Mucous membrane pemphigoid - Anogenital in
See also in: Overview,Oral Mucosal LesionAlerts and Notices
Synopsis
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). Skin, nasal, laryngeal, pharyngeal, 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).
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, 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
- Bullous pemphigoid, most frequently seen in the elderly, presents with nonfragile blisters.
- Pemphigus vulgaris presents with flaccid, fragile blisters.
- Erythema multiforme – These lesions are round, light-red spots with concentric zones and, at times, blisters and exudates.
- Lichen sclerosus lacks the vesicles and erosions seen in MMP.
- Hailey-Hailey disease occurs primarily in body folds such as the inguinal fold.
- Genital aphthous ulcers
- Behçet disease
- Zoon balanitis – Orange-brown glistering plaques are seen.
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.Subscription Required
References
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Last Reviewed:08/09/2022
Last Updated:08/10/2022
Last Updated:08/10/2022
Mucous membrane pemphigoid - Anogenital in
See also in: Overview,Oral Mucosal Lesion