Linear IgA bullous dermatosis
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.
- Micrograph: Subepidermal blister formation and neutrophils
| Linear IgA bullous 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 |
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