Track: Case Reports

Topic: Inflammatory Diseases

Abstract:

We report a case of BPP to highlight its clinical presentation and review the overlapping features with cicatricial/mucous membrane pemphigoid and epidermolysis bullosa. A 60-year-old female with a past medical history of human immunodeficiency virus (HIV) and adrenal insufficiency presented to the outpatient dermatology clinic with a one-year history of painful erosions of the scalp, forehead, and right ear. On examination, erythematous ulcerated plaques with patches of scarring alopecia and milia on the scalp were noted (Fig. 1A). Additionally, erythematous plaques on the face with crust were also present (Fig. 1B). Pathologic exam revealed ulcers with inflammatory infiltrate of eosinophils (Fig. 2). Linear deposition of IgG and C3 at the dermoepidermal junction was seen on direct immunofluorescence (Fig. 3A, 3B), consistent with pemphigoid. The patient was initiated on betamethasone dipropionate augmented ointment topically. Systemic steroids were deferred in the setting of adrenal insufficiency until the treatment could be discussed with her endocrinologist. The patient was lost to follow up for 11 months and subsequently presented with sparse tense bullae on her trunk and a 2-3 cm erosion on the left buccal mucosa that was causing dysphagia, in addition to the previously noted findings (Fig. 4). ELISA revealed positive anti-BP180 antibodies and negative anti-BP230 antibodies. BPP can often be challenging to diagnose, since patients may often present with only scarring and erosions in the affected sites, and the tense bullae and vesicles characterizing subepidermal immunobullous disorders may be absent. The main treatment options used for BPP include topical and systemic corticosteroids.

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Caroline Halverstam, MD