Full body exacerbation of pemphigus foliaceus following a lapse in care: Treatment and management

Summary: Abstract Body: Background: Pemphigus foliaceous (PF) is an autoimmune blistering disease where autoantibodies attack desmoglein-1, affecting primarily the skin from the upper torso and sparing the mucus membranes. It may be inherited or acquired, with some drug-induced cases. PF affects men and women equally and mean age of onset is between 50 and 60 years. Clinical Course: 40-year-old male presents to dermatology clinic in December 2024 with biopsy proven pemphigus foliaceus as a return patient who was lost to follow up for lack of insurance. His last treatment regimen two years ago included prednisone 40 mg, doxycycline 100mg, mycophenolate mofetil 500mg, and triamcinolone .1% ointment, which the patient reported was effective until May 2024 when the disease progressed to cover his entire body. He reports discomfort, flakiness, and itchiness. Physical exam is notable for diffuse, thick hyperkeratotic crusting and scaling of scalp, face, trunk, upper and lower extremities with several open fissures of the dorsal hands. He was prescribed prednisone 20mg and chlorhexidine 4% external liquid as needed for wound care and when bathing. Complete blood count and complete metabolic profile were ordered and mycophenolate mofetil will be initiated at the next visit in one month, pending labs and proper wound care. Conclusion: PF is typically a benign disease that responds well to treatment and may have a relapse-remitting course. The differential diagnosis includes other forms of pemphigus, bullous impetigo, subcorneal pustular dermatosis, subacute cutaneous lupus erythematosus, and seborrheic dermatitis. PF responds best to corticosteroids, though azathioprine with or without mycophenolate mofetil can be good adjuncts. Logan Bearfield², Marc Gebara¹, Sarah McCain³, Joseph Jorizzo³ 1. Center for Dermatology Research, Atrium Health Wake Forest Baptist Medical Center, Winston-Salem, NC, United States. 2. Wake Forest University School of Medicine, Winston-Salem, NC, United States. 3. Dermatology, Atrium Health Wake Forest Baptist Medical Center, Winston-Salem, NC, United States. Adaptive and Auto-Immunity