Pyoderma Gangrenosum Occurring in the Setting of Hypercortisolism Associated With Adrenocortical Adenoma: A Pathophysiological Paradox

January 2021 | Volume 20 | Issue 1 | Case Reports | 95 | Copyright © January 2021


Published online December 28, 2020

Chapman Wei a,b, Elizabeth Hazuka b, Peter DeRosa c, Jill M Paulson MDd, Adam J Friedman MDb

aThe George Washington University School of Medicine and Health Sciences, Washington, DC
bDepartment of Dermatology,The George Washington School of Medicine and Health Sciences, Washington, DC
cDepartment of Pathology, The George Washington School of Medicine and Health Sciences, Washington, DC
dDepartment of Medicine, Division of Endocrinology & Metabolism, The George Washington School of Medicine and Health Sciences, Washington, DC

Abstract
Pyoderma gangrenosum (PG) is a challenging, rare, ulcerating skin disease characterized by neutrophilic abundance and absence of infection, often associated with systemic diseases. We present a 25-year old previously healthy female with a 1.5-year history of treatment refractory PG. Features of Cushing’s syndrome such as facial plethora, striae, and lipodystrophy were noted on exam, which prompted several studies that ultimately revealed an adrenal adenoma. Following surgical excision of the adenoma, symptoms rapidly resolved and systemic immunosuppressants were discontinued. This rare case highlights the importance that adrenal adenoma and resultant Cushing’s syndrome may be a driver of PG despite the pathophysiologic paradox.

J Drugs Dermatol. 2021;20(1):95-97. doi:10.36849/JDD.2021.5566

CASE REPORT

A 25-year-old previously healthy female presented to dermatology with a 1.5-year history of steroid-unresponsive PG diagnosed by a previous dermatologist on her lower extremities. Previous treatment included multiple oral prednisone tapers, perilesional Kenalog injections, and topical clobetasol cream with limited to no improvement. On review of systems, the patient reported increased fatigue, easy bruisability, striae, facial redness, hip pain, and hair loss over the past six years; past laboratory testing was unremarkable. The patient denied any past medical history, including inflammatory bowel disease (IBD), arthritis, hematologic abnormalities, and HIV. She reported no relevant family or social history.

Physical examination revealed facial plethora, moon facies, supraclavicular fat pads, macular purpura on the bilateral upper extremities, truncal wide, purple striae, increased fine hair without terminal hair growth along her upper back, anterior clavicle, and neck (Figures 1a, b). Her left, distal, anteromedial lower leg had two cribriform scars from previous ulcers and her right distal anterior lower leg had two active ulcerations (Figure 2). Given the prior lack of response to systemic steroids, the patient started on cyclosporine 4 mg/kg daily for one month with limited to no improvement on wound closure no improvement on wound closure, at which time it was discontinued as a re- sult of new onset hypertension, and changed to mycophenolate mofetil (1000mg twice daily). Prior to taking cyclosporine, she never had a history of hypertension. Her active wounds were treated with local wound management, which included Santyl, dapsone, and bandaging with xeroform, non-adherent dressing, and Coban wrap.