Linear IgA Dermatosis

Key Points
Rare, blistering skin condition first affecting the limbs, though it may appear anywhere on the body
The condition is an auto-immune response, and may be idiopathic or appear in relation to other factors
Consists of oval or round blisters and red patches covered with vesicles or bullae (larger blisters), which may crust or ulcerate, and are accompanied by itching

Linear IgA dermatosis is a rare, blistering skin condition which typically affects the limbs first, and then spreads to other parts of the body. The condition consists of the formation of oval or round blisters on normal or slightly reddened skin. Red patches, which may be elevated or flat, form, and are covered with vesicles (tiny blisters) and/or bullae (larger blisters). Lesions may crust or ulcerate, and are typically accompanied by a varying degree of itching. Lesions usually form on the limbs first, then begin to appear in other areas, including the lips and mouth interior and eyes. Lesions on the eyes may cause dryness, sensitivity to light, vision problems, scarring, and may even lead to blindness in severe cases. Lesions may form on any area of the body, and new lesions may often arise around older lesions in a ring or in clusters.

Linear IgA dermatosis is an auto-immune response, in which the immune system attacks the epidermis. It may be idiopathic, or may appear as a drug reaction or following an infection. The drug most often associated with Linear IgA dermatosis is vanomycin, though a variety of other drugs may also cause such a reaction. Infrequently, the condition may be associated with lymphoma, hematological or rheumatological disorders, ulcerative colitis, and tumors.

Differential Diagnosis (Other conditions with similar appearance)Epidermolysis bullosa Acquisita
Chronic bullous disease of childhood
Erythema multiforme
Bullous pemphigoid
Dermatitis herpetiformis

Diagnosis
Key Points
Initial diagnosis based on appearance of the affected area
Blood tests and skin biopsies will be performed to confirm diagnosis and rule out other conditions

Linear IgA dermatosis is initially diagnosed based on the appearance of the affected area. Blood tests and skin biopsies will be performed to confirm the diagnosis and to rule out other, similar appearing conditions.

Treatment
Primary treatment consists of orally administered Dapsone
Goal of treatment is the control of symptoms
The condition may be cured, though long-term treatment is often required

Linear IgA dermatosis may be cured, but treatment of the condition may be long-term. The primary treatment used to control and reduce symptoms is orally-administered Dapsone. As lesion formation may continue if treatment is reduced or stopped, long-term treatment is often necessary to affect a cure. Other therapies which may be effective include corticosteroids, tetracycline, Erythromycin, sulfapyridine, Colchicine, Mycophenolate mofetil, and intravenous administration of immunoglobulins.