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Mucous Membrane Pemphigoid

By

Daniel M. Peraza

, MD, Geisel School of Medicine at Dartmouth University

Last full review/revision Sep 2020| Content last modified Sep 2020
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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.)

Oral mucous membrane pemphigoid and ocular mucous membrane pemphigoid are typical, but other mucosal sites and the skin (usually of the head and upper trunk) may be involved. Older people are most often affected, women more than men.

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. 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.

Diagnosis

  • 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. Linear basement membrane deposits may include IgG, IgA, and C3. Serum autoantibodies tend to be absent or at low titre.

Prognosis

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.

Treatment

  • For mild disease, corticosteroids and doxycycline plus nicotinamide

  • For severe disease, systemic immunosuppression

Treatment of mucous membrane pemphigoid is similar to that for bullous pemphigoid. Topical or intralesional corticosteroids and a combination of oral doxycycline 100 mg orally twice a day and nicotinamide 500 mg orally 3 times a day may be useful for milder cases.

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

Drugs Mentioned In This Article

Drug Name Select Trade
CYTOXAN (LYOPHILIZED)
Gammagard S/D
CELLCEPT
IMURAN
PERIOSTAT, VIBRAMYCIN
RAYOS
RITUXAN
ACZONE
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