EBA Brunsting-Perry typeL12.30

EBA with the picture of localized scarring pemphigoid Brunsting-Perry with blisters on the head and neck without mucosal involvement.

In the inflammatory forms of EBA (see Epidermolysis bullosa acquisita/overview below), 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 EBA, scarring pemphigoid-like, the lesions are only on the skin. They are limited to the head, neck and shoulder area. The disease can progress with persistent erosions and atrophic scars (Tanaka, N et al. 2009). It often presents as a chronic and therapy-resistant scarring alopecia with extensive erosions and crusts. Blisters are only rarely registered and then only in an acute flare-up phase. Mucosal infestation is absent (the clinical picture is almost identical to bullous pemphigoid type Brunsting-Perrry).

Depending on the scarring pemphigoid (glucocorticoids systemically or locally depending on the findings). In addition, diaminodiphenylsulfone (DADPS) is recommended.

Another treatment option is off-label use with dupilumab (Raef HS et al. 2021).

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