Esophageal involvement in cicatricial pemphigoid: a rare cause of dysphagia

Abstract

Cicatricial pemphigoid is a rare, autoimmune, blistering disorder affecting the mucous membranes and skin. Esophageal involvement affects a small proportion of affected individuals and may present up to 10 years after the initial onset of the disease. We present a case of esophageal cicatricial pemphigoid that presented initially as linear IgA disease. We describe the successful treatment of dysphagia by graded esophageal dilatations.

Esophageal Cicatricial Pemphigoid as an Isolated Involvement Treated with Mycophenolate Mofetil

“…Oral systemic corticoids are the most commonly used in advanced illnesses, specifically prednisone 1 mg/kg, with good short term [ 5 ] results. However, systematic glucocorticoids are often associated with immunosuppressors like azathioprine or cyclophosphamide as steroid-sparing agents [ 7 , 13 , 14 ]. But these treatments can cause significant side effects such as marrow aplasia, infections, neoplasia, or hemorrhagic cystitis.…”

Clinical, Imaging, and Pathologic Features of Conditions with Combined Esophageal and Cutaneous Manifestations

“…MMP is predominantly a disease of the elderly and is more common in women. Esophageal involvement may occur at initial manifestation of the disease or may not develop until years later (25). The diagnosis is established by characteristic Two patterns of disease are typically observed at esophagography: upper esophageal webs and smooth concentric or asymmetric strictures, although any part of the esophagus may be in volved (Fig 16) (27).…”

Case of subepidermal bullous disease with diffuse esophageal involvement presenting with immunoglobulin G autoantibodies to both the BP180 NC16a and C‐terminal domains, and immunoglobulin A autoantibodies to the BP180 NC16a domain

“…1 MMP with esophageal involvement is one of the severe forms, and shows lower motility of the esophageal wall, which results in dysphasia. 2 IgA autoantibodies targeting the C-terminus of BP180 may affect oral mucous membrane in pemphigoid gestationis. 3 Another study indicated that both IgG and IgA antibodies signified the disease severity.…”