Ofuji's papuloerythroderma: a study of 17 cases

In this retrospective study the clinical, laboratory and histological features, treatment methods and disease course in 17 patients with papuloerythroderma (PE) are reviewed. The median age at diagnosis was 72 years and the female/male ratio was 1:4:7. The most common abnormal laboratory findings were eosinophilia and an elevated serum IgE level. Psoralen photochemotherapy (PUVA) and oral prednisolone 10-20 mg daily given in combination or alone were very efficient treatments, while UVB phototherapy in combination with topical steroids was also successful. However, potent topical corticosteroids alone or in combination with oral antihistamines were not effective. The patients were observed for a median of 19 months from diagnosis, three being followed for more than 5 years. Five of the patients relapsed, but only one had multiple relapses, two developed cutaneous T-cell lymphoma (CTCL) and two others had histological features suggestive of that disorder. Six of the patients died, cardiovascular disease being the most common cause. PE is a distinct clinical entity with a polymorphous aetiology which frequently includes an association with CTCL or visceral malignancy. PUVA, oral corticosteroids and UVB in combination with topical corticosteroids appear to be effective therapeutic modalities.