CAJ | 학술논문

患者男,63岁,躯干、四肢反复出现风团14个月,双下肢骨关节疼痛10个月.近1年无明显诱因出现发热,平均每周发热1次,最高可达39℃,发热与皮疹不相关.体检:不规则发热,双腋窝、腹股沟淋巴结肿大.躯干、双上肢、双大腿皮肤可见多个红色水肿性斑块及丘疹.皮损组织病理检查:真皮浅层血管周围中性粒细胞及淋巴细胞浸润.实验室检查:白细胞增高,血细胞沉降率和C反应蛋白升高.免疫蛋白电泳示在γ区可见一条单克隆IgM κ成分.腹部超声:肝轻度增大,肝囊肿,脾轻度增大.PET-CT:双侧腋窝、双侧髂外血管旁、双侧腹股沟区及双腘窝淋巴结肿大,双膝关节腔积液,颈椎、腰椎退行性变.全身骨扫描:双肩、双肘、双膝关节现象异常,考虑关节炎性改变.诊断:Schnitzler综合征.治疗:口服泼尼松30 mg/d,患者第2天体温即恢复正常,病情好转.后加用雷公藤多苷片20 mg每日3次,泼尼松逐渐减量至15 mg/d,病情控制理想,未再出现发热、皮疹及其他不适,骨关节疼痛明显减轻.
환자남,63세,구간、사지반복출현풍단14개월,쌍하지골관절동통10개월.근1년무명현유인출현발열,평균매주발열1차,최고가체39℃,발열여피진불상관.체검:불규칙발열,쌍액와、복고구림파결종대.구간、쌍상지、쌍대퇴피부가견다개홍색수종성반괴급구진.피손조직병리검사:진피천층혈관주위중성립세포급림파세포침윤.실험실검사:백세포증고,혈세포침강솔화C반응단백승고.면역단백전영시재γ구가견일조단극륭IgM κ성분.복부초성:간경도증대,간낭종,비경도증대.PET-CT:쌍측액와、쌍측가외혈관방、쌍측복고구구급쌍객와림파결종대,쌍슬관절강적액,경추、요추퇴행성변.전신골소묘:쌍견、쌍주、쌍슬관절현상이상,고필관절염성개변.진단:Schnitzler종합정.치료:구복발니송30 mg/d,환자제2천체온즉회복정상,병정호전.후가용뢰공등다감편20 mg매일3차,발니송축점감량지15 mg/d,병정공제이상,미재출현발열、피진급기타불괄,골관절동통명현감경.
A 63-year-old man presented with non-pruritic urticaria for 14 months,fever of unknown origin for nearly 12 months,and arthralgias of both lower limbs for 10 months.The fever,which was unrelated to the occurrence of skin lesions,appeared once a week on average,and might be as high as 39 ℃.Physical examination revealed irregular fever and lymphadenopathy in both inguinal and axillary regions.Skin lesions,consisting of rosecolored,slightly elevated plaques and papules,affected the trunk,both upper extremities and thighs.Skin biopsy showed perivascular infiltration of neutrophils and lymphocytes in the superficial dermal layer.Laboratory examination exhibited leukocytosis,elevated erythrocyte sedimentation rate and increased C-reactive protein level.Serum protein electrophoresis revealed an IgM-κ component in the γ-globulin zone.Radiographic examination suggested slight hepatosplenomegaly and hepatic cysts.Positron emission tomography/computed tomography (PET-CT) revealed bilateral knee joint effusion,degeneration of cervical vertebrae and lumbar vertebrae,as well as lymphadenectasis in both axillary,inguinal and popliteal regions as well as around external iliac vessels.Wholebody bone scan showed abnormalities of bilateral shoulder joints,elbow joints and knee joints,which were suggestive of arthritis.A diagnosis of Schnitzler syndrome was made.The patient was given prednisone 30 mg per day,and body temperature returned to normal with the improvement of condition on the second day.Subsequently,glucoside tripterygium total 20 mg thrice daily was additionally given,and the dose of prednisone was gradually decreased to 15 mg per day,with a satisfactory control of condition,disappearance of fever,skin eruptions or other discomfort,and marked relief of ostalgia and arthralgia.