Pemphigus vulgaris is a rare autoimmune blistering disease in which antibodies attack desmoglein, a protein that holds skin cells together. It usually starts with painful mouth erosions and can be life-threatening without treatment because of fluid loss and infection.
Key symptoms
- Painful mouth sores, the first sign in most patients
- Fragile blisters that break easily and leave raw areas
- Trouble eating and swallowing
- More pain than itch
- Weight loss and fatigue with widespread disease
What you find on exam
- Flaccid blisters and erosions on the skin and mucous membranes
- Positive Nikolsky sign: rubbing normal-looking skin makes it peel off
- Erosions in the mouth, eyes or genitals
- Percent of body surface affected; signs of infection and dehydration
Tests
- Skin biopsy from the edge of a fresh blister for routine histology
- Direct immunofluorescence of nearby skin: net-like IgG between skin cells
- Blood test for antibodies to desmoglein 3 and 1
- Baseline labs before immune-suppressing drugs: blood count, liver, kidney, TB and hepatitis screening
Treatment basics
- Oral prednisone to control the disease quickly
- Rituximab as first-line steroid-sparing therapy
- Other steroid-sparing drugs: mycophenolate or azathioprine
- Wound care with non-stick dressings, pain control and mouth rinses
- Prevent steroid side effects: bone protection, glucose checks, infection prevention
Red flags
- Widespread raw skin with fever or low blood pressure (sepsis)
- Unable to eat or drink, with dehydration
- Eye pain or vision change from eye involvement
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Sources: NCCPA PANCE Content Blueprint; NBOME COMLEX-USA clinical presentations; AAMC Core Entrustable Professional Activities (Core EPAs). Written by Making Synapses. Making Synapses is not affiliated with these organizations.