Linear IgA bullous dermatosis
| Linear IgA bullous dermatosis | |
|---|---|
| Other names | Linear IgA dermatosis |
| (a) Widespread vesiculobullous eruption on the lower limbs with elements in a “string of pearls” arrangement; (b) targetoid vesicular lesions on erythematous skin involving the dorsa of the hands; (c) blisters with a “string of pearls” configuration and crusts in the perioral area; (d) erythematous, vesicular lesions partially eroded on the posterior aspects of both thighs; (e) vesicles involving the vulvar area in a child. | |
| Specialty | Immunology, dermatology |
Linear IgA bullous dermatosis is a rare immune-mediated blistering skin disease frequently associated with medication exposure, especially vancomycin, with men and women being equally affected.: 135 It was first described by Tadeusz Chorzelski in 1979 and may be divided into two types:: 587
Adult linear IgA disease is an acquired, autoimmune blistering disease that may present with a clinical pattern of vesicles indistinguishable from dermatitis herpetiformis, or with vesicles and bullae in a bullous pemphigoid-like appearance. This disease can often be difficult to treat even with usually effective medications such as rituximab.
Childhood linear IgA disease (also known as "Chronic bullous disease of childhood") is an acquired, self-limited bullous disease that may begin by the time the patient is age 2 to 3 and usually remits by age 13.