Sweet Syndrome
| Classification according to ICD-10 | |
|---|---|
| L98.2 | Acute febrile neutrophilic dermatosis [Sweet's syndrome] |
| ICD-10 online (WHO version 2019) | |
The Sweet syndrome is a sudden onset of illness with fever and multiple red nodules and plaques especially in the face and extremities. The disease is also known as acute febrile neutrophil dermatosis because of its clinical symptoms and the increase in a certain type of white blood cell in the blood (called neutrophils ) .
The syndrome was first described by Robert Douglas Sweet in 1964 .
Clinical appearance
Sweet's syndrome is more common in middle-aged women, often after a viral or bacterial infection. It can be associated with a variety of chronic diseases, including rheumatic diseases such as systemic lupus erythematosus and dermatomyositis, or hematological conditions such as acute myeloid leukemia (AML), hairy cell leukemia, and monoclonal gammopathies . Because of its association with AML, it can also be considered a facultative paraneoplastic syndrome . Medication can also be a trigger. The skin manifestations occur mainly on the face and extremities, but do not have to be restricted to these regions. Histologically, these areas show marked edematous changes and a dense infiltration with neutrophils. In addition to the involvement of the skin and blood count, about half of the patients experience arthralgia (joint pain) due to accompanying joint inflammation.
therapy
The disease is treated systemically with glucocorticoids , immunosuppressants, and potassium iodide . The offending disease should be treated. It usually heals within weeks.
literature
- Pschyrembel Clinical Dictionary. 261st edition. de Gruyter, 2007, ISBN 978-3-11-018534-8 .
- D. Terhorst: Basics Dermatology. elsevier, 2005, ISBN 3-437-42136-0 .
- PR Cohen: Sweet's syndrome - a comprehensive review of an acute febrile neutrophilic dermatosis. In: Orphanet J Rare Dis. 2, Jul 26, 2007, p. 34. PMID 17655751 , PMC 1963326 (free full text)
- RD Sweet: An Acute Febrile Neutrophilic Dermatosis. In: Br J Dermatol . 76, Aug-Sep 1964, pp. 349-356. PMID 14201182
Web links
- Pictures of the Sweet Syndrome at DermIS.net: dermis.net
