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 often 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).
Skin, nasal, laryngeal, pharyngeal, and esophageal mucosal surfaces can also be affected, leading to epistaxis, hoarseness, or dysphagia, respectively. Anogenital involvement in women may present with vulvovaginal or perianal blisters, erosions, 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, breast cancer, 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
- Bullous pemphigoid, most frequently seen in the elderly, presents with tense blisters.
- Pemphigus vulgaris presents with flaccid, fragile blisters.
- The lesions of erythema multiforme are round, light-red spots with concentric zones and, at times, blisters and exudates.
- Linear IgA bullous dermatosis
- Bullous systemic lupus erythematosus
- Lichen sclerosus lacks the vesicles and erosions seen in MMP.
- Genital erosive lichen planus
- Genital aphthous ulcers
- Behçet disease
- Reactive nonsexually related acute genital ulceration (RNSRAGU)
- Hailey-Hailey disease occurs primarily in body folds such as the inguinal fold.
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