CAJ | 학술논문

骨髓增生异常综合征（MDS）是一种异质性克隆性造血干细胞疾病，少部分MDS患者可并发急性发热性嗜中性皮病（SWEET综合征，SS），其皮肤改变主要为不对称性疼痛性红色丘疹、结节和斑块，后期可进展为脓疱，同时伴发热、白细胞升高及ESR增快，皮肤病理活组织检查（活检）以弥漫分布于真皮浅层的成熟中性粒细胞浸润为特征，糖皮质激素治疗有效而抗感染治疗无效，合并SS的MDS容易进展为急性髓系白血病，预后不佳。该文报道1例接受皮下输液港植入化学治疗后继发SS的MDS患者，患者化学治疗过程顺利，但其皮下输液港植入部位发生胸壁皮肤感染，伴有持续高热，胸壁皮肤切口部位上方有疼痛性红色丘疹，经抗感染治疗无效，皮肤活检结果示真皮浅层中性粒细胞浸润，诊断为MDS并发SS，予糖皮质激素治疗后体温降至正常，皮损愈合，随访示MDS处于完全缓解状态。因此，临床上对于存在发热、痛性红色丘疹且抗感染治疗无效的MDS患者需警惕SS的可能，应及早完善皮肤活检以便早诊断、早治疗。
골수증생이상종합정（MDS）시일충이질성극륭성조혈간세포질병，소부분MDS환자가병발급성발열성기중성피병（SWEET종합정，SS），기피부개변주요위불대칭성동통성홍색구진、결절화반괴，후기가진전위농포，동시반발열、백세포승고급ESR증쾌，피부병리활조직검사（활검）이미만분포우진피천층적성숙중성립세포침윤위특정，당피질격소치료유효이항감염치료무효，합병SS적MDS용역진전위급성수계백혈병，예후불가。해문보도1례접수피하수액항식입화학치료후계발SS적MDS환자，환자화학치료과정순리，단기피하수액항식입부위발생흉벽피부감염，반유지속고열，흉벽피부절구부위상방유동통성홍색구진，경항감염치료무효，피부활검결과시진피천층중성립세포침윤，진단위MDS병발SS，여당피질격소치료후체온강지정상，피손유합，수방시MDS처우완전완해상태。인차，림상상대우존재발열、통성홍색구진차항감염치료무효적MDS환자수경척SS적가능，응급조완선피부활검이편조진단、조치료。
Myelodysplastic syndrome (MDS )is a type of heterogeneous clonal hematopoiefic stem cell disease. A minority of MDS patients could be complicated with acute febrile neutrophilic dermatosis (Sweet's syndrome,SS),mainly characterized by asymmetric painful red papula,node and plaque,andpro-gresses into pustule during the advanced stage. Meanwhile,signs of fever,leukocytosis and accelerated eryth-rocyte sedimentation could occur. Skin pathological biopsy revealed infiltrated matureneutrophilic leukocytes scattering across superficial dermal layer. Glucocorticoid treatment was effective whereas anti-infectious therapy yielded no efficacy. MDS complicated with SS is likely to progress into acute myeloid leukemia with poor prog-nosis. In this article,we reported one MDS case complicated with SS after receiving chemotherapy via implant-able venous access port. The course of chemotherapy was successful,whereas cutaneous infection accompanied by persistent high fever was observed in the implanted site of venous access port. Painful red papula was found above the skin incision of the thoracic wall. Anti-infectious therapy yielded no efficacy. Skin biopsy revealed the signs of neutrophilic leukocyte infiltration into superficial dermal layer. The patient was diagnosed with MDS complicated with SS. Body temperature declined to normal level and the skin wound was healed following glu-cocorticoid administration. Subsequent follow-up demonstrated that the status of MDS was in complete re-sponse. Consequently,the possibility of MDS complicated with SS should be considered for those presenting with fever,painful red papula and no response towards anti-infectious therapy. Skin biopsy should be improved to make early diagnosis and deliver early treatment.