Mucous membrane pemphigoid - Anogenital in
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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 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.
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
L12.1 – Cicatricial pemphigoid
SNOMEDCT:
34250006 – Cicatricial pemphigoid
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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.
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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
Last Updated:08/10/2022
Mucous membrane pemphigoid - Anogenital in
See also in: Overview,Oral Mucosal Lesion