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KMID : 0363220190570080492
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Korean Journal of Dermatology
2019 Volume.57 No. 8 p.492 ~ p.495
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A Case of Immunoglobulin A Pemphigus: Intraepidermal Neutrophilic Dermatosis Type
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Heo Ji-Hye
Yoon Hee-Seong
Lee Si-Hyub
Yeom Seung-Dohn
Kim Lucia
Kim Soo-Chan
Byun Ji-Won
Shin Jeong-Hyun
Choi Gwang-Seong
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Abstract
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Immunoglobulin A (IgA) pemphigus is a rare variant of an autoimmune bullous disease with IgA antibodies. IgA pemphigus is divided into 2 major subtypes: the subcorneal pustular dermatosis (SPD) type and intraepidermal neutrophilic (IEN) dermatosis type. We documented a case of an 18-year-old woman with recurrent generalized blisters and pustules that were especially severe in the intertriginous areas. Some half-and-half blisters and coalesced pustules in an annular pattern with crusts were simultaneously observed. A biopsy specimen from one of the halfand- half blister lesions showed intraepidermal separation with multiple neutrophils. Direct immunofluorescence staining revealed lace-like intercellular deposition of IgA in the entire epidermis. IgA antibody deposits were also observed in the patient's serum. The eruptions cleared with systemic steroids and colchicine 0.6 mg for 1 week, and the patient remained in partial remission at the 8-month follow-up. Herein, we report a case of IEN-type IgA pemphigus, clinically mimicking SPD with half-and-half blisters.
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KEYWORD
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IgA pemphigus, Intercellular IgA dermatosis, Intraepidermal neutrophilic IgA dermatosis, Subcorneal pustular dermatosis
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