Pemphigus vulgaris - Oral Mucosal Lesion
The estimated incidence worldwide is 0.76-5 cases per million per year, although PV occurs in higher incidences in individuals of Jewish ancestry, as well as in certain geographic areas (Middle East, Southeastern Europe, and India). Variants of the ST18 gene have been found to confer increased risk of PV in some populations. PV is typically a disease of adults, with average age of onset between 40 and 60 years, but PV rarely can occur in childhood and young adulthood. There does not appear to be a consistent sex predilection.
Almost all patients with PV develop painful erosions on the oral mucosa, and individuals with the mucocutaneous type develop flaccid bullae and erosions on the skin. Before the introduction of systemic corticosteroids, the mortality of PV was 75%. Still, severe cases of PV can be life-threatening, and complications can be related immunosuppression from drugs used to treat severe PV, secondary infections, loss of the skin barrier, and poor oral intake.
Patients usually complain of painful sores in the mouth and bleeding gums when brushing their teeth. Lesions most likely involve the palatine and buccal mucosa. Lesions can rarely extend to the vermillion of the lips. Involvement of the throat may cause sore throat, pain with swallowing, and hoarseness. In severe disease, involvement of the entire esophagus has been reported. The oral cavity is frequently the first site of involvement, with painful skin lesions sometimes appearing months or years later. Other mucosal sites that may be involved include the nasal mucosa, throat, eyes, and genital areas.
L10.0 – Pemphigus vulgaris
49420001 – Pemphigus vulgaris
Differential Diagnosis & Pitfalls
- Lichen planus – Erosive oral lesions of lichen planus may resemble PV but can be distinguished by the presence of characteristic radiating reticulations or keratotic striae, known as Wickham striae.
- Oral ulcers of systemic lupus erythematosus
- Mucous membrane pemphigoid – This condition mostly affects the mucosa and almost always the gingiva, although other sites may be involved. Only 20% of patients show cutaneous involvement.
- Aphthous stomatitis – This would not involve the attached gingiva. Ulcers remit completely and then recur.
- Primary herpes gingivostomatitis – The oral ulcerations may be diffuse but are composed of smaller lesions (1-2 mm) that coalesce.
- Plasma cell gingivitis – This hypersensitivity reaction presents as marked erythema of the gingiva (desquamative gingivitis) without blister formation.
- Epidermolysis bullosa acquisita – This almost never presents intraorally without skin involvement.
- Linear IgA disease – This almost never presents intraorally without skin involvement.
- Chronic oral erythema multiforme – These ulcers remit completely and then recur and are associated with recurrent herpes simplex virus infection.
- Bullous pemphigoid – This autoimmune bullous dermatosis is less likely to have oral involvement (estimated 20% of cases) and can be distinguished by histological and immunofluorescence studies.
- IgG/IgA pemphigus – A rarely reported condition. Unclear whether it represents a separate entity or pemphigus vulgaris that it transitioning to IgA pemphigus. Clinical findings in a recently reported series resembled pemphigus vulgaris, pemphigus foliaceus, and IgA pemphigus. In IgG/IgA pemphigus, dapsone can be used as an ancillary treatment.
Drug Reaction Data