Presentation

A priority face-to-face review was arranged for a 40-year-old Caucasian female with known Behcet’s disease (BD) after receiving smartphone images showing well-demarcated ulcerated lesions on her intestinal stoma (Fig. 1). At review, she described 5 days of malaise, nonspecific abdominal pain, and concurrent painful orogenital ulceration. Inflammatory markers (C-reactive protein and erythrocyte sedimentation rate) were unremarkable. Intramuscular triamcinolone 80 mg resolved symptoms within 1 week, with no recurrence at 6 months.

Fig. 1
figure 1

Smartphone photographs taken by the patient of the ileostomy site. a and b Yellow arrows show two discrete, painful ulcerated lesions with concurrent orogenital ulceration noted

Full size image

Prior to her BD diagnosis, she experienced a non-ST-elevation myocardial infarction requiring coronary stenting at age 33. At 35, she presented with fulminant colitis, which was attributed to Crohn’s disease. This required emergency subtotal colectomy and conversion proctectomy; remission was achieved with short-term oral glucocorticoids. Alongside a history of recurrent non-herpetic orogenital ulceration, a national centre confirmed her BD diagnosis at age 37. Treatment of her BD with daily azathioprine (100 mg) and colchicine (as required) maintained a near disease-free status.

A review of histopathological specimens, prompted by her Behcet’s diagnosis, showed no obvious granulomatous disease, vasculitis, or pathognomonic Crohn’s features. Alongside her most recent flare with concurrent orogenital and stomal ulceration, the team reconsidered their initial diagnosis of Crohn’s disease. The previous episode of colitis is now attributed to BD only.

Discussion

Differentiating Crohn’s and Behcet’s diseases from histopathological specimens is challenging [1, 2]. Although drugs, including infliximab, can treat both conditions, it is important to distinguish between them [2, 3]. Here, concurrent orogenital and stomal ulceration supports that this patient does not have Crohn’s disease, with Behcet’s being the unifying diagnosis.

BD classically affects the ileocecal area, but stoma disease is not widely described [3, 4]. This case presents a rare visible manifestation of Behcet’s intestinal disease, with identification and review facilitated by telemedicine. Telemedicine developments will be increasingly important in managing rheumatic diseases in the post-COVID-19 era [5].