J Rheum Dis.  2015 Jun;22(3):175-179. 10.4078/jrd.2015.22.3.175.

A Case of Sweet's Syndrome Diagnosed Concomitantly with Sjogren's Syndrome

Affiliations
  • 1Department of Internal Medicine, Kangbuk Samsung Hospital, Sungkyunkwan University School of Medicine, Seoul, Korea. mdahnjk@skku.edu

Abstract

Sweet's syndrome is an uncommon reactive dermatoses characterized by fever, polymorphonuclear leukocytosis, painful erythematous plaques, and dense dermal infiltrate of neutrophils. Sweet's syndrome can be associated with several diseases, including infectious diseases, malignant tumors, and autoimmune diseases. However, no case of Sweet's syndrome associated with Sjogren's syndrome (SjS) has been reported in Korea. A 44-year-old woman presented with acute pustular rashes and fever. The patient had multiple papulopustular skin rashes, and complained of fever, chills, and headache. Our patient had the characteristic clinical and histopathological features of Sweet's syndrome, in association with SjS, diagnosed by salivary gland scan, positive anti-SS-A/SS-B antibody, and sicca symptoms simultaneously. Thus, we report on a case of a patient with Sweet's syndrome with concomitant diagnosis of SjS.

Keyword

Sweet syndrome; Sjogren's syndrome

MeSH Terms

Adult
Autoimmune Diseases
Chills
Communicable Diseases
Diagnosis
Exanthema
Female
Fever
Headache
Humans
Korea
Leukocytosis
Neutrophils
Salivary Glands
Sjogren's Syndrome*
Skin Diseases
Sweet Syndrome*

Figure

  • Figure 1. Erythematous papulopustular rash over face (A), neck (B), left forearm (C), and right auricle (D).

  • Figure 2. (A) Diffuse swelling and perimuscular inflammatory infiltration of the left psoas muscle. (B) After treatment of Sweet's syndrome, significant remission of swelling and perimuscular inflammatory infiltration has occurred.

  • Figure 3. The salivary gland scan shows decreased uptake and delayed washout of the radiotracer by both parotid glands and both submandibular glands. This finding suggests primary Sjögren's syndrome. ANT: anterior view, LAT: lateral view, LT: left, RT: right, PG: parotid gland, EF: ejection fraction, SMG: submandibular gland.

  • Figure 4. (A) The epidermis desquamated and diffuse infiltration of inflammatory cells in deep dermis (H&E, ×40). (B) An infiltration of predominantly neutrophils, without evidence of vasculitis (H&E, ×400).


Reference

1. Sweet RD. An acute febrile neutrophilic dermatosis. Br J Dermatol. 1964; 76:349–56.
2. Gunawardena DA, Gunawardena KA, Ratnayaka RM, Vasanthanathan NS. The clinical spectrum of Sweet's syndrome (acute febrile neutrophilic dermatosis)-a report of eighteen cases. Br J Dermatol. 1975; 92:363–73.
Article
3. Cohen PR, Talpaz M, Kurzrock R. Malignancy-associated Sweet's syndrome: review of the world literature. J Clin Oncol. 1988; 6:1887–97.
Article
4. White JM, Mufti GJ, Salisbury JR, du Vivier AW. Cutaneous manifestations of granulocyte colony-stimulating factor. Clin Exp Dermatol. 2006; 31:206–7.
Article
5. Colović MD, Janković GM, Novak AZ, Strahinja RM, Colović NR. Sweet's syndrome associated with paracentric inversion of chromosome 3q in a patient with multiple myeloma. Eur J Haematol. 1996; 57:188–9.
6. Cohen PR. Sweet's syndrome: a comprehensive review of an acute febrile neutrophilic dermatosis. Orphanet J Rare Dis. 2007; 2:34.
Article
7. Prystowsky SD, Fye KH, Goette KD, Daniels TE. Acute febrile neutrophilic dermatosis associated with Sjögren's syndrome. Arch Dermatol. 1978; 114:1234–5.
Article
8. Osawa H, Yamabe H, Seino S, Fukushi K, Miyata M, Inuma H, et al. A case of Sjögren's syndrome associated with Sweet's syndrome. Clin Rheumatol. 1997; 16:101–5.
Article
9. Mrabet D, Saadi F, Zaraa I, Chelly I, Sahli H, Ben Osmane A, et al. Sweet's syndrome in a patient with rheumatoid arthritis, Sjögren's syndrome and lymph node tuberculosis. BMJ Case Rep. 2011; Feb 2 [Epub].DOI: DOI: 10.1136/bcr.07.2010.3137.
10. Su WP, Liu HN. Diagnostic criteria for Sweet's syndrome. Cutis. 1986; 37:167–74.
11. von den Driesch P. Sweet's syndrome (acute febrile neutrophilic dermatosis). J Am Acad Dermatol. 1994; 31:535–56. quiz 557-60.
Article
12. Medeiros S, Santos R, Carneiro V, Estrela F. Sweet syndrome associated with Hashimoto thyroiditis. Dermatol Online J. 2008; 14:10.
Article
13. Cohen PR, Kurzrock R. Sweet's syndrome revisited: a review of disease concepts. Int J Dermatol. 2003; 42:761–78.
Article
Full Text Links
  • JRD
Actions
Cited
CITED
export Copy
Close
Share
  • Twitter
  • Facebook
Similar articles
Copyright © 2024 by Korean Association of Medical Journal Editors. All rights reserved.     E-mail: koreamed@kamje.or.kr