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

Abstract

BackgroundVon Zumbusch type of generalized pustular psoriasis is a rare variant of psoriasis in children. It can occur in patients with or without psoriasis vulgaris.ObjectiveThe 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.MethodsA 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.ResultsA 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 limitationsThis study is a retrospective one and the number of cases is small.CONCLUSIONVon 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.

Clinical analysis of generalized pustular psoriasis in Chinese patients: A retrospective study of 110 patients

“…4 , 5 The mean onset age of pediatric patients was 10.2 years, which is slightly higher than the age of 6.9 years that Wei Liu observed in his report. 2 In previous studies, GPP more frequently occurred in women, and Youn et al 6 reported a male:female ratio of 1:2. In this study, the male:female ratio was 2.7:1, which revealed that the proportion of men in our study was considerably higher than that in previous studies.…”

“…Few publications have described the clinical course of GPP in Chinese patients; previous publications include a report in which Wang et al studied the clinical features of GPP in 26 pediatric patients. 2 Despite its heterogeneity, it is important to recognize the overall clinical course of GPP for an accurate prognosis of the disease. In this study, we retrospectively investigated the clinical characteristics, laboratory examinations, and therapeutic and systemic effects of 110 GPP patients treated at the dermatology unit of our hospital over the past 6 years.…”

Generalized pustular psoriasis (von Zumbusch)

“…In a retrospective study of 102 patients with acute GPP, systemic glucocorticoids were considered the trigger in 44% of cases, and abrupt withdrawal of corticosteroids or use of depot corticoids as the only strategy for treating psoriasis or for treating another associated condition seems to be the most frequent situation. 43 …”

Keberhasilan Terapi Kombinasi Menggunakan Metotreksat Injeksi dan Siklosporin pada Psoriasis Pustulosa Generalisata

“…10 Pemeriksaan laboratorium pada pasien PPG dapat ditemukan leukositosis, hipokalsemia, hipoalbuminemia, dan peningkatan laju endap darah (LED). 9,10 Psoriasis pustulosa generalisata tersebut juga membutuhkan terapi yang efektif untuk dapat mencegah komplikasi. 2 Terdapat beberapa pilihan pengobatan sistemik yang merupakan lini pertama pada pengobatan PPG, yaitu MTX, siklosporin dan asitretin.…”