en POLSKI
eISSN: 2084-9834
ISSN: 0034-6233
Reumatologia/Rheumatology
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6/2017
vol. 55
 
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abstract:
Case report

A child with subcorneal pustular dermatosis responded to IVIG treatment (Sneddon-Wilkinson disease)

Selcen Kundak
,
Özlem Bağ
,
Nesrin Gülez
,
Malik Ergin

Reumatologia 2017; 55, 6: 323-327
Online publish date: 2017/12/30
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Subcorneal pustular dermatosis (SPD) is a rare, chronic, recurrent dermatosis characterised by sterile pustules. It develops mainly in middle-aged or elder women, but is also rarely seen in children. The exact aetiology of the disease is unknown. In literature, cases associated with IgA gammopathy have been reported. In this article; we report a case of a five-year-old girl who was diagnosed as SPD by clinical features, histopathological characteristics, and direct immunofluorescence analysis results. IgA was high, and IgG-IgM and CD19+ B cell were low. We noticed that during IVIG treatment for immunodeficiency, dermatological symptoms were recovered rapidly. Clinical profile of SPD and its association with systemic diseases may provide early detection of immune dysfunction.
keywords:

subcorneal pustular dermatosis, Sneddon-Wilkinson disease, subcorneal dermatosis







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