<abstract xmlns="http://www.w3.org/1999/xhtml"><p>IgA pemphigus (IGAP) is a rare autoimmune bullous disease characterized by IgA deposits on keratinocyte cell surfaces. The IGAP is classified into: 1) subcorneal pustular dermatosis (SPD) type, and 2) intraepidermal neutrophilic (IEN) IgA dermatosis type. So far, only 9 children with IGAP have been described in the literature, of whom only 3 with SPD type. We report a 3-year-old boy with SPD type of IGAP. Clinically, he presented with pruritic vesicles, pustules and erosions on the face, trunk, groin area, and extremities. Histopathology showed subcorneal pustules containing a few acantholytic cells. Direct immunofluorescence (DIF) test of Tzanck smear showed intercellular IgA deposits on the surface of the groups of epidermal cells. Oral dapsone and prednisone induced remission after two weeks; the treatment was discontinued 11 months later, and complete remission was achieved during 19 months without any treatment. Direct immunofluorescence of Tzanck smear is a simple, sensitive, rapid and non-aggressive test, very suitable for the diagnosis of IGAP in children.</p></abstract>

IgA pemphigus (IGAP) is a rare autoimmune bullous disease characterized by IgA deposits on keratinocyte cell surfaces. The IGAP is classified into: 1) subcorneal pustular dermatosis (SPD) type, and 2) intraepidermal neutrophilic (IEN) IgA dermatosis type. So far, only 9 children with IGAP have been described in the literature, of whom only 3 with SPD type. We report a 3-year-old boy with SPD type of IGAP. Clinically, he presented with pruritic vesicles, pustules and erosions on the face, trunk, groin area, and extremities. Histopathology showed subcorneal pustules containing a few acantholytic cells. Direct immunofluorescence (DIF) test of Tzanck smear showed intercellular IgA deposits on the surface of the groups of epidermal cells. Oral dapsone and prednisone induced remission after two weeks; the treatment was discontinued 11 months later, and complete remission was achieved during 19 months without any treatment. Direct immunofluorescence of Tzanck smear is a simple, sensitive, rapid and non-aggressive test, very suitable for the diagnosis of IGAP in children.

IgA Pemphigus in a Child – a Case Report

Serbian Journal of Dermatology and Venereology

This work is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 3.0 License.

{"article-title":"IgA Pemphigus in a Child \u2013 a Case Report"}

IgA pemphigus (IGAP) is a rare autoimmune bullous disease characterized by IgA deposits on keratinocyte cell surfaces. The IGAP is classified into: 1)...

IgA Pemphigus in a Child – a Case Report

Published Online: Sep 23, 2017

Page range: 14 - 21

DOI: https://doi.org/10.1515/sjdv-2017-0003

Keywords
Immunoglobulin A, Pemphigus, Child, Preschool, Signs and Symptoms, Diagnosis, Cytodiagnosis, Skin Tests, Dapsone, Prednisone, Treatment Outcome

© 2017 Branislav Lekić et al., published by De Gruyter Open

This work is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 3.0 License.

IgA Pemphigus in a Child – a Case Report

Published Online: Sep 23, 2017

Page range: 14 - 21

DOI: https://doi.org/10.1515/sjdv-2017-0003

KeywordsImmunoglobulin A, Pemphigus, Child, Preschool, Signs and Symptoms, Diagnosis, Cytodiagnosis, Skin Tests, Dapsone, Prednisone, Treatment Outcome

© 2017 Branislav Lekić et al., published by De Gruyter Open

This work is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 3.0 License.

Keywords
Immunoglobulin A, Pemphigus, Child, Preschool, Signs and Symptoms, Diagnosis, Cytodiagnosis, Skin Tests, Dapsone, Prednisone, Treatment Outcome