Pemphigus vulgaris - Anogenital in
See also in: Overview,Oral Mucosal LesionAlerts and Notices
Synopsis
The estimated incidence of PV worldwide is 0.76-5 cases per million per year, although it 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 onset between the ages of 40 and 60 years, but PV rarely can occur in childhood and young adulthood. The mean age of onset for male anogenital pemphigus ranges from 32-67 years of age.
Almost all patients with PV develop painful erosions on the oral mucosa, and individuals with the mucocutaneous type develop flaccid bullae, erosions, and crusted erosions / plaques 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 to immunosuppression from drugs used to treat severe PV, secondary infections, loss of the skin barrier, and poor oral intake.
Oral mucosal involvement is more common than genital involvement. The larynx, esophagus, conjunctiva, nasopharynx, and urethra can be involved rarely. The presence of genital lesions in men has only been described in a few case reports and small case series. It is less commonly observed than anogenital involvement in women. In rare cases, penile erosions can be the first manifestation of PV, followed by classic mucocutaneous findings. Lesions may be seen on the glans, shaft, and anus, as well as other mucosal sites.
Codes
ICD10CM:L10.0 – Pemphigus vulgaris
SNOMEDCT:
49420001 – Pemphigus vulgaris
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Diagnostic Pearls
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Differential Diagnosis & Pitfalls
- Pemphigus foliaceus
- IgG/IgA pemphigus – A rarely reported condition. Unclear whether it represents a separate entity or pemphigus vulgaris that is 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.
- 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.
- Reactive infectious mucocutaneous eruption (RIME) – Hemorrhagic crusting of lips is accompanied by a sparse macular, papular, or vesicular cutaneous eruption.
- Epidermolysis bullosa acquisita
- Hailey-Hailey disease
- Drug-induced erythroderma
- Erythrodermic psoriasis
- Contact dermatitis
- Lichen planus
- Cicatricial pemphigoid
- Bullous pemphigoid – This autoimmune bullous dermatosis is less likely to have genital involvement and can be distinguished by histological and immunofluorescence studies.
- Candidiasis
- Aphthous ulcers
- Behçet disease
- Herpes simplex virus (HSV) – The genital ulcerations may be diffuse but are composed of smaller lesions (1-2 mm) that coalesce. Unlike pemphigus, significant cutaneous involvement in genital herpes would be unusual.
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Management Pearls
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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.Subscription Required
References
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Last Reviewed:06/17/2020
Last Updated:02/16/2022
Last Updated:02/16/2022
Pemphigus vulgaris - Anogenital in
See also in: Overview,Oral Mucosal Lesion