Linear IgA bullous dermatosis

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(Redirected from Linear IgA disease)

Linear IgA bullous dermatosis (LABD) is a rare, chronic autoimmune skin disorder characterized by blisters on the skin and mucous membranes. The disease is named for the linear deposition of IgA antibodies in the skin.

Signs and Symptoms[edit | edit source]

Patients with LABD often present with tense blisters and urticarial plaques. The blisters may be filled with clear fluid or blood, and are often surrounded by red, inflamed skin. The blisters can appear anywhere on the body, but are most common on the arms, legs, and torso. Mucous membranes, such as the mouth and genitals, may also be affected.

Causes[edit | edit source]

The exact cause of LABD is unknown, but it is thought to be an autoimmune response. This means that the body's immune system mistakenly attacks its own cells. In the case of LABD, the immune system attacks the skin cells, leading to the formation of blisters.

Diagnosis[edit | edit source]

Diagnosis of LABD is typically made through a combination of clinical examination and skin biopsy. The biopsy will show a linear deposition of IgA at the basement membrane zone, which is characteristic of the disease.

Treatment[edit | edit source]

Treatment for LABD typically involves medications to suppress the immune system and reduce inflammation. These may include corticosteroids, dapsone, and other immunosuppressive drugs.

Prognosis[edit | edit source]

The prognosis for LABD is generally good, with most patients achieving remission with treatment. However, the disease can be chronic and relapsing, and some patients may experience significant morbidity.

See Also[edit | edit source]

References[edit | edit source]


Linear IgA bullous dermatosis Resources
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Contributors: Prab R. Tumpati, MD