Chapter 156: Pemphigus

Patient Story

A teenage boy presented with painful blisters on his face and mouth (Figure 156-1). 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.

Introduction

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

Epidemiology of the three major types of pemphigus:

• PV (Figures 156-1 to 156-4):

  • Most common form of pemphigus is in the US.

  • Annual incidence is 0.75 to 5 cases per 1 million population.¹

  • Usually occurs between 30 and 50 years of age, but can occur in childhood.²

  • Increased incidence in Ashkenazi Jews and persons of Mediterranean origin.²

  • Pemphigus vegetans is a variant form of PV (Figures 156-5 and 156-6).

• Pemphigus foliaceus (PF): Superficial form of pemphigus (Figure 156-7).

  • 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 20.¹

• Paraneoplastic pemphigus (PNP):

  • Onset at age 60 years and older.

  • Associated with occult neoplasms commonly lymphoreticular.