Pemphigus Vulgaris with Pyoderma Gangrenosum: An Unusual Association or a Coincidence?

Abstract

Introduction: Pemphigus vulgaris is characterized by intraepidermal split and falls under the group of autoimmune blistering disorders. Pyoderma gangrenosum is a neutrophilic dermatosis that primarily involves the skin and is frequently associated with systemic diseases. The coexistence of pemphigus vulgaris and pyoderma gangrenosum is very rare. Herein, we report such a case presenting with pemphigus vulgaris along with pyoderma gangrenosum. Case Presentation: A 42-year-old female presented with complaints of multiple fluid-filled blisters and erosions over the body, along with oral intolerance to spicy food. She was diagnosed with pemphigus vulgaris. There were non-healing ulcers present over the buttock and elbow, with biopsy results suggestive of pyoderma gangrenosum. She was treated with injection dexamethasone 100 mg pulse therapy. Other immunosuppressives like azathioprine, mycophenolate mofetil, or cyclophosphamide were not administered due to deranged lab parameters. Consequently, the patient received 1 g of injection rituximab after 3 weeks of injection dexamethasone pulse therapy. The erosions over the trunk healed with hyperpigmentation, whereas the ulcers over the buttocks and elbow healed with cribriform scarring after 8 weeks of treatment. Conclusions: Pyoderma gangrenosum is associated with many systemic diseases like rheumatoid arthritis, ulcerative colitis, and systemic lupus erythematosus. To date, there are only three case reports found to be associated with vesiculobullous disorders. Pemphigus vulgaris with pyoderma gangrenosum is a very rare case scenario, as both are associated with IL-8.