Linear IGA bullous dermatosis in Tunisian children : 31 cases

Abstract

Background: Linear IgA bullous dermatosis (LAD) of children is relatively frequent in Africa. Aim: We undertook this study to evaluate the frequency of this disease among autoimmune bullous diseases (AIBDs) in Tunisian children. Materials and Methods: We present a 32-year retrospective study (January 1976 to December 2007). Children with chronic acquired bullous diseases seen at the Charles Nicolle Hospital of Tunis and for who direct immunofluorescence (DIF) of the perilesional skin demonstrated linear IgA immunoglobulin deposits were included in the study population. Results: Thirty-one children with LAD were selected representing 65.9% of all AIBDs of children selected in the same period, with a mean age of 5.5 years and a sex ratio (M/F) of 2.4. Most of the children had generalized eruption (28/31), more profuse on the face, pelvic region, buttocks and limbs. Mucosal lesions happened in only four children (12.9%). The mean duration of the disease was 14 months. DIF demonstrated linear IgA deposits along the dermal-epidermal junction in all patients. IgG, IgM, and complement were also seen (20/31). Indirect immunofluorescence was negative in 67% of cases. Eight patients responded to dapsone; however, prednisone had to be added in seven children to control the disease and erythromycin in four others. A long-term remission period was achieved in 76.1% of patients. Conclusion: This study confirms that LAD is the most common AIBD in children in Tunisia which frequently occurs in preschool-aged males. Independently of the used drug, a long-term remission is frequently observed.

Keywords

• Linear IgA bullous dermatosis
• children
• direct immunofluorescence
• Tunisian children