Epidermolysis Bullosa Acquisita: Gastrointestinal Features

Abstract

Epidermolysis bullosa acquisita is an inherited connective tissue disease that causes blistering of the skin and mucous membranes. The incidence is estimated at 1/50,000, with severity ranging from mild to debilitating. Acquisita occurs in 0.2–0.5/million persons/year, with increased incidence in persons of sub-Saharan African descent. Mean age of onset is 50.

The gastrointestinal (GI) symptoms associated with this disease include:

Dysphagia, chronic constipation, fecal impaction

Gastrointestinal clinical signs and findings include [1–4]:

Mucosal involvement in 20 % of cases: mostly oral but also laryngeal and conjunctival

Esophagus can have blisters, erosions, scars, webs, ulcers, and strictures, which can lead to esophageal shortening and narrowing particularly in the dystrophic form (these symptoms are thought to be more prevalent in the dystrophic form because it presents earlier in life with a more narrow esophagus than acquisita)