P048 Metastatic Crohn's Disease Debuting with Severe Oral Manifestation and Vulvar Involvement - A Diagnostic Challenge

Background: Metastatic Crohn's disease (MCD) is an unusual cutaneous manifestation in Crohn's disease (CD), and concomitant oral and vulvar involvement is even more unusual. It can debut with cavity lesions oral such as canker sores, ulcers, lip edema, granulomatosis, dry mouth, abscesses in the salivary ducts, erythema, gingivitis, glossitis, among others, however, simultaneous compromise with several oral lesions and so severe with loss of multiple pieces dental is very rare.Patient in the fourth decade of life with a family history of autoimmunity who debuts with severe oral manifestations with a requirement for extraction of 14 teeth, severe gingivitis, smooth tongue and glossitis, aphthous stomatitis, ulcers, lip edema and angular cheilitis, without clear cause, and in management by the oral pathology group. Associated with this, there was vulvo-perineal compromise with ulcerated, inflammatory, erythematous and infiltrated lesions. It was initially suspected of Behçet's disease, HLA B51 was performed, it was negative, also, negative pathergy test, and no other suggestive systemic findings. A vulvar biopsy was performed with marked edema of the dermis, dilated lymphatics with perivascular and interstitial lymphoplasmacytic infiltrate and noncaseating granulomas, negative for microorganisms. At this level, it was compatible with MCD, without presence of gastrointestinal symptoms and calprotectin levels in stool in normal range. High and low endoscopic studies and capsule endoscopy were performed in small intestine, without alterations, it was managed by dermatology with topical steroids and by dentistry with dental implants. It was considered patient with inflammatory bowel disease (IBD) type CD with severe extraintestinal manifestations (EIM), although it did not present compromise intestinal treatment, it was decided to start treatment with anti-TNF initially with adalimumab developing paradoxical psoriasis, later treatment with infliximab, again with presentation of severe paradoxical psoriasis, for which it was suspended. Cyclosporine was also used as an immunomodulator, presenting intolerable tachycardia. 18 months after these symptoms, she presented episcleritis of the left eye and begins with colicky abdominal pain and average diarrheal stools 5-a-day, it was performed high and low endoscopic studies without alterations and new capsule endoscopic of small intestine documenting Crohn's enteritis involving the duodenum, jejunum and ileum, considering a patient with IBD type CD, with EIM with vulvo-perineal compromise, severe oral involvement and episcleritis. Currently is under management with azathioprine and Ustekinumab, with clinical improvement significant.MCD represents a diagnostic challenge, it can debut without gastrointestinal involvement, and its clinical and histopathological findings simulate other entities. A timely diagnosis is required to seek early benefit in the patient.