IgA-EBA L12.30

The inflammatory forms of EBA include a rare variant (around 3% of all EBA cases) of the epidermolysis bullosa acquisita group. In the inflammatory form, skin changes occur all over the skin, not just in the areas most frequently exposed to trauma. Therefore, it may resemble other subepidermal autoimmune bullous dermatoses, such as bullous pemphigoid (BP), mucous membrane pemphigoid (MMP), linear IgA bullous dermatosis and Brunsting-Perry pemphigoid. The occurrence of scars and milia during the course of the disease is rarer than in mechanobullous, classic EBA.

In linear IgA dermatosis-like EBA (IgA EBA), linear deposits of IgA are found in the BMZ on the skin side. Clinically, it resembles linear IgA bullous dermatosis with ring-shaped lesions (rosettes), few scars and milia. However, mucosal scarring rarely occurs, including significant damage to the eyes. This variant may respond to treatment with dapsone in a similar way to linear IgA bullous dermatosis (Vodegel RM et al. 2002).

The differentiation of IgA-EBA from other IgA-associated dermatoses such as LAB, IgA-p200 pemphigoid or IgA-MMP is often difficult.

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