Linear IgA bullous dermatosis

Linear IgA bullous dermatosis is a rare autoimmune blistering disorder, afecting both children and adults. The goal of this review is to summarize the clinical, diagnostic, and therapeutic aspects of the disease. Subepidermal, tense blisters, urticariform plaques and erosions develop on the skin, typically in a grouped pattern. Annularly arranged lesions are referred to as “cluster of jewels”, while lesions forming a linear or arched pattern are named “string of pearls”. Mucosal involvement is also present in some cases, and scarring may lead to permanent complications. Specifc autoimmune diagnostic tests are essential for the defnitive diagnosis. The frst line treatment is dapsone and the disease shows a benign course in most cases.