest that histological aspects, such as exocytosis, may represent a link between Ofuji's papuloerythroderma and lymphoma. Azathioprine led to clinical and biological improvement in our 2 patients. Because of its adverse effects, it could be proposed as second-line therapy in patients presenting resistance or intolerance to usual treatments.