- Author:
Tanya S. Ramas-Uypitching
1
;
Maria Jasmin J. Jamora
2
;
Vanessa Anne C. Bernal
1
;
Julia Erika P. Pangasinan
1
Author Information
- Publication Type:Case Reports
- Keywords: lgA Pemphigus; lntraepidermal neutrophilic dermatosis
- From: Journal of the Philippine Medical Association 2021;99(2):51-54
- CountryPhilippines
- Language:English
-
Abstract:
INTRODUCTION:lgA pemphigus is a rare, chronic,
relapsing, benign group of autoimmune
intraepidermal blistering dermatosis with an
unknown etiology. It is characterized by
significantly pruritic, vesiculopustular lesions that
occur mainly on the trunk and proximal extremities.
Histopathologic and immunofluorescence studies
show intraepidermal blisters and deposition of
immunoglobulin A in the intercellular spaces of the
epidermis, respectively.
CASE REPORT:To our knowledge, we present the first reported pediatric case of lgA pemphigus, intraepidermal neutrophilic type, in an 8-year old Filipino female with a 2-year history of generalized papules and flaccid pustules, some forming an annular pattern. Diagnosis was confirmed by histopathology and direct immunofluorescence. Enzyme-linked immunosorbent assay for Desmoglein 1 was negative. Complete clearance of lesions was achieved with dapsone, colchicine and prednisone. - Full text:lgA pemphigus, intraepidermal neutrophillic.pdf