A Sweet Case of Crohnʼs

Abstract

We report a case of Crohn's disease and Classical Sweet's syndrome, highlighting a unique presentation. RA is a 19 year old male with Crohn's disease on 6-mercaptopurine (6-MP) and mesalamine for over 2 years who was transferred from an outside hospital for evaluation of neutropenic fever and skin lesions. On arrival, patient was febrile and tachycardic with pancytopenia: WBC: 1.2, Hb7.6, Hct 23.7, Plt 85, ANC 340. He reported a 4 day history of initially painless erythematous lesions on various parts of his body and oral mucosa. He also had night sweats, fevers, and chills, but denied diarrhea, bloody stools, or abdominal pain. Dermatologic exam revealed painful hemorrhagic dusky, necrotic papules surrounded by a pink violaceous rim on the dorsum of his hands, upper lip, left eyelid, left cheek, and right knee (Image 1); as well as 3 shallow erythematous ulcers on his tongue. CRP 24, ESR >140. HIV, HSV, VDRL were negative. Skin and bone marrow biopsies were obtained. Patient was started on vancomycin and zosyn and 6-MP and mesalamine were held. Despite antibiotic therapy, skin lesions progressed to draining hemorrhagic ulcers with edema and spreading necrosis (Image 2) with continued fluctuating fevers. Given the clinical course and progression of skin lesions, the diagnosis of Sweet's syndrome was considered. Malignancy work-up was negative and bone marrow biopsy was consistent with drug induced myelosuppression secondary to 6-MP. Skin biopsy revealed neutrophillic infiltrate consistent with Sweet's syndrome. The patient was started on prednisone 60mg with significant improvement in skin lesions (Image 3). Sweet's syndrome, also known as acute febrile neutrophilic dermatosis, is an uncommon inflammatory disorder characterized by abrupt painful, edematous, and erythematous skin lesions often associated with fever and leukocytosis. It is often missed on presentation as it closely resembles severe infections, such as pyoderma gangrenosum and ecthyma gangrenosum. There are 3 subtypes of Sweet's syndrome. This case describes Classical Sweet's syndrome in a young male, which is the subtype associated with IBD and usually seen in older females, given that malignancy was ruled out. Sweet's syndrome responds rapidly to systemic steroids and even topical steroids in milder cases. This case also stresses the importance of continued monitoring for cytopenia in IBD patients on chronic myelosuppressive therapy.Figure 1Figure 2Figure 3