A young man presented with painful blisters on his face and mouth (Figure 185-1). The patient was referred to dermatology that day. The dermatologist recognized likely pemphigus vulgaris (PV) and did shave biopsies for histopathology and direct immunofluorescence of facial vesicles/bullae to confirm the presumed diagnosis. The patient was started on 60 mg of prednisone daily until the pathology confirmed PV. Steroid-sparing therapy was then discussed and started in 2 weeks from presentation.
Pemphigus vulgaris on the face of a young man with mouth involvement. (Courtesy of Eric Kraus, MD.)
Pemphigus is a rare group of autoimmune bullous diseases of skin and mucous membranes characterized by flaccid bulla and erosions. The three main types of pemphigus are PV (with the pemphigus vegetans variant), pemphigus foliaceous (with the pemphigus erythematosus variant), and paraneoplastic pemphigus. All types of pemphigus cause significant morbidity and mortality. Although pemphigus is not curable, it can be controlled with systemic steroids and immunosuppressive medications. These medications can be lifesaving, but also place pemphigus patients at risk for a number of complications. The word pemphigus is derived from the Greek word pemphix, which means bubble or blister.
Epidemiology of the three major types of pemphigus:
- PV (Figures 185-1, 185-2, 185-3, and 185-4):
- Most common form of pemphigus in the United States.
- Annual incidence is 0.75 to 5 cases per 1 million population.1
- Usually occurs between 30 and 50 years of age.2
- Increased incidence in Ashkenazi Jews and persons of Mediterranean origin.2
- Pemphigus vegetans is a variant form of PV (Figures 185-5 and 185-6).
- Pemphigus foliaceus (PF) (Figures 185-7, 185-8, 185-9, and 185-10): Superficial form of pemphigus.
- More prevalent in Africa (Figures 185-11 and 185-12).1
- Variant forms include pemphigus erythematosus (resembles the malar rash of lupus erythematosus) and fogo selvagem.
- Fogo selvagem is an endemic form of PF seen in Brazil and affects teenagers and individuals in their twenties.1
- Paraneoplastic pemphigus (PNP)
- Onset at age 60 years and older.
- Associated with occult neoplasms commonly lymphoreticular.
- Malignancies like non-Hodgkin lymphoma and chronic lymphocytic leukemia.
- Also associated with benign neoplasms such as thymoma and Castleman disease (angiofollicular lymph node hyperplasia).2
Pemphigus vulgaris on the back with crusted and intact bullae. Downward pressure on a bulla demonstrates a positive Asboe-Hansen sign with lateral spread of a fresh bullae. (Courtesy of Eric Kraus, MD.)
Pemphigus vulgaris involving the lips and palate of a 55-year-old woman. (Courtesy of Dan Shaked, MD.)
Severe fatal pemphigus ...