P0825 - A Rare Case of Concurrent Vulvar Crohn’s Disease and Pyoderma Gangrenosum

Jaya Bommireddipally, MD¹, Kent Broussard, MD², Hanadi Osman, MD³, Ann Uhlhorn, RN⁴, Nisha Loganantharaj, MD^5
1Tulane University, New Orleans, LA; ²Louisiana State University School of Medicine, Metairie, LA; ³Tulane University School of Medicine, New Orleans, LA; ⁴Louisiana State University, New Orleans, LA; ⁵Louisiana State University School of Medicine, New Orleans, LA

Introduction: Crohn’s disease (CD) is a chronic inflammatory condition affecting the gastrointestinal tract and can result in extra-intestinal inflammation. Vulvar CD (VCD) is a rare extra-intestinal manifestation of CD with one study reporting VCD in less than 0.25% of women with inflammatory bowel disease (IBD). Pyoderma gangrenosum (PG) is an autoinflammatory dermatitis with neutrophilic dysfunction that has also been infrequently seen in patients with IBD. Coexistent VCD and PG is exceedingly rare with only a few cases reported in the literature.

Case Description/Methods: A 33-year-old female with fistulizing ileocolonic and perianal CD status-post total proctocolectomy with end ileostomy presents with dysuria, vaginal pruritis, and foul-smelling vaginal discharge. She denies any changes in her ileostomy output or bleeding from her ostomy. Her medications include ustekinumab injections (90 mg every 8 weeks) and azathioprine daily. Physical exam reveals bilateral labia minora and majora swelling, linear ulcerations, and granulation tissue. Pathology of vulvar biopsies showed squamous tissue with granulomatous inflammation and mixed inflammatory cell infiltrates consistent with VCD. Tests for HSV, Chlamydia, and Gonorrhea were negative. She then had repeat vulvar biopsies with neutrophil aggregates in the dermis and ulceration consistent with concurrent PG (Figure 1). She had persistent symptoms and vulvar ulceration despite therapy with topical methocarbamol, metronidazole, and dose-optimization of her ustekinumab to 90 mg every 4 weeks. She had mild clinical improvement on a short course of oral prednisone but was ultimately started on infliximab and azathioprine. After 6 months of therapy, she had resolution of her symptoms, improvement in labial swelling, and reduced ulceration of the vulva.

Discussion: VCD is an uncommon clinical entity requiring a multidisciplinary team with Gastroenterology, Dermatology, and Gynecology. Treatment guidelines do not exist due to the rarity of the condition, but multiple regimens have been employed. Mild VCD can be treated with topical corticosteroids or tacrolimus. Prior studies recommend the early use of cyclosporine or tumor necrosis alpha inhibitors (TNF-α) including infliximab for moderate-to-severe VCD. Surgical management with vulvectomy and wide-local excision can be considered in medically refractory disease. PG often rapidly responds to immunosuppressive medications including systemic corticosteroids or TNF-α inhibitors and wound care.



Disclosures:


Jaya Bommireddipally, MD¹, Kent Broussard, MD², Hanadi Osman, MD³, Ann Uhlhorn, RN⁴, Nisha Loganantharaj, MD⁵. P0825 - A Rare Case of Concurrent Vulvar Crohn’s Disease and Pyoderma Gangrenosum, ACG 2023 Annual Scientific Meeting Abstracts. Vancouver, BC, Canada: American College of Gastroenterology.