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AT-A-GLANCE

AT-A-GLANCE

  • A chronic autoimmune subepithelial blistering disease characterized by erosive lesions of mucous membranes and skin that typically results in scarring in at least some sites of involvement.

  • Lesions commonly involve the oral and ocular mucosae; other sites that may be involved include the nasopharyngeal, laryngeal, esophageal, and anogenital mucosae.

  • A rare disorder with a predominance in the elderly and an estimated incidence of 1 to 2 cases per million annually; females are affected 1.5 to 2 times as often as males.

  • A progressive disorder that may result in serious complications (eg, blindness, loss of the airway, esophageal stricture formation).

  • Severity of involvement in one organ does not necessarily correlate with the presence or severity of disease at other sites.

  • Immunopathologic studies of perilesional mucosa and skin demonstrate in situ deposits of immunoreactants in epithelial basement membranes; circulating anti–basement membrane autoantibodies are detected in the sera of some but not all patients.

  • A variety of different autoantigens are recognized by autoantibodies from patients, suggesting that mucous membrane pemphigoid is not a single nosologic entity but a disease phenotype.

DEFINITION

Mucous membrane pemphigoid (MMP) is a rare chronic autoimmune subepithelial blistering disease characterized by erosive lesions of mucous membranes and skin that typically results in scarring of at least some sites of involvement.1-4

HISTORICAL PERSPECTIVE

In 1794, Wichmann described a chronic blistering disorder characterized by ocular involvement.5 What are thought to be related cases were reported by Thost in 1911.6 In 1949, Civatte separated this disorder from pemphigus on the basis of histopathologic findings.5 Lever affirmed this distinction and suggested that this disorder be named “benign mucous membrane pemphigoid,” a designation now regarded a misnomer given the potential devastating consequences of MMP.4 Over the years, a variety of alternate designations, such as cicatricial pemphigoid, oral pemphigoid, desquamative gingivitis, ocular pemphigoid, ocular cicatricial pemphigoid, essential shrinkage of the conjunctivae, ocular pemphigus, have been applied to MMP.

In 2002, an international consensus conference agreed upon the designation of this disorder as MMP in that this nomenclature is inclusive of patients with disease affecting any mucosal surface and that it emphasizes the mucosal predominant character of this disorder.1 In 2015, an international panel of experts issued a consensus statement providing accurate and reproducible definitions for disease extent, activity, outcome measures, end points, and therapeutic response.7

EPIDEMIOLOGY

MMP has been estimated to have an incidence of 1 to 2 cases per million annually; females are affected 1.5 to 2.0 times as often as males.8-10 MMP has a mean age of onset in the early to middle 60s.10 Although there is no known racial or geographic predilection, the HLADQB1*0301 allele is significantly increased in frequency in patients with oral, ocular, and generalized bullous pemphigoid; amino acid residues at positions 57 and 71 to 77 of ...

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