This healthy 21-month-old boy developed a slowly progressive widespread bullous eruption associated with pruritus and pain when he pressed against the bullae on his back. Note the "string of pearls" or "cluster of jewels" configuration of some of the clusters of vesicles. Histopathology demonstrated a subepidermal bulla, and Immunofluorescence showed linear IgA staining along the basement membrane zone. Linear IgA bullous dermatosis (LABD) of childhood is a chronic, acquired, self-limited autoimmune blistering disease. It is characterized by large tense subepidermal blisters, typically most prominent on the abdomen and perineum but may also involve the trunk, extremities, face, and mucous membranes. New lesions typically appear at the periphery of older blisters, forming a configuration known as a “cluster of jewels” or a “string of pearls.” The target antigen of the IgA autoantibodies is a 120-kd secretion portion of the BP180 antigen. However, other antigens have been reported, including those with molecular weights of 285, 230, 180, 145, 100, and 97 kd. The diagnosis of this disease requires three criteria: a vesicular or bullous eruption; a subepidermal vesicle with a predominantly neutrophilic infiltrate on histology; and basement membrance zone-specific IgA antibody deposited in a linear pattern on direct immunofluorescence. There have been cases that also involved IgG antibodies deposited linearly at the basement membrane in addition to the IgA. These cases are thought to represent an overlap between LABD and bullous pemphigoid.
In the majority of cases of LABD of childhood, spontaneous remission occurs between 3 and 6 years after disease onset. Potent topical steroids may be sufficient in mild disease or may be used as an adjunct to systemic therapy in more severe disease. The most well-recognized treatments for LABD are dapsone or sulfapyridine. Systemic steroids are frequently used for early control of the disease and tapered in favor of steroid-sparing agents. Conservative treatment is preferable, as most cases are self-remitting. Other therapies with reported success include tetracycline, erythromycin, dicloxacillin, colchicine, and mycophenolate mofetil.
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