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Mucous membrane pemphigoid - Anogenital in
See also in: Overview,Oral Mucosal Lesion
Other Resources UpToDate PubMed

Mucous membrane pemphigoid - Anogenital in

See also in: Overview,Oral Mucosal Lesion
Contributors: Erin X. Wei MD, Susan Burgin MD
Other Resources UpToDate PubMed

Synopsis

Mucous membrane pemphigoid (MMP), also known as cicatricial pemphigoid, is a heterogeneous group of chronic autoimmune blistering diseases caused by autoimmunity to various components of the basement membrane. To date, autoantibodies to over 10 different antigens in the basement membrane zone have been identified: bullous pemphigoid antigen 1 (BP230), bullous pemphigoid antigen 2 (BP180, type XVII collagen), laminin-5 (laminin-332), laminin-6 (laminin-311), type VII collagen, integrin beta 4 subunit of α6β4 integrin, 45-kD unknown epithelial protein, 168-kD unknown epithelial protein, 120-kD unknown epithelial protein, and uncein. Patients with autoantibodies to β4 integrin present predominantly with ocular disease.

MMP is almost twice as common in females as it is in males, and it is seen most frequently in older individuals.

MMP affects the mucous membranes and, less commonly, the skin. The mouth is involved most frequently (ie, painful erosions and ulcers), followed by the conjunctiva (corneal inflammation and scarring, conjunctiva inflammation, trichiasis, ectropion, symblepharon, ankyloblepharon, and blindness). Nasal, laryngeal, and esophageal mucosal surfaces can also be affected, leading to epistaxis, hoarseness, or dysphagia, respectively.

Uncircumcised men may experience phimosis secondary to scarring. Perianal disease may present as perianal erythema and scarring.

Cutaneous disease, when present, most frequently accompanies mucous membrane disease. Occasionally, cutaneous blistering and scarring dominate the clinical picture (so-called Brunsting-Perry variant).

Codes

ICD10CM:
L12.1 – Cicatricial pemphigoid

SNOMEDCT:
34250006 – Cicatricial pemphigoid

Look For

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

Best Tests

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Management Pearls

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Therapy

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Drug Reaction Data

Below is a list of drugs with literature evidence indicating an adverse association with this diagnosis. The list is continually updated through ongoing research and new medication approvals. Click on Citations to sort by number of citations or click on Medication to sort the medications alphabetically.

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References

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Last Reviewed:11/28/2021
Last Updated:12/05/2021
Copyright © 2022 VisualDx®. All rights reserved.
Mucous membrane pemphigoid - Anogenital in
See also in: Overview,Oral Mucosal Lesion
Mucous membrane pemphigoid : Oral erosions, Painful oral ulcers, Scarring alopecia, Erythematous plaques
Clinical image of Mucous membrane pemphigoid
Erosions on the labial mucosa.
Copyright © 2022 VisualDx®. All rights reserved.