sweet outcome for a rare inflammatory condition · 2020. 1. 30. · cohen pr1 kr. sweet’s...

89 NZMJ 19 February 2016, Vol 129 No 1430ISSN 1175-8716 © NZMAwww.nzma.org.nz/journal

CLINICAL CORRESPONDENCE

Sweet outcome for a rare inflammatory condition

Salah Abdel Jalil, Owais Abdul-Kafi, James Smith, Ala’ Abdel Jalil

ABSTRACTSweet syndrome is a rare inflammatory condition that was first described by Douglas Sweet in 1964 as an acute febrile neutrophilic dermatosis.1 It can be associated with infections, inflammatory conditions, pregnancy, drugs, and malignancy. It is usually divided into three subtypes based on etiology: classical (idiopathic); malignancy-associated; and drug-induced.

We describe a patient with classical Sweet syndrome who had a dramatic response to corticosteroids. Our patient met the major criteria for diagnosis (positive histopathology and an abrupt onset of a painful rash), along with 4 minor criteria (fever, preceding upper respiratory tract infection, dramatic response to steroids, and leukocytosis).

PresentationA 47-year-old Caucasian female with

an unremarkable past medical history presented to the emergency room with a chief complaint of fever and chills for 2 days. On the morning of her presentation, she developed a non-pruritic, tender rash over her extremities that started as papules and plaques and rapidly progressed into pseudovesicular lesions. She also had cough and malaise over the preceding three weeks. She denied any antibiotic or non-steroidal drug exposure before her illness. She also denied anorexia, weight loss, abdominal pain, blood in her stool, or changes in bowel habits.

Although she was allergic to penicillin, this rash was not similar to the macular rash she developed after penicillin. She smoked cigarettes, and admitted to occasional marijuana and cocaine use.

AssessmentOn physical exam, she had a temperature

of 38°C, blood pressure of 162/87 mmHg, pulse rate of 120 beats per minute, and was in no apparent distress. Skin exam revealed tender, erythematous papules and plaques (Figure 1), along with pseudove-sicular lesions with raised edges and central pallor resembling ‘targetoid- lesions’ of different stages (Figure 2). Some lesions developed eschar formation over the upper

and lower extremities. The rash notably spared the trunk, palms and soles. She did not have any lymphadenopathy or organo-megally. The rest of her physical exam was unremarkable.

She was admitted for further evaluation. Laboratory tests revealed a white blood cell count of 12,400/Ul with 80% neutrophils and evidence of mild iron deficiency anaemia.

Chest x-ray was negative for pneumonia. Collagen vascular disease antibodies, HIV, cryoglobulins, and serum immunoglobulin levels were all within the normal limits. Hepatitis C antibody was positive, but PCR and genotype testing were negative and liver enzymes were within normal limits. Occult malignancy work-up with a mammogram and upper and lower endoscopy was negative, and she did not have manifes-tations of a lymphoproliferative disease. Hematologic malignancy was felt less likely, so bone marrow biopsy was deferred. A skin biopsy was obtained, which demonstrated dense neutrophilic dermatosis without evidence of leukocytoclastic vasculitis, suggestive of Sweet syndrome, (Figure 3).

ManagementThe patient was started on prednisone 40

mg daily and had a dramatic improvement in her skin lesions within the first few days (Figure 4). The precipitating aetiology of this patient’s Sweet syndrome was felt to be a preceding upper respiratory tract infection.



Figure 1: Lower extremities image shows erythematous papules and plaques bilaterally.



Figure 2: Bullous lesions on lateral aspect of right hand and forearm.

Figure 3: Hematoxylin & eosin stain microscopy (100X), showing dense neutrophilic infiltration of dermis, without evidence of leukocytoclastic vasculitis.



DiscussionSweet syndrome is characterised by

an abrupt onset of painful skin papules, plaques, or nodules. Fever and leukocytosis usually accompany the cutaneous lesions. Sweet syndrome has been observed in asso-ciation with different conditions and has been classified into classical, malignancy-as-sociated, and drug-induced.2

Classical (idiopathic) Sweet syndrome is most frequently preceded by a gastroin-testinal, upper respiratory tract, or other infection. It might also be associated with inflammatory conditions like inflam-matory bowel disease, pregnancy, and, less frequently, other autoimmune diseases.2

Malignancy-associated Sweet syndrome, which constitutes 20–25 % of cases, is more common in older patients, and is most often associated with a haematological malignancy, especially acute myelogenous leukaemia (AML) and the myeloprolifer-ative disorders.3 The rash could precede, accompany, or follow the malignancy diag-nosis. Drug-induced Sweet syndrome has a temporal relation with certain medications, including non-steroidal anti-inflammatory medications, colony-stimulating factors, antibiotics, contraceptives, and other medications. The skin lesions usually resolve after the offending medication is discontinued.4

According to the screening procedure originally suggested by Su and Liu in 1986,

and modified by Von Den Driesch in 1994,5,6 the diagnosis of classical Sweet syndrome requires both major criteria: a) an abrupt onset of painful erythematous skin papules or nodules; and b) histopathologic evidence of dense neutrophilic infiltrate without evidence of leukocytoclastic vasculitis. In addition, the diagnosis requires meeting at least two of the four minor criteria: a) fever >38°C; b) association with an underlying haematologic or solid malignancy, preg-nancy, inflammatory condition (eg, IBD), or preceding upper respiratory or gastroin-testinal infection; c) excellent response to treatment with corticosteroids or potassium iodide; and d) at least three of the four abnormal laboratory values (ESR >20mm/hour, elevated CRP, WBC count >8,000/Ul, neutrophils >70% of WBC count).4

Characteristic skin lesions of Sweet syndrome are in the form of tender erythem-atous papules or nodules which can develop into erythematous plaques with a char-acteristic papillomatous surface.1 Bullous Sweet syndrome and subcutaneous Sweet syndrome are less common manifestations.7 A skin biopsy should be performed whenever possible. A punch biopsy is usually obtained for histologic examination, and microbial stains should also be performed to rule out an infectious aetiology.7

Systemic corticosteroids therapy is the first-line treatment for Sweet syndrome, and usually results in dramatic clinical improvement.8 Colchicine, Dapsone, and

Figure 4: Lower extremities few days after treatment with corticosteroids was started, showing dramatic healing of skin lesions.



potassium iodide are additional effective therapies. Symptoms often begin to improve within 48 hours and skin lesions usually resolve within one to two weeks. Relapse

may occur after tapering or discontinuation of glucocorticoids, and may be more likely to occur in patients with malignancy-asso-ciated disease.9

Acknowledgements: Authors appreciate Dr Tarek Bishara/Pathology Department, and Sidney Glass/Media Center,

at McLeod Regional Medical Center in Florence, South Carolina, for help in obtaining microscopic and photographic images, respectively, for this patient.

Author information:Salah Abdel Jalil, Resident in general surgery department, Najah National University Hospital, Aseera Street, Nablus , West Bank-Palestine; Owais Abdul-Kafi, University of

Missouri School of Medicine, One Hospital Drive, MA204, DC018.00, Columbia, MO 65212, US; Ala’ A Abdel Jalil, Division of Gastroenterology & Hepatology, CE 405 – DC 043.00, University

of Missouri-Columbia; James Smith, Hematology & Oncology, McLeod Regional Medical Center, 506 E. Cheves St. Florence, SC 29506, US.

Corresponding author: Salah Abdel Jalil, Resident in general surgery department, Najah National University

Hospital, Aseera street, Nablus , West [email protected]

URL:www.nzma.org.nz/journal/read-the-journal/all-issues/2010-2019/2016/vol-129-no-1430-19-

february-2016/6818

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2. Cohen PR. Sweet’s syndrome--a compre-hensive review of an acute febrile neutrophilic dermatosis. Orphanet journal of rare diseases. 2007;2:34. PubMed PMID: 17655751. Pubmed Central PMCID: PMC1963326. Epub 2007/07/28. eng.

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4. Kaszewski S, Czajkowski R, Protas-Drozd F, Placek

W, Jakubowski S. Sweet’s syndrome with idiopathic thrombocythemia. Postepy dermatologii i alergologii. 2014 Feb;31(1):47-52. PubMed PMID: 24683399. Pubmed Central PMCID: 3952057.

5. Hospach T, von den Driesch P, Dannecker GE. Acute febrile neutrophilic dermatosis (Sweet’s syndrome) in childhood and adolescence: two new patients and review of the literature on associ-ated diseases. European journal of pediatrics. 2009 Jan;168(1):1-9. PubMed PMID: 18830624. Epub 2008/10/03. eng.

6. Jo MS, Lim YB, Shin HK, Choe J, Seul JH, Jang TJ. A Case Report of Sweet’s Syndrome with Parotitis. Archives of

plastic surgery. 2012 Jan;39(1):59-62. PubMed PMID: 22783494. Pubmed Central PMCID: 3385293.

7. Rochet NM, Chavan RN, Cappel MA, Wada DA, Gibson LE. Sweet syndrome: clinical presentation, associations, and response to treat-ment in 77 patients. J Am Acad Dermatol. 2013 Oct;69(4):557-64. PubMed PMID: 23891394.

8. Cohen PR1 KR. Sweet’s syndrome: a review of current treatment options. Am J Clin Dermatol. 2002.

9. Choi HJ CS, Lee MW, Choi JH, Moon KC, Koh JK. A case of recurrent Sweet’s syndrome in an 80-year-old man: a clue to an underlying malignancy. Int J Dermatol. 2006.

REFERENCES:

sweet outcome for a rare inflammatory condition · 2020. 1. 30. · cohen pr1 kr. sweet’s...

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