A Case of an Inflammatory Variant of Epidermolysis bullosa acquisita: Chronic Bullous Dermatosis Associated with Nonscarring Mucosal Blisters and Circulating IgG Anti-Type-VII-Collagen Antibody

Abstract

A 42-year-old man showed prominent blistering lesions of the mouth and esophagus in addition to a few bullous lesions of the skin. Direct immunofluorescence microscopy revealed distinct linear deposition of IgG and C3 at the epidermal basement membrane zone where slight deposition of IgA and IgM was also observed. In direct immunoelectron-microscopic examination, antibody was detected in the sublamina densa of the basement membrane zone. Immunoblot analysis with dermal extracts demonstrated that the patient’s serum contained circulating IgG antibodies against the 290-kD protein, which comigrated with type VII collagen. The lesions healed without any scars. The results of these studies corresponded to the laboratory findings in epidermolysis bullosa acquisita (EBA), although the clinical features were distinct from classic EBA.

Inflammatory Variant of Epidermolysis Bullosa Acquisita With IgG Autoantibodies Against Type VII Collagen and Laminin α3

“…But mucosal lesions are not a specific symptom and may also be present in patients with EBA, even if the mucosal lesions are nonscarring. 19 Our patient had the inflammatory variant of EBA, which is often accompanied by mucosal lesions. 3 A diagnosis of antiepiligrin (laminin 5) CP was also considered, 20 since circulating antibodies against laminin 5 were demonstrated in our patient.…”

Mucosal Morbidity in Patients With Epidermolysis Bullosa Acquisita

“…[6][7][8] In addition to cutaneous disease, EBA can affect mucous membranes or even present as a mucosal-predominant disorder that mimics cicatricial pemphigoid. [9][10][11][12][13][14][15] In the past 5 years, mucous membrane involvement in 4 patients with well-documented EBA has been evaluated and managed by a multidisciplinary team. In this report, we describe the character and extent of mucosal disease in these patients; documentthecomplicationsofsuchinvolvement; summarize the team management of these patients' mucosal disease; and report the use of noninvasive imaging techniques for the evaluation of anatomic and functional characteristicsofpharyngolaryngealinvolvement in patients with EBA with respiratory tract compromise, dysphagia, or both.…”

Epidermolysis bullosa acquisita

“…Similar to CP, the lesions and scars usually involve the mouth, upper esophagus, conjunctivae, anus, and vagina [28, 30] with or without similar lesions on the glabrous skin. There has also been evidence of involvement of the trachea in these patients [31] and of mucosal involvement without scarring [32]. Unlike classical EBA, patients with the CP-like presentation often do not show significant skin fragility, evidence of trauma-induced lesions, or a predilection for blistering on extensor skin surfaces.…”