Korean J Dermatol.  2019 Sep;57(8):492-495.

A Case of Immunoglobulin A Pemphigus: Intraepidermal Neutrophilic Dermatosis Type

Affiliations
  • 1Department of Dermatology, Inha University School of Medicine, Incheon, Korea. garden@inha.ac.kr
  • 2Department of Pathology, Inha University School of Medicine, Incheon, Korea.
  • 3Department of Dermatology, Gangnam Severance Hospital, Seoul, Korea.

Abstract

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 half-and-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.

Keyword

IgA pemphigus; Intercellular IgA dermatosis; Intraepidermal neutrophilic IgA dermatosis; Subcorneal pustular dermatosis

MeSH Terms

Adolescent
Antibodies
Biopsy
Blister
Colchicine
Epidermis
Female
Fluorescent Antibody Technique, Direct
Follow-Up Studies
Humans
Immunoglobulin A*
Immunoglobulins*
Neutrophils*
Pemphigus*
Skin Diseases*
Skin Diseases, Vesiculobullous
Steroids
Antibodies
Colchicine
Immunoglobulin A
Immunoglobulins
Steroids
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