Pemphigus vulgaris - Anogenital in
Pemphigus vulgaris affects men and women equally, and the mean age of onset is 50-60 years. The estimated incidence worldwide is 0.76-5 cases per million per year, although it occurs in higher incidences in individuals of Jewish ancestry. There are 2 subtypes of PV: the mucosal-dominant type and the mucocutaneous type. 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 PG was 75%. Still, severe cases of PV can be life threatening, and complications are related to the use of immunosuppression, secondary infection, loss of the skin barrier, and poor oral intake.
Oral mucosal involvement is more common than genital involvement. Genital involvement is usually observed in the setting of extensive mucocutaneous disease and, as such, is considered to be a marker of disease severity and associated with treatment resistance and poor prognosis. In a small study (n=34), 44% of women had genital involvement of PV, and it was primarily observed in patients with moderate-to-severe mucocutaneous disease. PV is rarely seen in isolation on genital skin.
Patients present with erosions that can occur on the labia majora, labia minora, cervix, vagina, and clitoris.
For more information on familial pemphigus vulgaris, see OMIM.
L10.0 – Pemphigus vulgaris
49420001 – Pemphigus vulgaris
- Pemphigus foliaceus
- Paraneoplastic pemphigus – Associated with underlying neoplasms (non-Hodgkin lymphoma, chronic lymphocytic leukemia, Castleman disease, thymomas, sarcomas, Waldenström macroglobulinemia).
- Erythema multiforme
- Stevens-Johnson syndrome – Usually drug-induced and accompanied by high fever, skin tenderness, mucosal erosions, and desquamation 1-3 weeks after starting the inciting medication.
- Epidermolysis bullosa acquisita
- Hailey-Hailey disease
- Contact dermatitis
- Lichen planus
- Mucous membrane pemphigoid
- Bullous pemphigoid – This autoimmune bullous dermatosis is less likely to have genital involvement and can be distinguished by histological and immunofluorescence studies.
- Aphthous ulcers
- Behçet disease
- Herpes simplex virus (HSV) – The genital ulcerations may be diffuse but are comprised of smaller lesions (1-2 mm) that coalesce. Unlike pemphigus, significant cutaneous involvement in genital herpes would be unusual.
- Reactive nonsexually related acute genital ulcers (RNSRAGU)
Last Updated: 04/09/2018