Pemphigus vulgaris in Adult
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 onset between the ages of 40 and 60 years, but PV rarely can occur in childhood and young adulthood. There does not appear to be a consistent sex predilection.
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.
L10.0 – Pemphigus vulgaris
49420001 – Pemphigus vulgaris
Differential Diagnosis & Pitfalls
- Pemphigus foliaceus
- Drug-induced pemphigus (penicillamine, captopril, thiol-containing compounds)
- IgG/IgA pemphigus – A rarely reported condition. It is 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.
- 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 targetoid lesions of the skin is 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 starting the inciting medication.
- Reactive infectious mucocutaneous eruption (RIME) – 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, most commonly the gingiva and palatal mucosa, with only 20% of patients showing cutaneous lesions.
- Primary herpes gingivostomatitis – The oral ulcerations may be diffuse but are composed 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.
Drug Reaction Data