Atypical Vulvar Mass: Metastatic Vulvar Crohn’s Disease in a Young Female with Rheumatoid Arthritis: A Case Report

Abstract

BACKGROUND: Crohn’s disease (CD) is an inflammatory bowel disease that usually manifests in any part of the gastrointestinal tract from the oral cavity to the anus, with well-known extraintestinal presentations such as uveitis, erythema nodosum and arthritis. Rarely, it can also present with metastatic vulvar lesions, a conundrum for the gynecologist and gastroenterologist, owing to its similarity with other granulomatous and dermatological conditions like hidradenitis suppurativa, cellulitis and secondary abscess, making it a diagnostic dilemma.  

CASE: We present the case of a 24-years-old female, married with bilateral swelling in the vulvar region, insidious in onset, associated with pain, watery discharge, red discolouration of the surrounding skin and mild itching since 5 years that worsened in the past 6 months. She also had pain in multiple joints bilaterally, associated with early morning stiffness since childhood that worsened in the past 2 years and a history of hypothyroidism since 6 years followed by irregular menses since 4 years. She consulted a private clinic and the prescribed medications provided relief. However, her symptoms recurred a year later along with additional presentations of fissures and skin tags that gradually increased in severity. On visiting another hospital, a diagnosis of vulvar CD was confirmed on biopsy. She was started on methotrexate, oral steroids and azathioprine; which she adhered to for 6 months with failure to follow up thereafter. It eventually flared up for which she was referred to our hospital, where a thorough evaluation was done by several relevant departments. Positive biopsy findings showing non-caseating granulomas with giant cells along with positive ANA (Anti-nuclear antibody) and anti-CCP antibody (Anti-cyclic citrullinated peptide) correlated with the clinical symptoms confirmed the diagnosis of CD and rheumatoid arthritis respectively. The debulking procedure was done considering the severity of the lesion and the failure of the medications. Post-operative pathological evaluation of the procured tissue confirmed the pre-operative diagnosis.

CONCLUSION: This case underscores the difficulties encountered during the diagnosis of vulvar CD, especially in the absence of any gastrointestinal symptoms and signs on colonoscopy. It also emphasizes the importance of considering the differential of CD when faced with multiple vulvar symptoms. Undoubtedly, biopsy remains the cornerstone of diagnosis; and early diagnosis and treatment significantly is quintessential to improve the quality of life.