A 61-year-old white male with a self-reported unknown dermatologic
disorder was admitted to the burn intensive care unit for several weeks of worsening erosions involving
his head, upper chest, and upper back. His lesions began 12 weeks earlier and had gradually progressed to involve more of his skin. The patient reported mild trauma to the head while working in the attic of his home that resulted in the first erosion, but he denied
any other trauma to other affected areas. He had failed multiple
topical treatments as an outpatient, including fluocinonide cream 0.1% (Vanos; Medicis Pharmaceutical Corporation, Scottsdale,
AZ), triamcinolone, petroleum jelly, Aquaphor (Beiersdorf Inc, Wilton, CT), and bacitracin. A review of the patient’s outside records (which were unavailable at the time of initial evaluation) showed he had had biopsies taken from multiple lesions on his body that were suggestive of pemphigus vulgaris (PV).
On physical examination, approximately 50% of total body surface area was involved. Large erosions were present on the face, bilateral upper extremities, and on the anterior and posterior trunk (Figure 1). Several scattered intact bullae were also present, and the patient exhibited a positive Nikolsky sign. There were also erosions on the buccal mucosa and the gums. No lesions were found on the conjunctiva or genitalia. The presence of antibodies to desmoglein types 1 (129 U/mL) and 3 (152 U/mL) were detected in serum.
Once hospitalized in the burn unit, the patient inappropriately underwent debridement by the plastic surgery department and was started on intravenous methylprednisolone at 125 mg every 6 hours. His conditioned deteriorated, and subsequently,
a dermatology consultation was ordered. His treatment was then replaced with mycophenolate mofetil (MMF) at 1.5 g twice daily combined with prednisone 80 mg daily. After 1 week, he was discharged on this regimen combined with Peridex oral rinse (3M, St. Paul, MN), magic mouthwash (2% viscous lidocaine, calcium carbonate, diphenhydramine), and calcium and vitamin D supplements. Topically, the erosions were managed with petrolatum and gauze dressings.
One week after starting the combined treatment of prednisone and MMF, the patient had developed new blisters on the palmar surfaces
of both hands, and he also had a breakout on the back of his right leg and buttocks. The prednisone dosage was increased to 100 mg each day, Ceftin (GlaxoSmithKline, Research Triangle Park, NC) 250 mg twice a day was added, and MMF was continued.
Over the next 14 weeks, the MMF dosage was maintained, while the prednisone was tapered to 20 mg every other day without recurrence of any lesions. After 2 weeks, the skin began to reepithelialize,
pain was greatly reduced, and there were no oral or conjunctival lesions. However, the bullae on his hands and soles persisted. At 4 weeks, the patient developed +2 bilateral lower extremity edema. After 5 weeks, there was a 33% reduction in levels of antibodies to desmoglein 1 (87 U/mL), and a 15% reduction
in antibodies to desmoglein 3 (131 U/mL). At 6 weeks, there was near-complete reepithelialization, and his bilateral lower extremity
edema had resolved (Figure 2). At 14 weeks, no lesions were noted. His PV was well controlled, and all laboratory values were within normal limits (Figure 3). Twenty-four weeks after initiating
treatment, the patient was still in remission with MMF 1.5 g twice daily and alternate-day dosing of prednisone 12.5 mg.
PV is an autoimmune disease characterized by the development of immunoglobulin G (IgG) antibodies against specific adhesion proteins on the surface of keratinocytes, causing acantholysis