Linear immunoglobulin A (IgA) disease is an uncommon bullous disease distinguished from bullous pemphigoid and dermatitis herpetiformis by the linear deposits of IgA in the basement membrane zone.

Linear IgA disease occurs in adults and children. The childhood form is most frequently termed chronic bullous disease of childhood.

In linear IgA disease, vesicular or bullous skin lesions occur frequently in a clustered (herpetiform) arrangement. There is a predilection for flexural areas (eg, inguinal crease). As in dermatitis herpetiformis, severe burning and pruritus of cutaneous lesions are prominent features. It was previously considered a form of dermatitis herpetiformis but has no concomitant gluten-sensitive enteropathy and immunopathology. Also, genetic studies indicate that linear IgA disease is a separate disorder. Drug-induced linear IgA disease, most commonly associated with vancomycin, has been reported.

Diagnosis is by skin biopsy. Dapsone is the treatment of choice. Doses should be similar to those used for dermatitis herpetiformis (see Bullous Diseases: Treatment), and CBC monitoring should follow the same parameters. Other treatment options include glucocorticoids (systemic, topical, and intralesional), cyclophosphamide, azathioprine, colchicine, tetracycline and nicotinamide, and cyclosporine.

Last full review/revision September 2008 by Julie E. Russak, MD