INTRODUCTION: Bullous IgA linear dermatosis is relatively frequent in children in Africa. The aim of this study was to evaluate the frequency of this disease among autoimmune bullous diseases in children in Mali. PATIENTS AND METHODS: Children with chronic bullous disease seen at the Marchoux Institute in Bamako and for whom direct immunofluorescence of the perilesional skin demonstrated immunoglobulin and/or complement deposits were included in the study population. RESULTS: Twelve children were included. Direct immunofluorescence showed linear IgA deposit along the dermo-epidermal junction in 10, once with a pemphigoid aspect and once with a pemphigus aspect. The skin lesions in the children with linear IgA deposits were stereotypic: disseminated bullae, rosettes (9 out of 10), involving the external genital organs (10/10), the limbs, the face and the trunk. Histology showed subepidermal bullae (10/10), with neutrophil papillar abscesses (9/10). Indirect immunofluorescence of the cleaved skin NaCl in 5 patients showed that the cleavage always lied at the roof of the cleavage. All the children responded to dapsone (1 to 2.5 mg/kg/d) which gave complete (7/10), or incomplete remission. Local corticosteroids were used in 2 patients and general corticosteroids in 1. DISCUSSION: This confirms the frequency of bullous IgA linear dermatosis in Africa, in contrast with uncommon dermatitis herpetiformis. Diagnosis is facilitated with immunofluorescence. The low cost of this test makes it a useful diagnostic tool in these countries.
INTRODUCTION: Bullous IgA linear dermatosis is relatively frequent in children in Africa. The aim of this study was to evaluate the frequency of this disease among autoimmune bullous diseases in children in Mali. PATIENTS AND METHODS: Children with chronic bullous disease seen at the Marchoux Institute in Bamako and for whom direct immunofluorescence of the perilesional skin demonstrated immunoglobulin and/or complement deposits were included in the study population. RESULTS: Twelve children were included. Direct immunofluorescence showed linear IgA deposit along the dermo-epidermal junction in 10, once with a pemphigoid aspect and once with a pemphigus aspect. The skin lesions in the children with linear IgA deposits were stereotypic: disseminated bullae, rosettes (9 out of 10), involving the external genital organs (10/10), the limbs, the face and the trunk. Histology showed subepidermal bullae (10/10), with neutrophil papillar abscesses (9/10). Indirect immunofluorescence of the cleaved skin NaCl in 5 patients showed that the cleavage always lied at the roof of the cleavage. All the children responded to dapsone (1 to 2.5 mg/kg/d) which gave complete (7/10), or incomplete remission. Local corticosteroids were used in 2 patients and general corticosteroids in 1. DISCUSSION: This confirms the frequency of bullous IgA linear dermatosis in Africa, in contrast with uncommon dermatitis herpetiformis. Diagnosis is facilitated with immunofluorescence. The low cost of this test makes it a useful diagnostic tool in these countries.