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Sweet Syndrome With Systemic Inflammatory Response Syndrome

Sweet Syndrome With Systemic Inflammatory Response Syndrome Report of a Case A 42-year-old man presented with a several-day history of fever (40°C [104°F]), pneumonia, and an eruption on the forehead, scalp, arms, and trunk. He was initially diagnosed with varicella and was started on acyclovir treatment. However, owing to worsening dyspnea, he was evaluated in the emergency department and admitted for hypotension (blood pressure, 89/60 mm Hg; heart rate, 116 bpm) requiring treatment with vasopressors and intubation for respiratory distress. He was otherwise healthy and taking no medications. A complete blood cell count revealed leukocytosis (white blood cell count [WBC], 13 700/μL) and an elevated neutrophil count (9380 cells/μL) but was otherwise normal. Chest radiography showed an interstitial infiltrate. His C-reactive protein level was elevated at 168 mg/L. (To convert white blood cells and neutrophils to number of cells × 109/L, multiply by 0.001; to convert CRP to nanomoles per liter, multiply by 9.524.) On examination, he had erythematous, edematous papules on the face, scalp, trunk, and arms coalescing into plaques on the neck (Figure 1). Infectious workup findings were negative, including blood, skin, and respiratory tract cultures as well as viral blood tests (for adenovirus, human meta-pneumovirus, influenza A/B, novel H1N1 influenza, parainfluenza 1/2/3, respiratory syncytial virus A/B). His varicella zoster virus (VZV) blood test results were compatible with prior infection (IgM negative and IgG positive). Findings of herpes simplex virus and VZV antigen and culture studies from the skin were negative. A skin biopsy specimen demonstrated subepidermal edema with a superficial dermal neutrophilic infiltrate consistent with Sweet syndrome (Figure 2). Treatment with empirical antibiotics and acyclovir was discontinued, and a methylprednisolone regimen, 40 mg twice daily, was begun. He was subsequently weaned off of vasopressors and extubated within 24 and 48 hours, respectively. Within 5 days he was discharged home with a prescription for prednisone taper. After 1 year, there had been no recurrence. View LargeDownload Figure 1. Erythematous edematous plaques on the neck and upper back. View LargeDownload Figure 2. Perivascular and interstitial neutrophilic infiltrate with subepidermal edema (hematoxylin-eosin, original magnification ×200). Comment Sweet syndrome is a neutrophilic dermatosis that presents with abrupt onset of tender erythematous plaques and a dense dermal neutrophilic infiltrate without evidence of vasculitis on histopathologic examination. Other associated features include fever, leukocytosis with neutrophilia, elevated inflammatory markers, excellent response to systemic corticosteroids, and association with a preceding infection and/or internal process (eg, malignancy, pregnancy, autoimmune disease). Extracutaneous manifestations of the disease have been reported in almost every organ system, including the lungs.1-4 Systemic inflammatory response syndrome (SIRS) is diagnosed when a patient meets 2 of the following criteria: temperature of 36°C or lower or 38°C or higher; heart rate of 90 bpm or higher; respiratory rate of 20 breaths per minute or higher; partial pressure of carbon dioxide in the arterial blood (PaCO2) lower than 32 mm Hg; or WBC count of 12 000 cells/μL or higher or 4000 cells/μL or lower or greater than 10% bands. Sepsis is defined as SIRS caused by infection. Our patient's fever, tachycardia, and leukocytosis met the criteria for SIRS. The negative infectious workup results, lack of response to broad-spectrum antibiotics, and rapid improvement following initiation of corticosteroid therapy suggest that his SIRS was due to Sweet syndrome and not an infectious process. To our knowledge there are only 5 prior reports in the English literature of SIRS associated with Sweet syndrome,1-5 and none of these reports appear in the dermatology literature, making it likely that many dermatologists are unaware of this complication. It is important to recognize SIRS as a rare but important extracutaneous manifestation of Sweet syndrome to avoid delay in instituting systemic corticosteroid treatment because SIRS can progress to multiple organ dysfunction and death. Back to top Article Information Correspondence: Dr Swick, Department of Dermatology, University of Iowa, 200 Hawkins Dr, 40025 PFP, Iowa City, IA 52242 (brian-swick@uiowa.edu). Financial Disclosure: None reported. References 1. Shugarman IL, Schmit JM, Sbicca JA, Wirk B. Easily missed extracutaneous manifestation of malignancy-associated Sweet's syndrome: systemic inflammatory response syndrome [published online July 11, 2011]. J Clin Oncol. 2011;29(24):e702-e70521747081PubMedGoogle ScholarCrossref 2. Sawicki J, Morton RA, Ellis AK. Sweet syndrome with associated systemic inflammatory response syndrome: an ultimately fatal case. Ann Allergy Asthma Immunol. 2010;105(4):321-32320934634PubMedGoogle ScholarCrossref 3. Naz E, Ruano M, Vidaurrázaga C, et al. Sweet's syndrome as a life-threatening dermatosis. Am J Med. 2000;109(1):73-7410991744PubMedGoogle ScholarCrossref 4. Matthews PC, Willatts SM. Sweet's syndrome associated with systemic inflammatory response syndrome. Intensive Care Med. 1998;24(10):1106-11099840248PubMedGoogle ScholarCrossref 5. Otheo E, Ros P, Vázquez JL, et al. Systemic inflammatory response syndrome associated with Sweet's syndrome. Pediatr Crit Care Med. 2002;3(2):190-19312780994PubMedGoogle ScholarCrossref http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Dermatology American Medical Association

Sweet Syndrome With Systemic Inflammatory Response Syndrome

Archives of Dermatology , Volume 148 (8) – Aug 1, 2012

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Publisher
American Medical Association
Copyright
Copyright © 2012 American Medical Association. All Rights Reserved.
ISSN
0003-987X
eISSN
1538-3652
DOI
10.1001/archdermatol.2012.766
Publisher site
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Abstract

Report of a Case A 42-year-old man presented with a several-day history of fever (40°C [104°F]), pneumonia, and an eruption on the forehead, scalp, arms, and trunk. He was initially diagnosed with varicella and was started on acyclovir treatment. However, owing to worsening dyspnea, he was evaluated in the emergency department and admitted for hypotension (blood pressure, 89/60 mm Hg; heart rate, 116 bpm) requiring treatment with vasopressors and intubation for respiratory distress. He was otherwise healthy and taking no medications. A complete blood cell count revealed leukocytosis (white blood cell count [WBC], 13 700/μL) and an elevated neutrophil count (9380 cells/μL) but was otherwise normal. Chest radiography showed an interstitial infiltrate. His C-reactive protein level was elevated at 168 mg/L. (To convert white blood cells and neutrophils to number of cells × 109/L, multiply by 0.001; to convert CRP to nanomoles per liter, multiply by 9.524.) On examination, he had erythematous, edematous papules on the face, scalp, trunk, and arms coalescing into plaques on the neck (Figure 1). Infectious workup findings were negative, including blood, skin, and respiratory tract cultures as well as viral blood tests (for adenovirus, human meta-pneumovirus, influenza A/B, novel H1N1 influenza, parainfluenza 1/2/3, respiratory syncytial virus A/B). His varicella zoster virus (VZV) blood test results were compatible with prior infection (IgM negative and IgG positive). Findings of herpes simplex virus and VZV antigen and culture studies from the skin were negative. A skin biopsy specimen demonstrated subepidermal edema with a superficial dermal neutrophilic infiltrate consistent with Sweet syndrome (Figure 2). Treatment with empirical antibiotics and acyclovir was discontinued, and a methylprednisolone regimen, 40 mg twice daily, was begun. He was subsequently weaned off of vasopressors and extubated within 24 and 48 hours, respectively. Within 5 days he was discharged home with a prescription for prednisone taper. After 1 year, there had been no recurrence. View LargeDownload Figure 1. Erythematous edematous plaques on the neck and upper back. View LargeDownload Figure 2. Perivascular and interstitial neutrophilic infiltrate with subepidermal edema (hematoxylin-eosin, original magnification ×200). Comment Sweet syndrome is a neutrophilic dermatosis that presents with abrupt onset of tender erythematous plaques and a dense dermal neutrophilic infiltrate without evidence of vasculitis on histopathologic examination. Other associated features include fever, leukocytosis with neutrophilia, elevated inflammatory markers, excellent response to systemic corticosteroids, and association with a preceding infection and/or internal process (eg, malignancy, pregnancy, autoimmune disease). Extracutaneous manifestations of the disease have been reported in almost every organ system, including the lungs.1-4 Systemic inflammatory response syndrome (SIRS) is diagnosed when a patient meets 2 of the following criteria: temperature of 36°C or lower or 38°C or higher; heart rate of 90 bpm or higher; respiratory rate of 20 breaths per minute or higher; partial pressure of carbon dioxide in the arterial blood (PaCO2) lower than 32 mm Hg; or WBC count of 12 000 cells/μL or higher or 4000 cells/μL or lower or greater than 10% bands. Sepsis is defined as SIRS caused by infection. Our patient's fever, tachycardia, and leukocytosis met the criteria for SIRS. The negative infectious workup results, lack of response to broad-spectrum antibiotics, and rapid improvement following initiation of corticosteroid therapy suggest that his SIRS was due to Sweet syndrome and not an infectious process. To our knowledge there are only 5 prior reports in the English literature of SIRS associated with Sweet syndrome,1-5 and none of these reports appear in the dermatology literature, making it likely that many dermatologists are unaware of this complication. It is important to recognize SIRS as a rare but important extracutaneous manifestation of Sweet syndrome to avoid delay in instituting systemic corticosteroid treatment because SIRS can progress to multiple organ dysfunction and death. Back to top Article Information Correspondence: Dr Swick, Department of Dermatology, University of Iowa, 200 Hawkins Dr, 40025 PFP, Iowa City, IA 52242 (brian-swick@uiowa.edu). Financial Disclosure: None reported. References 1. Shugarman IL, Schmit JM, Sbicca JA, Wirk B. Easily missed extracutaneous manifestation of malignancy-associated Sweet's syndrome: systemic inflammatory response syndrome [published online July 11, 2011]. J Clin Oncol. 2011;29(24):e702-e70521747081PubMedGoogle ScholarCrossref 2. Sawicki J, Morton RA, Ellis AK. Sweet syndrome with associated systemic inflammatory response syndrome: an ultimately fatal case. Ann Allergy Asthma Immunol. 2010;105(4):321-32320934634PubMedGoogle ScholarCrossref 3. Naz E, Ruano M, Vidaurrázaga C, et al. Sweet's syndrome as a life-threatening dermatosis. Am J Med. 2000;109(1):73-7410991744PubMedGoogle ScholarCrossref 4. Matthews PC, Willatts SM. Sweet's syndrome associated with systemic inflammatory response syndrome. Intensive Care Med. 1998;24(10):1106-11099840248PubMedGoogle ScholarCrossref 5. Otheo E, Ros P, Vázquez JL, et al. Systemic inflammatory response syndrome associated with Sweet's syndrome. Pediatr Crit Care Med. 2002;3(2):190-19312780994PubMedGoogle ScholarCrossref

Journal

Archives of DermatologyAmerican Medical Association

Published: Aug 1, 2012

Keywords: systemic inflammatory response syndrome,sweet's syndrome

References