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

Mucous membrane pemphigoid in Child

See also in: Anogenital,Oral Mucosal Lesion
Contributors: Vivian Wong MD, PhD, 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 often (ie, painful erosions and ulcers), followed by the conjunctiva (corneal inflammation and scarring, conjunctiva inflammation, trichiasis, ectropion, symblepharon, ankyloblepharon, and blindness). See ocular cicatricial pemphigoid.

Skin, nasal, anogenital, laryngeal, pharyngeal, 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, where it is less frequent.

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

In a 2022 study, malignancies, especially solid organ tumors, were reported in up to 13.8% of patients. These include lung carcinoma, prostate cancer, penile cancer, breast cancer (female or male), endometrial cancer, vulvar carcinoma, and non-Hodgkin lymphoma. The malignancy rate was higher when autoantibodies against laminin-332 were found.

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

Oral lesions:
Genital lesions:
Cutaneous lesions:

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:08/09/2022
Last Updated:08/10/2022
Copyright © 2022 VisualDx®. All rights reserved.
Mucous membrane pemphigoid in Child
See also in: Anogenital,Oral Mucosal Lesion
A medical illustration showing key findings of Mucous membrane pemphigoid : Oral erosions, Painful oral ulcers, Scarring alopecia, Erythematous plaques
Clinical image of Mucous membrane pemphigoid - imageId=109411. Click to open in gallery.  caption: 'Erosions on the labial mucosa.'
Erosions on the labial mucosa.
Copyright © 2022 VisualDx®. All rights reserved.