Generalized pustular psoriasis in Japan: two distinct groups formed by differences in symptoms and genetic background.

Abstract

A multi-center study for investigating generalized pustular psoriasis was carried out in Japan in order to clarify the prevalence, the etiology, and the standard therapy. Through questionnaires, 541 cases of patients with generalized pustular psoriasis and related disorders were collected, and the history, the precipitating factors, the symptoms and the laboratory findings were analyzed from medical records. The statistic analysis revealed that 902 (+/- 102.2) patients with generalized pustular psoriasis were expected at the time, and that the prevalence of generalized pustular psoriasis in Japan was 7.46/million. Of 541 patients with generalized pustular psoriasis, 208 cases had the recurrent episodes of symptoms with generalized pustules which had been referred to von Zumbusch type pustular psoriasis or to the acute form of generalized pustular psoriasis. Two hundred and eight patients were further subdivided into two groups: one with a preceding history of ordinary psoriasis (pso+ generalized pustular psoriasis