Linear IgA bullous dermatosis

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Linear IgA bullous dermatosis
Linear IgA bullous dermatosis
Synonyms	Chronic bullous disease of childhood
Pronounce	N/A
Specialty	N/A
Symptoms	Blisters, itching, rash
Complications	Infection, scarring
Onset	Any age, commonly in children
Duration	Chronic
Types	N/A
Causes	Autoimmune disease
Risks	Genetic predisposition, medications
Diagnosis	Skin biopsy, direct immunofluorescence
Differential diagnosis	Bullous pemphigoid, Dermatitis herpetiformis
Prevention	N/A
Treatment	Dapsone, corticosteroids
Medication	Dapsone, prednisone
Prognosis	N/A
Frequency	Rare
Deaths	N/A

IgA Bullous Dermatosis

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

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

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

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

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

Prognosis

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

• Dermatitis herpetiformis
• Bullous pemphigoid
• Pemphigus vulgaris

References

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