Sweet's syndrome associated with recurrent fever in a patient with trisomy 8 myelodysplastic syndrome.
In the case of a 69-year-old man suffering from recurrent high fever, laboratory data demonstrated elevated inflammatory findings such as neutrophilic leukocytosis and an increase in serum level of C-reactive protein. A nonsteroidal antiinflammatory drug or a low dose of prednisolone proved to be temporarily effective. A bone marrow specimen showed myelodysplastic syndrome (MDS) of the refractory anemia (RA) type with trisomy 8. Seven months after onset, painful erythematous eruptions were found on both legs. Biopsy of a skin lesion demonstrated neutrophic infiltration into the dermis. As a result, Sweet's syndrome (SS) was diagnosed on the basis of clinical and histopathological findings. Administration of prednisolone (30 mg/d) was started and resulted in prompt defervescence and resolution of the lesions within 1 week. We also investigated serum levels of 3 cytokines: interleukin 1beta (IL-1beta), IL-6, and granulocyte colony-stimulating factor (G-CSF). Levels of IL-6 and G-CSF were elevated during the active phase, but the level of IL-1beta did not increase. Because cases of MDS with trisomy 8 and SS or Behçet disease have been reported recently, our findings suggested that cytokine production, which enhances neutrophil function, is elevated in some MDS patients with trisomy 8; that is, not only cytokines but also trisomy 8 may be related to the pathogenesis of SS in MDS. It is thus advisable to watch for development of SS during follow-up of MDS patients with recurrent fever or trisomy 8.
Hattori
Hideki
H
Department of Internal Medicine, Yao Municipal Hospital, Osaka, Japan.
Hoshida
Shiro
S
Yoneda
Shotaro
S
D000368:Aged; D002898:Chromosomes, Human, Pair 8; D005334:Fever / Q000209:etiology*; D016179:Granulocyte Colony-Stimulating Factor / Q000097:blood; D006801:Humans; D007375:Interleukin-1 / Q000097:blood; D015850:Interleukin-6 / Q000097:blood; D008297:Male; D009190:Myelodysplastic Syndromes / Q000150:complications* / Q000188:drug therapy; D011239:Prednisolone / Q000627:therapeutic use; D012008:Recurrence; D016463:Sweet Syndrome / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology*; D014314:Trisomy
D007375:Interleukin-1; D015850:Interleukin-6; D016179:Granulocyte Colony-Stimulating Factor; D011239:Prednisolone