Cicatricial (Mucous Membrane) Pemphigoid Guidelines

Updated: Oct 01, 2021
  • Author: Manuel Valdebran, MD; Chief Editor: Dirk M Elston, MD  more...
  • Print

Guidelines Summary

Clinical practice guidelines on the diagnosis and management of mucous membrane pemphigoid were published in September 2021 by the European Academy of Dermatology and Venereology. [15, 16]

Direct immunofluorescence should be performed on all patients suspected of having mucous membrane pemphigoid.

For direct immunofluorescence, obtain a 3- to 4-mm punch biopsy specimen. It is preferred the biopsy be taken from perilesional skin; however, if this is not practical for the clinician or is too painful for the patient, normal skin or mucosa is acceptable.

If direct immunoelectron microscopy is available, it also may be used in the diagnosis of mucous membrane pemphigoid. It allows detection of tissue-bound IgG and IgA.

Topical corticosteroids are recommended for mild or moderate oral or ocular mucous membrane pemphigoid. Topical corticosteroids can be used in addition to systemic therapy in moderate-to-severe oral or ocular mucous membrane pemphigoid.

High-potency topical corticosteroids can be considered in mild-to-moderate genital mucous membrane pemphigoid.

Oral dapsone, methotrexate, or tetracycline can be combined with topical corticosteroids or used as monotherapy for mild-to-moderate mucous membrane pemphigoid. Careful monitoring for adverse effects is recommended.

In refractory cases, oral corticosteroids, mycophenolate mofetil, cyclosporine, or azathioprine can be added.

For severe mucous membrane pemphigoid, first-line treatment is dapsone in combination with cyclophosphamide or oral corticosteroids.

For patients with refractory severe mucous membrane pemphigoid, additional agents that can be considered (in order of preference) are rituximab, intravenous immunoglobulins, or a tumor necrosis factor-alpha inhibitor.