A Case of Immunoglobulin A Pemphigus: Intraepidermal Neutrophilic Dermatosis Type
- Author:
Ji Hye HEO
1
;
Hee Seong YOON
;
Si Hyub LEE
;
Seung Dohn YEOM
;
Lucia KIM
;
Soo Chan KIM
;
Ji Won BYUN
;
Jeonghyun SHIN
;
Gwang Seong CHOI
Author Information
1. Department of Dermatology, Inha University School of Medicine, Incheon, Korea. garden@inha.ac.kr
- Publication Type:Case Report
- Keywords:
IgA pemphigus;
Intercellular IgA dermatosis;
Intraepidermal neutrophilic IgA dermatosis;
Subcorneal pustular dermatosis
- MeSH:
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
- From:Korean Journal of Dermatology
2019;57(8):492-495
- CountryRepublic of Korea
- Language:English
-
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.
