Oral lesions are the first manifestation of pemphigus vulgaris in 50%–90% of the cases.

PEMPHIGUS VULGARIS

Pemphigus vulgaris (PV) is an autoimmune blistering disorder caused by circulating antibodies to epidermal adhesion molecules, primarily desmoglein 3.

PV is a potentially fatal autoimmune blistering disease.
The average age at presentation is 40-60 (compared to bullous pemphigoid which is 60-80).
Oral lesions are the first manifestation PV in 50%–90% of the cases.
Rituximab is first-line therapy.
A high incidence of PV is seen in patients of Jewish descent.
PV has been associated with solid malignancies of the larynx and the esophagus.
In the one study, significant associations were observed between pemphigus and chronic leukemia, multiple myeloma, and non-Hodgkin lymphoma.
A 5% risk of venous thromboembolism is seen it the year after PV diagnosis.

Widespread, flaccid bulla and crusted erosions in a middle-aged patient is classic. Oral erosions are characteristic and the patient may present with only oral ulcerations. The esophagus may be involved and sloughing of its entire surface has been reported. Nikolsky sign is positive. Untreated PV has a high mortality. With treatment, current mortality averages about 6%. Rarely, nail dystrophy or paronychia may occur. There is an increased susceptibility to the development of autoimmune diseases in the family members of patients with PV. Nikolsky's sign is positive: splitting of the epidermal of normal skin with shear force.

Genital involvement is not uncommon. In one study of 34 women with PV, vulvar involvement was observed in 44.1% and was significantly associated with nasal mucosa involvement.

Immunoflourescence

DIF shows intercellular IgG and/or C3. IIF is usually positive (looking for antibodies to desmoglien 1 and/or 3) and its titer may correlate with disease activity.

PV can have blisters but they tend to be fragile and break open quickly leaving shallow ulcers or erosions. Bullous pemphigoid in contrast presents with stronger or “tense” blisters that don't so easily break.

Scalp involvement is observed in up to 60% of people with pemphigus and in some cases, the scalp may be the first location affected.

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