(Last Updated On: October 30, 2018)

Authors: Marianne Isabel A. Sayo, MD, Fatima D.C. Jacinto-Calimag, MD, Ma. Angela M. Lavadia, MD, FPDS MD, FPDS, Elizabeth P. Prieto, MD, FPDS, Milali T. Torres, MD, FPDS

Abstract

Introduction: Linear IgA dermatosis is an autoimmune subepidermal blistering disease presented as tense bullae with a string-of-pearls appearance located most commonly in the trunk. It occurs bimodally with a slight predominance of females. This disease is caused by an antibody deposition leading to complement activation and neutrophil chemotaxis which eventuates in loss of adhesion at the dermo-epidermal junction and formation of a blister.

Case Summary: A 15-year old male presented with a 3-month history of a solitary, clear-fluid pruritic vesicle at the left infra-auricular area. In the interim, there was an increase in the number of lesions described as multiple, well-defined, irregularly-shaped, clear fluid-filled tense and flaccid bullae on the anterior, posterior trunk, and both upper and lower extremities. The patient noted lesions to be pruritic with no accompanying pain nor burning sensation. Histopathology results showed subepidermal blistering disease with unremarkable direct immunofluorescence. The patient was treated with Dapsone 50 mg/tab OD, Prednisone 20 mg/day, Cetirizine 10 mg/tab BID as needed for itch, Clobetasol and petroleum jelly on new and erythematous lesions BID. The patient responded well with improvement of lesions and arrest of spread of disease activity.

Conclusion: Linear IgA dermatosis is a rare immune-mediated blistering disease. An important feature is the presence of continuous linear IgA deposits at the basement membrane zone (BMZ) against heterogenous basement membrane zone antigens however since the direct immunofluorescence is negative, the histopathology result and the response to Dapsone as the first-line treatment which strengthens the diagnosis of this disease.

 

Citation

 

Keywords

Dapsone, Prednison, Clobetasol, Cetirizine

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