Clinical features of von Zumbusch type of generalized pustular psoriasis in children: a retrospective study of 26 patients in southwestern China

BACKGROUND: Von Zumbusch type of generalized pustular psoriasis is a rare variant of psoriasis in children. It can occur in patients with or without psoriasis vulgaris. OBJECTIVE: The aim of the study was to discuss the precipitating factors, clinical manifestations, laboratory data and therapy of von Zumbusch type of generalized pustular psoriasis in children from southwestern China and to improve the diagnosis and treatment level. METHODS: A retrospective analysis was conducted for inpatients aged 14 years old or less with von Zumbusch type of generalized pustular psoriasis in our department from 2005 to 2014. RESULTS: A total of 26 patients were included, of whom four (15.38%) had previous history of psoriasis vulgaris and one (3.85%) had previous history of psoriasis arthropathica. Mean onset age was 6.90 years. Gender distribution was equivalent. Incidence of the disease in summer and autumn was higher than that in winter and spring. Nineteen (73.08%) cases were triggered by infection, two (7.69%) cases were caused by sudden discontinuation of systemic use of corticosteroid. Twenty-four (92.31%) cases had concomitant fever. The initial lesion manifested as non-follicular sterile pustules on erythema. Sixteen patients responded well to acitretin, 11 to Tripterygium wilfordii Hook F (TwHF), two to cyclosporine, and one to methotrexate. STUDY LIMITATIONS: This study is a retrospective one and the number of cases is small. CONCLUSION: Von Zumbusch type of generalized pustular psoriasis is a rare disease in children, infection is the most common precipitating factor, acitretin is the first-line therapy, traditional Chinese medicine TwHF also can be used.