Mucous membrane pemphigoid in Adult
See also in: Anogenital,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). 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:- Lichen planus – This may present as desquamative gingivitis, and Wickham striae may be present on the buccal mucosa.
- Hypersensitivity reactions such as plasma cell gingivitis may also present similarly.
- Pemphigus vulgaris – This is often (although not always) associated with skin lesions. The mouth ulcers are often the first manifestation and may precede skin lesions by months to years. Lesions of pemphigus are more likely to be ragged, superficial erosions.
- Epidermolysis bullosa acquisita – This almost invariably presents with typical skin lesions.
- Linear IgA disease – This may be indistinguishable, clinically.
- Bullous pemphigoid – This is almost always associated with skin lesions.
- Chronic (oral) erythema multiforme
- Severe aphthous ulcerations – They do not affect the keratinized attached mucosa of the gingiva.
- Behçet disease
- Systemic lupus erythematosus
- 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
- Genital erosive lichen planus
- Genital aphthous ulcers
- Behçet disease
- Reactive nonsexually related acute genital ulceration (RNSRAGU)
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 in Adult
See also in: Anogenital,Oral Mucosal Lesion