골수 이형성 증후군에 발생한 특이한 급성 발열성 호중구성 피부병(Sweet 증후군) 1례

Abstract

Sweet's syndrome, initially described in 1964 as acute febrile neutrophilic dermatosis by Sweet, is characterized by five cardinal feature: fever; neutrophilia; abrupt appearance of erythematous, pailful, cutaneous plaque, primarily located on the upper extremities, head and neck; a dermal infiltrate of mature neutrophils; and a rapid response to steroid therapy. More than 500 cases of Sweet's syndrome have been documented since original description of Sweet, of which, approximately, 15∼20 percent of published cases occurred in patient with hematologic malignancy. The authors describe an unusual case of Sweet's syndrome in a man, who affected with myelodysplastic syndrome(RAEB). A 56-year-old male patient admitted to Yonsei University, Medical Center because of sustained fever and cellulitis of right lower leg. With anemia and thrombocytopenia, the bone marrow study revealed myelodysplastic syndrome(RAEB). On 4th hospital day, with the development of pneumonia, he was treated with mechanical ventilation and broad spectrum of antibiotics. Although the treatment of pneumonia was successful, erythematous nodules, showing Koebner phenomenon and strong positive pathergy reaction, was appeared on the whole body(21st hospital day). Skin biopsy was taken. Under the diagnosis of Sweet's syndrome associated with myelodysplastic syndrome(RAEB), oral prednisolone 60mg/day was prescribed. The cuteneous lesions were regressed, but the brownish pigmentation was remained, till the patient discharged.