Linear IgA Bullous Dermatosis of Childhood: A Long Term Study

KANOKVALAI KULTHANAN, M.D.*, THADA PIAMPHONGSANT, M.D.**, RUTSANEE AKARAPHANTH, M.D.**, PREY A KULLAVANIJAYA, M.D.**

Affiliation : * Department of Dermatology, Faculty of Medicine, Siriraj Hospital, Mahidol University, Bangkok 10700, ** Institute of Dermatology, Department of Medical Services, Ministry & Public Health, Bangkok I 0400, Thailand.

AbstractBackground : Linear IgA bullous dermatosis (LAD) of childhood is a rare acquired sub- epidermal blistering disease of young children. Most of the studies were reported from the USA and European countries.
Method : Twelve cases of Thai patients diagnosed as LAD of childhood were analyzed concerning clinical, histopathological, immunopathological findings including treatment responses and courses compared with those of Caucasians. Result: The mean age of onset was 5.1 years. The areas of common involvement were the perioral region, lower abdomen, perineum, buttock, inner thighs and extremities. Histopathology in half of the cases showed features of dermatitis herpetiformjs or bullous pemphigoid. All patients had positive linear lgA band at the basement membrane zone (BMZ) by direct immunofluorescence. Only one patient had positive circulating anti BMZ antibody at the titer of I: I 0. Most patients res- ponded well to dapsone. The mean duration before remission was 1.9 years.
Conclusion : Our study in Thai patients with LAD of childhood produced data similar to previous studies carried out in the Caucasian nations.

Keywords : Linear lgA, Bullous Dermatosis, Childhood

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