LINEAR IGA DERMATOSIS OF CHILDHOOD - A SERIES OF 12 TUNISIAN PATIENTS

Background. Linear IgA bullous dermatosis (LABD) of childhood is one of the chronic, non-hereditary blistering diseases of childhood in which clinical, histologic and therapeutic findings are similar to those associated with bullous pemphigoid (BP) and dermatitis herpetiformis (DH). LABD, however, can be distinguished From BP of DH by direct immunofluorescence (IF) demonstration of linear IgA deposits along the basement membrane zone (BMZ). Results. During the period 1984-1993, 12 children with LABD were studied. Their ages ranged from 2 years to 15 years with a mean of 8.5 years. There were 7 males and 5 females. All children had a generalized eruption consisting of large tense blisters arising on normal skin. The blisters were more profuse on the lower trunk, pelvic region and limbs. Face and scalp were also affected. Occasionnaly, annular blister formation producing a << rosette >> or << cluster of jewels >> was found. Pruritus was frequent. Histological features of BP and DH were seen. Direct IF showed linear deposits of IgA at the BMZ in all cases. IgM, IgG, and complement were also seen in 8 cases. Four patients showed IgA BMZ antibodies by indirect IF. There were no symptoms of malabsorption and 3 patients had a mild bowel lesions. HLA studies showed the B8DR3 antigen in 7 of the 10 patients studied. Nine patients were treated with dapsone associated in 3 patients with prednisone. Three patients were controlled on oxacillin. Conclusion. LABD of childhood is a definite clinical entity. It is the most frequent chronic, non hereditary bullous disease of childhood in Tunisia. It is characterized by a self limiting blistering eruption which resembles BP or DH histologically and has a characteristic linear deposits of IgA at the BMZ of the skin. The treatment consisted on dapsone therapy, but 3 patients in our study were well controlled on oxacillin.