Mucous membrane pemphigoid - Oral Mucosal Lesion
To date, autoantibodies to 3 different antigens in the basement membrane zone have been identified: laminin 332 (previously laminin 5), the β4 unit of α6β4 integrin, and BP180. Those patients with autoantibodies to α6β4 integrin present with predominantly ocular disease.
MMP affects the mucous membranes and, less commonly, the skin. The mouth is involved most frequently, 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.
Nasal, anogenital, laryngeal, 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.
L12.1 – Cicatricial pemphigoid
34250006 – Cicatricial pemphigoid
- 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
- Mycoplasma pneumoniae-induced rash and mucositis (MIRM)
- Severe aphthous ulcerations – They do not affect the keratinized attached mucosa of the gingiva.
- Behçet disease
- Mucositis from chemotherapy