Journal of Crohn's and Colitis Advance Access published April 18, 2015 Journal of Crohn's and Colitis, 2015, 1–1 doi:10.1093/ecco-jcc/jjv049 Letter to the Editor
Letter to the Editor Ulcerative Colitis Flare with a Sweet Ending Benjamin Robert Disneya, Sheldon Charles Coopera,b, Sauid Ishaqa Department of Gastroenterology, Russells Hall Hospital, Dudley, UK bDepartment of Gastroenterology, University Hospitals Birmingham, Birmingham, UK
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Corresponding author: Dr Sauid Ishaq, Department of Gastroenterology, Dudley Group of Hospitals NHS Trust, Russells Hall Hospital, Dudley DY1 2HQ, UK. Email: [email protected] A 43-year-old lady with a known history of ulcerative colitis (UC) was admitted with a 2-day history of profuse bloody diarrhoea and generalised abdominal tenderness. On examination, the patient looked unwell with a temperature of 38.7°C, pulse rate of 98 beats per min and blood pressure 138/68 mmHg. Blood testing showed white blood cell count of 11.6 x 109/l with a differential neutrophil count of 9.65 x 109/l. Haemoglobin, platelets, renal function and liver function tests were within normal limits. C-reactive protein was elevated at 249 mg/l. Blood cultures were negative. An abdominal radiograph was unremarkable. An urgent flexible sigmoidoscopy showed severe Mayo 3 colitis. The patient continued with infliximab and prednisolone treatment with subsequent improvement of both the skin lesions and colitis. On Day 7 of admission, the patient developed tense pustular lesions on her chest, legs and arms. The patient tested negative for varicella zoster and herpes simplex IgM. A dermatology review and punch biopsies from the skin were taken. The biopsy features were in keeping with the late stage of Sweet’s syndrome (Figure 1). The
patient continued with infliximab and prednisolone treatment, with subsequent improvement of both the skin lesions. Sweet’s syndrome, also known as acute febrile neutrophilic dermatosis, is a rare cutaneous manifestation of inflammatory bowel disease (IBD). Diagnosis is made on clinical history and histopathology, which shows a dense neutrophilic infiltrate, epidermal pustules and oedema. It may occur while patients are on immunosuppressive therapy. The most effective treatment of Sweet’s syndrome is with systemic corticosteroids. Other reported treatment modalities include colchicines, indomethacin, clofazimine, cyclosporin and dapsone.1 Sweet’s syndrome associated with IBD tends to be more common in women (87%) and is usually associated with active disease.2 Pyoderma gangrenosum, another neutrophilic dermatosis, develops in 1–3% of IBD cases whereas Sweet’s syndrome is considerably rarer.3 Sweet’s syndrome can be mistaken as herpes infection because skin lesions may show vesicles. This can lead to reduction in immunosuppressant treatment resulting in worsening of symptoms. The above case highlights the need for clinicians to be aware of the signs of Sweet’s syndrome, its association with UC and its treatment.
References 1. Cohen PR. Sweet’s syndrome a comprehensive review of an acute febrile neutrophilic dermatosis. Orphanet J Rare Dis 2007;26;2–34. 2. Ali M, Duerksen DR. Ulcerative colitis and Sweet’s syndrome: a case report and review of the literature. Can J Gastroenterol 2008;22:296–8. 3. Marzano AV, Menicanti C, Crosti C, Trevisan V. Neutrophilic dermatoses and inflammatory bowel diseases. G Ital Dermatol Venereol 2013;148:185–96.
Figure 1. Skin biopsy showing dense dermal inflammatory cell infiltrate composed of lymphocytes and histiocytes, with scattered neutrophil clusters, associated with nuclear dusts / leukocytoclasia. The features are in keeping with the late stage of Sweet’s syndrome.
Copyright © 2015 European Crohn’s and Colitis Organisation (ECCO). Published by Oxford University Press. All rights reserved. For permissions, please email: [email protected]
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Letter to the Editor Ulcerative Colitis Flare with a Sweet Ending Benjamin Robert Disneya, Sheldon Charles Coopera,b, Sauid Ishaqa Department of Gastroenterology, Russells Hall Hospital, Dudley, UK bDepartment of Gastroenterology, University Hospitals Birmingham, Birmingham, UK
a
Corresponding author: Dr Sauid Ishaq, Department of Gastroenterology, Dudley Group of Hospitals NHS Trust, Russells Hall Hospital, Dudley DY1 2HQ, UK. Email: [email protected] A 43-year-old lady with a known history of ulcerative colitis (UC) was admitted with a 2-day history of profuse bloody diarrhoea and generalised abdominal tenderness. On examination, the patient looked unwell with a temperature of 38.7°C, pulse rate of 98 beats per min and blood pressure 138/68 mmHg. Blood testing showed white blood cell count of 11.6 x 109/l with a differential neutrophil count of 9.65 x 109/l. Haemoglobin, platelets, renal function and liver function tests were within normal limits. C-reactive protein was elevated at 249 mg/l. Blood cultures were negative. An abdominal radiograph was unremarkable. An urgent flexible sigmoidoscopy showed severe Mayo 3 colitis. The patient continued with infliximab and prednisolone treatment with subsequent improvement of both the skin lesions and colitis. On Day 7 of admission, the patient developed tense pustular lesions on her chest, legs and arms. The patient tested negative for varicella zoster and herpes simplex IgM. A dermatology review and punch biopsies from the skin were taken. The biopsy features were in keeping with the late stage of Sweet’s syndrome (Figure 1). The
patient continued with infliximab and prednisolone treatment, with subsequent improvement of both the skin lesions. Sweet’s syndrome, also known as acute febrile neutrophilic dermatosis, is a rare cutaneous manifestation of inflammatory bowel disease (IBD). Diagnosis is made on clinical history and histopathology, which shows a dense neutrophilic infiltrate, epidermal pustules and oedema. It may occur while patients are on immunosuppressive therapy. The most effective treatment of Sweet’s syndrome is with systemic corticosteroids. Other reported treatment modalities include colchicines, indomethacin, clofazimine, cyclosporin and dapsone.1 Sweet’s syndrome associated with IBD tends to be more common in women (87%) and is usually associated with active disease.2 Pyoderma gangrenosum, another neutrophilic dermatosis, develops in 1–3% of IBD cases whereas Sweet’s syndrome is considerably rarer.3 Sweet’s syndrome can be mistaken as herpes infection because skin lesions may show vesicles. This can lead to reduction in immunosuppressant treatment resulting in worsening of symptoms. The above case highlights the need for clinicians to be aware of the signs of Sweet’s syndrome, its association with UC and its treatment.
References 1. Cohen PR. Sweet’s syndrome a comprehensive review of an acute febrile neutrophilic dermatosis. Orphanet J Rare Dis 2007;26;2–34. 2. Ali M, Duerksen DR. Ulcerative colitis and Sweet’s syndrome: a case report and review of the literature. Can J Gastroenterol 2008;22:296–8. 3. Marzano AV, Menicanti C, Crosti C, Trevisan V. Neutrophilic dermatoses and inflammatory bowel diseases. G Ital Dermatol Venereol 2013;148:185–96.
Figure 1. Skin biopsy showing dense dermal inflammatory cell infiltrate composed of lymphocytes and histiocytes, with scattered neutrophil clusters, associated with nuclear dusts / leukocytoclasia. The features are in keeping with the late stage of Sweet’s syndrome.
Copyright © 2015 European Crohn’s and Colitis Organisation (ECCO). Published by Oxford University Press. All rights reserved. For permissions, please email: [email protected]
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