Benign chronic bullous dermatosis of childhood: a review

Chronic bullous dermatosis of childhood is one of the nonhereditary blistering diseases of children. Clinically, it is characterized by predominantly monomorphous, large tense bullae, which often form a "rosette pattern" or "jewel-like" clustering and have a predilection for the lower trunk, pelvic region, and lower extremities. Histologically, a subepidermal blister is seen, which is indistinguishable from either bullous pemphigoid or dermatitis herpetiformis. Although usually responsive to sulfone therapy, some cases require the combination of sulfones and systemic corticosteroids or corticosteroids alone to control the disease. Recent advances in immunologic techniques reveal: 1. a linear band of IgA at the dermal-epidermal junction on direct immunofluorescence that has been reported both in the lamina lucida and below the basal lamina on immunoelectron microscopy; 2. IgA antibasement membrane antibodies on indirect immunofluorescence; 3. normal jejunal biopsies; and 4. a high association with HLA-B8. It remains unclear whether chronic bullous dermatosis of childhood represents a separate disease entity or is merely a variant of dermatitis herpetiformis. Chronic bullous dermatosis of childhood also differs from linear IgA dermatosis of the adult in that the latter is not associated with HLA-B8, and thus should not be confused with this disease by similar nomenclature.