Extraintestinal manifestations of Crohn's disease affect 26% to 45% of patients. Metastatic, or cutaneous, Crohn's disease (CD) is a rare complication in which cutaneous skin lesions, histopathologically similar to CD, manifest in noncontiguous fashion with or without involvement of the gastrointestinal tract (GI). While limited data is available regarding effective regiments for cutaneous CD, available therapy can be delayed due to delayed diagnosis. Moreover, diagnosis of metastatic CD can be masqueraded by other skin conditions, such as Hidradenitis Suppurativa, as up to 20% of patients present without GI symptoms but are later found to have endoscopic evidence of CD. Herein we present the first case of cutaneous Crohn's disease without any GI involvement.Methods:
The patient is a 47-year-old female with a history of metastatic CD of gluteal folds, vulva, and perineum that started at age 33. Although, she had no GI symptoms at the time of presentation, she was diagnosed with cutaneous CD by skin biopsy demonstrating dermal based dense lymphoplasmacytic inflammatory changes with easily identifiable noncaseating granulomas. Initially she was treated with oral steroid and antibiotics, with transition to biologics for refractory disease. Over 8 years she received multiple biologics including infliximab, certolizumab and adalimumab with limited degrees of response. Notably, her treatment with biologics was frequently interrupted because of her insurance coverage and that may have contributed to her refractory disease. Recently she was treated with ciprofloxacin, metronidazole, methotrexate, and prednisone. Endoscopic evaluations with biopsy since the onset of her disease have not demonstrated any evidence of inflammatory bowel disease. She again presented to us with increasing fistula drainage, pain and discomfort for 2 months' duration and was found to be septic secondary to severe wound infection.Results:
She was initially started on intravenous piperacillin/tazobactam and vancomycin. Gastroenterology, plastic surgery, and dermatology were consulted, and she was discharged with clindamycin and methotrexate, with plans to begin adalimumab as an outpatient after dermatology follow up.Conclusions:
CD restricted to the skin without GI manifestation is rare. Based on our literature search, this case appears to be the first of cutaneous CD without any gastrointestinal involvement. One other patient has been reported to have a case of cutaneous CD without major GI complaints. While their patient had endoscopic evidence of CD involving the terminal ileum, such findings have been absent in our patient after multiple endoscopic evaluations over 10 years. Our case further highlights the need for histopathologic confirmation along with detailed history of present illness in patients presenting with ulcerated nodules and plaques with or without drainage. Once diagnosed, options for acute symptoms includes systemic glucocorticoids, immunomodulators, and antibiotics, although no standardized therapy response or duration is available. Alternatives for refractory disease include a combination of surgery, antibiotics, and biologics.