IGA LINEAR DERMATOSIS OF CHILDHOOD IN MAL I

Introduction. Bullous IgA linear dermatosis is relatively frequent in children in Africa. The aim of this study was to evaluate the frequenc y of this disease among autoimmune bullous diseases in children in Mal i. Patients and methods. Children with chronic bullous disease seen at the Marchoux Institute in Bamako and for whom direct immunofluorescen ce of the perilesional skin demonstrated immunoglobulin and/or complem ent deposits were included in the study population. Results. Twelve ch ildren were included. Direct immunofluorescence showed linear IgA depo sit along the dermo-epidermal junction in 10, once with a pemphigoid a spect and once with a pemphigus aspect. The skin lesions in the childr en with linear IgA deposits were stereotypic: disseminated bullae, ros ettes (9 out of 10), involving the external genital organs (10/10), th e limbs, the face and the trunk. Histology showed subepidermal bullae (10/10), with neutrophil papillar abscesses (9/10). Indirect immunoflu orescence of the cleaved skin NaCl in 5 patients showed that the cleav age always lied al the roof of the cleavage. All the children responde d to dapsone (1 to 2.5 mg/kg/d) which gave complete (7/10), or incompl ete remission. Local corticosteroids were used in 2 patients and gener al corticosteroids in 1. Discussion. This confirms the frequency of bu llous ISA linear dermatosis in Africa, in contrast with uncommon derma titis herpetiformis. Diagnosis is facilitated with immunofluorescence. The low cast of this test makes it a useful diagnostic tool in these countries.