Linear IgA disease is a blistering disorder that affects both children and adults. It is nearly identical to a similar condition that affects children, “chronic bullous disease of childhood”. Chronic bullous disease of childhood usually presents before puberty with an abrupt onset of blistering in the genital region, later affecting hands, feet and face. In adults with linear IgA disease, the limbs are more often the first sites, although any area of the body may be affected later.
In the disease, clear round or oval blisters may arise from normal-looking or red skin. Red flat or elevated patches may arise, studded with small blisters (vesicles) or large ones (bullae), often target-shaped. The tendency for new blisters to arise in a ring around an old one is called the “string of beads” sign, and groups of small blisters may be described as a “cluster of jewels”. Crusts, scratch-marks, sores and ulcers may arise. The lesions can resemble other uncommon blistering skin diseases.
Most patients with linear IgA disease improve or clear with the medication dapsone or systemic steriods such as prednisone or prednisolone. This is a chronic disorder and lesions may persist indefinitely.
Linear IgA disease has been reported in association with inflammatory bowel disease, in particular ulcerative colitis.