(74) Metastatic Vulvar Crohn's Disease in the Pediatric Population

Background: Metastatic Crohn’s disease (MCD) is a rare dermatologic manifestation of Crohn’s disease (CD) that is defined by presence of skin lesions non-contiguous from the gastrointestinal tract. The presence of vulvar lesions is rare, with only 101 reported cases since 1965. The aim of this study is to further investigate metastatic vulvar Crohn’s disease (MVCD) in the pediatric population and assess presentation and therapeutic options.

Methods: We performed a retrospective review of patients who were treated for metastatic vulvar Crohn’s disease at a single institution using electronic medical records from between January 1, 2014, and August 1, 2024 (IRB #2024-0503). Patients without a specific diagnosis of MVCD were excluded.

Results: A total of 12 patients were identified with the diagnosis of MVCD. Average age at of diagnosis of MVCD was 13 years old, while average age for diagnosis of CD was 10 (both range 7-17). Two patients presented with vulvar disease prior to the diagnosis of gastrointestinal CD. Two patients were diagnosed with CD and MVCD concurrently. The duration of vulvar symptoms prior to diagnosis ranged from 2 weeks to 2 years but was not documented for many. All patients were diagnosed on physical exam alone with only 1 patient having a vulvar biopsy. The diagnosis was made by gynecologists 67% of the time (8/12), typically in collaboration with gastroenterology or dermatology. The most common physical exam findings were vulvar edema (8/12, 67%) and linear fissures (4/12, 33%). All patients except 1 had concerns for MVCD on magnetic resonance enterography (MRE). Seven patients were on biologic treatment for CD at the time of diagnosis, most commonly infliximab (4/7, 57%). All patients were prescribed antibiotics such as metronidazole (10/12, 83%) and ciprofloxacin (4/12, 33%), often simultaneously. Half of patients (6/12, 50%) were given topical steroids. Most patients had changes to their biologic prescriptions after MVCD diagnosis including increasing dose, frequency, or changing medication. Unfortunately, most patients had either persistent or recurrent MVCD symptoms with fewer than half with documented remission (5/12, 42%).

Conclusions: MVCD findings are subtle, and it may be underdiagnosed. This study highlights the importance of multidisciplinary care for children and adolescents with Crohn’s disease and familiarity with metastatic vulvar Crohn’s disease for gynecologists. Our study illuminates the utility of MRE for identifying patients with MVCD. In addition to better identifying those with MVCD, treatment standards are lacking, as there is no consensus regarding optimal therapies.