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

See also in: Overview,Anogenital
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Contributors: Susan Burgin MD, Carl Allen DDS, MSD, Sook-Bin Woo MS, DMD, MMSc
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 affects the mucous membranes and, less commonly, the skin. The mouth is involved most frequently, followed by the conjunctiva. MMP causes painful ulcers and erosions in the oral cavity. Because oral hygiene is difficult to perform, gingival lesions are exacerbated by plaque build-up. Patients will often report pain and bleeding on tooth brushing. Patients also avoid hard and spicy foods and may lose weight because of reduced food intake. While cicatrization is common in the larynx, eye, and skin, it is uncommon in the oral mucosa. Oral MMP is almost twice as common in females as it is in males, and it is seen most frequently in older individuals.

Nasal, anogenital, laryngeal, and esophageal mucosal surfaces can also be affected, leading to epistaxis, perianal erythema and scarring, phimosis or vaginal scarring, and hoarseness or dysphagia, respectively. Scarring is the endpoint for all sites of involvement except the oral mucosa. Cutaneous disease, when present, most frequently accompanies mucous membrane disease. Occasionally, cutaneous blistering and scarring dominate the clinical picture (so-called Brunsting-Perry variant).

Lesions develop over weeks to months.

Codes

ICD10CM:
L12.1 – Cicatricial pemphigoid

SNOMEDCT:
34250006 – Cicatricial pemphigoid

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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 - Oral Mucosal Lesion
See also in: Overview,Anogenital
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Mucous membrane pemphigoid : Oral erosions, Painful oral ulcers
Clinical image of Mucous membrane pemphigoid
Erosions on the labial mucosa.
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