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Mucous membrane pemphigoid - Anogenital in
See also in: Overview,Oral Mucosal Lesion
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Mucous membrane pemphigoid - Anogenital in

See also in: Overview,Oral Mucosal Lesion
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Contributors: Susan Burgin MD, Lowell A. Goldsmith MD, MPH
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 3 different antigens in the basement membrane zone have been identified: laminin 332 (previously laminin 5), the β4 unit of α6β4 integrin, and BP180. Those patients with autoantibodies to α6β4 integrin present with predominantly 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 (painful erosions and ulcers), followed by the conjunctiva (corneal injection, scarring that leads to symblepharon 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: 12/19/2017
Last Updated: 01/23/2018
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Mucous membrane pemphigoid - Anogenital in
See also in: Overview,Oral Mucosal Lesion
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Mucous membrane pemphigoid : Oral erosions, Painful oral ulcers
Clinical image of Mucous membrane pemphigoid
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