Mucous membrane pemphigoid in Adult
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 frequently (painful erosions and ulcers), followed by the conjunctiva (corneal injection, scarring that leads to symblepharon, and blindness). See Ocular cicatricial pemphigoid.
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 Brunstig-Perry variant).
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
- Severe aphthous ulcerations – They do not affect the keratinized attached mucosa of the gingiva.
- Behçet disease
- Bullous pemphigoid – Most frequently seen in the elderly; presents with nonfragile blisters that cause less scarring because they occur closer to the skin surface.
- 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.
- 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)
Last Updated: 01/23/2018