Lymphangioma Circumscriptum, A Cutaneous Manifestation of Crohnʼs Disease

Abstract

Purpose: A 39-year-old female referred to GI clinic for complaint of bright red blood per rectum that was initially attributed to her hemorrhoids. Blood was noted with every bowel movement, coughing, and intercourse. Associated symptoms were abdominal cramps in the morning that was relieved with 4-5 clusters of loose stool and 20 lbs of unintentional weight loss. Furthermore, she reported a rash on her buttocks that appeared to be enlarging despite using mupirocin and clotrimazole cream. Pertinent physical examination showed two large inflamed 2-cm skin tags protruding from the anal canal and multiple verrucous pink papules coalescing into confluent 2-4 cm plaques on both buttocks. Colonoscopy showed a strictured and inflamed IC valve and scattered aphthous ulcerations in the descending and sigmoid colon. Pathology showed mild colitis only in the rectum. CT enterography demonstrated acute inflammatory changes involving the terminal ileum and blind ending sinus tracts extending from TI into the mesentery and cecum suggesting possible entero-cecal fistula. Patient was referred to dermatology and a shave biopsy was performed of the perianal plaques. Pathology revealed many vascular spaces resembling lymphatics with substantial epidermal hyperplasia with elongated retes suggestive of lymphangioma circumscriptum (LC). Patient was diagnosed with Crohn's disease with LC. Patient was started on azathioprine 50 mg daily and prednisone 40 mg taper with gradual resolution of the skin lesions and a decrease in abdominal pain and bleeding. A variety of dermatologic extraintestinal manifestations are associated with inflammatory bowel disease. Most common are erythema nodosum, pyoderma gangrenosum, and aphthous stomatitis. LC is a rare proliferation of the lymphatic system that can be caused by infection, fistulizing Crohn's disease, and radiation therapy. The proposed mechanism for formation is direct mechanical disruption caused by fibrosis associated with fistula tract formation. Composed of dilated native lymphatic channels in the cutaneous and subcutaneous tissues, its manifestation can vary from ill-defined sessile masses resembling cobblestones and pedunculated polypoid lesions to discrete collections of vesicles resembling frog spawn. While treatment options include surgical resection, which is frequently unsuccessful with rapid relapse, sclerotherapy, vaporization with CO2 laser, or observation, there is currently little literature to guide treatment in patients with Crohn's disease. This case demonstrates the importance of recognizing acquired LC as a dermatologic extraintestinal manifestation of Crohn's disease and that further research into appropriate long-term clinical management is necessary.