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Mucous Membrane Pemphigoid

By

Daniel M. Peraza

, MD, Geisel School of Medicine at Dartmouth University

Reviewed/Revised Feb 2024
View PATIENT EDUCATION

Mucous membrane pemphigoid is the designation given to a heterogeneous group of rare chronic autoimmune disorders that tend to cause waxing and waning bullous lesions of the mucous membranes, often with subsequent scarring and morbidity. Diagnosis is by skin biopsy and direct immunofluorescence. Topical or intralesional corticosteroids and a combination of doxycycline and nicotinamide may be used for mild disease. Systemic immunosuppression may be needed for severe disease.

Bullae are elevated, fluid-filled blisters ≥ 10 mm in diameter.

(Synonyms of mucous membrane pemphigoid that are no longer used include cicatricial pemphigoid, ocular cicatricial pemphigoid, and benign mucous membrane pemphigoid.)

Mucous membrane pemphigoid (MMP) is characterized by subepithelial lesions caused by autoantibodies against molecules of epithelial basement membranes. The target molecules lie deeper than those of bullous pemphigoid Bullous Pemphigoid Bullous pemphigoid is a chronic autoimmune skin disorder resulting in generalized, pruritic, bullous lesions in older adults. Mucous membrane involvement is rare. Diagnosis is by skin biopsy... read more Bullous Pemphigoid . Several autoantibodies have been identified, including those against BPAG2, laminin-332, and type VII collagen. Antibodies to beta-4 integrin have been identified in generalized MMP and ocular MMP, and antibodies to alpha-6 integrin have been identified in oral MMP.

General reference

  • 1. Rashid H, Lamberts A, Borradori L, et al: European guidelines (S3) on diagnosis and management of mucous membrane pemphigoid, initiated by the European Academy of Dermatology and Venereology - Part I. J Eur Acad Dermatol Venereol 35(9):1750-1764, 2021. doi: 10.1111/jdv.17397

Diagnosis of Mucous Membrane Pemphigoid

  • Skin biopsy and direct immunofluorescence

Prevalent mucosal involvement and scarring lesions help distinguish MMP from bullous pemphigoid. Diagnosis of mucous membrane pemphigoid is supported by lesion biopsy and direct immunofluorescence (1 Diagnosis reference Mucous membrane pemphigoid is the designation given to a heterogeneous group of rare chronic autoimmune disorders that tend to cause waxing and waning bullous lesions of the mucous membranes... read more ). Linear basement membrane deposits may include IgG, IgA, and C3. Serum autoantibodies tend to be absent or at low titer.

Diagnosis reference

  • 1. Schmidt E, Rashid H, Marzano AV, et al: European Guidelines (S3) on diagnosis and management of mucous membrane pemphigoid, initiated by the European Academy of Dermatology and Venereology - Part II. J Eur Acad Dermatol Venereol 35(10):1926-1948,2021. doi: 10.1111/jdv.17395

Treatment of Mucous Membrane Pemphigoid

  • For mild disease, corticosteroids and doxycycline plus nicotinamide

  • For severe disease, systemic immunosuppression

Severe disease may require systemic immunosuppression with dapsone or prednisone or sometimes high-dose prednisone with immunosuppressants (eg, rituximab, azathioprine, mycophenolate, cyclophosphamide) and IV immune globulin.

Treatment reference

  • 1. Schmidt E, Rashid H, Marzano AV, et al: European Guidelines (S3) on diagnosis and management of mucous membrane pemphigoid, initiated by the European Academy of Dermatology and Venereology - Part II. J Eur Acad Dermatol Venereol 35(10):1926-1948, 2021. doi: 10.1111/jdv.17395

Prognosis for Mucous Membrane Pemphigoid

Mucous membrane pemphigoid progresses slowly, rarely remits spontaneously, and often responds incompletely to treatment. Depending on the site affected, serious sequelae may include ocular damage and blindness, airway erosions and destruction, and strictures of the esophagus or anogenital regions. Patients with anti-laminin-332 MMP have an increased risk of internal cancer; if this form is suspected (eg, by immunofluorescence results), patients should be screened for cancers common to their age and sex.

Key Points

  • Mucous membrane pemphigoid is a group of rare chronic autoimmune disorders causing waxing and waning bullous lesions of the mucous membranes, often with scarring.

  • Diagnose using skin biopsy and direct immunofluorescence.

  • Treat with corticosteroids and doxycycline plus nicotinamide, or for severe disease, immunosuppressants.

Drugs Mentioned In This Article

Drug Name Select Trade
Acticlate, Adoxa, Adoxa Pak, Avidoxy, Doryx, Doxal, Doxy 100, LYMEPAK, Mondoxyne NL, Monodox, Morgidox 1x, Morgidox 2x , Okebo, Oracea, Oraxyl, Periostat, TARGADOX, Vibramycin, Vibra-Tabs
Aczone
Deltasone, Predone, RAYOS, Sterapred, Sterapred DS
RIABNI, Rituxan, RUXIENCE, truxima
Azasan, Imuran
CellCept, Myfortic
Cyclophosphamide, Cytoxan, Neosar
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