Mucous membrane pemphigoid - Oral Mucosal Lesion
See also in: Overview,AnogenitalAlerts and Notices
Synopsis
MMP affects the mucous membranes and, less commonly, the skin. The mouth is involved most often, 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.
If MMP affects the eye, there may be corneal inflammation and scarring, conjunctiva inflammation, trichiasis, ectropion, symblepharon, ankyloblepharon, and blindness. 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. 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.
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
Subscription Required
Diagnostic Pearls
Subscription Required
Differential Diagnosis & Pitfalls
- 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
- Reactive infectious mucocutaneous eruption (RIME)
- Severe aphthous ulcerations – They do not affect the keratinized attached mucosa of the gingiva.
- Behçet disease
- Mucositis from chemotherapy
- Systemic lupus erythematosus
Best Tests
Subscription Required
Management Pearls
Subscription Required
Therapy
Subscription Required
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
Subscription Required
Last Reviewed:08/09/2022
Last Updated:08/10/2022
Last Updated:08/10/2022
Mucous membrane pemphigoid - Oral Mucosal Lesion
See also in: Overview,Anogenital