Pemphigus vulgaris in Adult
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.
For more information on familial pemphigus vulgaris, see OMIM.
L10.0 – Pemphigus vulgaris
49420001 – Pemphigus vulgaris
- Pemphigus foliaceus
- Drug-induced pemphigus (penicillamine, captopril, thiol-containing compounds)
- Paraneoplastic pemphigus – Associated with underlying neoplasms (non-Hodgkin lymphoma, chronic lymphocytic leukemia, Castleman disease, thymomas, sarcomas, Waldenström macroglobulinemia).
- Erythema multiforme – This condition may appear clinically identical to paraneoplastic pemphigus. Patients may have a history of previous episodes that resolved within 2-4 weeks. The presence of classic "target" lesions of the skin are more consistent with erythema multiforme.
- Bullous pemphigoid – Tense vesicles and bullae are the predominant feature of this condition, and only 20% of patients have oral involvement.
- Stevens-Johnson syndrome – Usually drug-induced and accompanied by high fever, skin tenderness, mucosal erosions, and desquamation 1-3 weeks after start of the inciting medication.
- Mycoplasma-induced rash and mucositis (MIRM) – Hemorrhagic crusting of lips is accompanied by a sparse macular, papular, or vesicular cutaneous eruption.
- Staphylococcal and streptococcal toxic shock syndrome and toxic shock-like syndrome – Look for sudden onset of exanthematous eruption.
- Epidermolysis bullosa acquisita
- Linear IgA dermatosis
- Acute graft-versus-host disease – Clinical history of bone marrow transplantation.
- Drug-induced hypersensitivity syndrome (drug reaction with eosinophilia and systemic symptoms [DRESS]) – Look for facial edema (hallmark of DRESS), eosinophilia, hepatitis, and other visceral involvement.
- Subcorneal pustular dermatosis (Sneddon-Wilkinson syndrome)
- Hailey-Hailey disease
- Dermatitis herpetiformis
- Drug-induced erythroderma
- Erythrodermic psoriasis
- Atopic dermatitis with erythroderma
- Contact dermatitis
- Sézary syndrome (see cutaneous T-cell lymphoma)
- Phototoxic reaction
- Photoallergic reaction
- Lichen planus – The erosive oral lesions of lichen planus are usually surrounded by characteristic radiating keratotic striae.
- Oral ulcers of systemic lupus erythematosus
- Mucous membrane pemphigoid – This condition mostly affects the mucosa at various sites, with only 20% of patients showing cutaneous bullae.
- Primary herpes gingivostomatitis – The oral ulcerations may be diffuse but are comprised of smaller lesions (1-2 mm) that coalesce.
- Aphthous stomatitis
- Chemotherapy-induced mucositis
- 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 (20% of cases) and can be distinguished by histological and immunofluorescence studies.
Last Updated: 04/20/2018