LINEAR IgA BULLOUS DERMATOSIS

By Gary M. White, MD

Linear IgA bullous dermatosis


Linear IgA bullous dermatosis (LIGABD) is a rare autoimmune bullous dermatosis with IgA found at the dermoepidermal auction.

Clinical

Circinate, serpiginous, erythematous patches and plaques with vesicles and bulla at the periphery giving the appearance of a string of pearls is characteristic. Adult LIGABD (as opposed to chronic bullous disease of childhood) has its mean onset in the 5th decade. Clinically and histologically it may resemble either dermatitis herpetiformis or bullous pemphigoid. Occasionally, it may resemble TEN clinically. A gluten-sensitive enteropathy is not associated. This disease may be drug-induced (e.g. vancomycin) or associated with malignancy. Such an associated neoplasia is infrequent (4-5%) and the disease does not necessarily clear with removal of the tumor. Oral involvement commonly occurs and ocular involvement with the potential for conjunctival scarring and blindness may be seen. Rare cases present as desquamative gingivitis. There are case reports of LABD being associated with inflammatory bowel disease. In this case, the disease remits after colectomy.

Drug-Induced LIGABD

Vancomycin is the classic drug known to induce LIGABD. Other drugs which have been reported include captopril, lithium carbonate, trimethoprim with sulfamethoxazole, diclofenac sodium, cefamandole nafate, phenytoin, somatostatin, and amiodarone [AD 1996;132;1289]. Clinically the drug-induced and the non-drug induced LIGABD are virtually indistinguishable although it has been suggested that the drug-induced form has less mucosal involvement and a lower incidence of circulating autoantibodies than the idiopathic form. Various case reports document how vancomycin-induced LIGABD can be of such acute onset and severe as to mimic toxic epidermal necrolysis.

Adult, Idiopathic, Linear IgA Bullous Dermatosis

In a series of 72 patients > 15 years of age with idiopathic LIGABD, the following was found: mean age 54 years. 60% has mucous membrane involvement. [Br J Dermatol 2017;177;212].

Treatment

Super-potent topical steroids either alone or in combination with dapsone and/or sulfasalazine is first line therapy. If not sufficient or in the case of severe disease, rituximab, cyclophosphamide, mycophenolate mofetil, methotrexate, or IvIg may be added.

Additional Pictures

Linear IgA bullous dermatosis

References

An. Bras. Dermatol. vol.88 no.4 Rio de Janeiro July/Aug. 2013

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