CAJ | 학술논문

患者男,48岁,入院前3年无明显诱因出现持续性四肢肌肉疼痛、无力;皮肤感觉异常,呈烧灼感,肤色逐渐加深、粗糙;下肢皮肤增厚略发硬,表面呈蛇皮状外观.2年前查肌电图示周围神经病;CT示肝、脾、淋巴结肿大,腹水,胸腔、心包积液.取多个淋巴结活检:淋巴滤泡间血管明显增生,并累及淋巴滤泡,结合组化及特殊染色,提示为Castleman病,透明血管型.3个月前,双大腿出现红斑,逐渐扩大,并出现疼痛感,进而皮损中央颜色逐渐加深变黑,皮损周围皮肤发红;2个月前右大腿内侧出现一黄豆大小结节,破溃,留少许脓液,而后形成浅溃疡.体检:下肢肌肉略萎缩,双上肢肌力5级,双下肢肌力4级.躯干、四肢皮肤粗糙,弥漫性颜色加深,四肢皮肤呈现鱼鳞病样外观,皮肤弥漫性增厚,均以四肢皮肤为著.双大腿内侧均可见约5 cm×2 cm的紫黑色斑片,中央表面粗糙,呈糙纸样外观,表面附有干燥结痂及脱屑,皮损周围皮肤呈环状淡紫红色晕,压之褪色.实验室检查提示甲状腺功能减退、血胰岛素分泌水平降低以及钙磷代谢异常,血清免疫固定电泳IgG型轻链M带弱阳性;多次复查外周血,血小板呈进行性升高.诊断:PEOMS综合征伴多中心Castleman病、血小板增多症.治疗:口服甲泼尼龙和沙利度胺等治疗后,POEMS综合征主要症候群均得到有效改善,但血小板增多现象反而逐渐加重.
환자남,48세,입원전3년무명현유인출현지속성사지기육동통、무력;피부감각이상,정소작감,부색축점가심、조조;하지피부증후략발경,표면정사피상외관.2년전사기전도시주위신경병;CT시간、비、림파결종대,복수,흉강、심포적액.취다개림파결활검:림파려포간혈관명현증생,병루급림파려포,결합조화급특수염색,제시위Castleman병,투명혈관형.3개월전,쌍대퇴출현홍반,축점확대,병출현동통감,진이피손중앙안색축점가심변흑,피손주위피부발홍;2개월전우대퇴내측출현일황두대소결절,파궤,류소허농액,이후형성천궤양.체검:하지기육략위축,쌍상지기력5급,쌍하지기력4급.구간、사지피부조조,미만성안색가심,사지피부정현어린병양외관,피부미만성증후,균이사지피부위저.쌍대퇴내측균가견약5 cm×2 cm적자흑색반편,중앙표면조조,정조지양외관,표면부유간조결가급탈설,피손주위피부정배상담자홍색훈,압지퇴색.실험실검사제시갑상선공능감퇴、혈이도소분비수평강저이급개린대사이상,혈청면역고정전영IgG형경련M대약양성;다차복사외주혈,혈소판정진행성승고.진단:PEOMS종합정반다중심Castleman병、혈소판증다증.치료:구복갑발니룡화사리도알등치료후,POEMS종합정주요증후군균득도유효개선,단혈소판증다현상반이축점가중.
A rare case of POEMS (polyneuropathy,organomegaly,endocrinopathy,M-band,skin changes) syndrome associated with multicentre Castleman's disease and thrombocytosis is reported in a 48-year-old man.Three years prior to the presentation,he developed a series of symptoms without obvious triggers,including persistent myalgia and myasthenia of extremities,abnormal skin sensation (burning),gradually increased roughness and pigmentation of skin,thickening and mild induration of lower-extremity skin giving an ichthyosiform appearance.Two years prior to the presentation,electromyography indicated peripheral neuropathy,and computed tomography (CT) revealed hepatomegaly,splenomegaly,lymphadenectasis,ascites,hydrotborax and hydropericardium.Multiple biopsies of lymph nodes indicated an obvious vascular proliferation between lymphoid follicles with the involvement of lymphoid follicles,which,together with the histochemical findings and special staining results,was suggestive of hyaline-vascular type Castleman's disease.Three months prior to the presentation,painful erythema emerged and gradually spread in both thighs,with a progressive deepening in the colour of central lesions,and flushing of periphery lesions.Two months prior to the presentation,a soybean-sized nodule arised in the erythema on the right inner thigh,which was then ulcerated with a little pus left and a shallow ulcer formed.Physical examination revealed a mild atrophy of lower-extremity muscles,with muscle strength graded as 5 for bilateral upper extremities and as 4 for bilateral lower extremities.The skin was rough,diffusely pigmented and thickened,giving an ichthyosiform appearance,which was more apparent in the extremities than in the trunk.An atropurpureus patch sized 5 cm × 2 cm was seen respectively in bilateral inner thighs; the surface of the central lesions was coarse and covered with dry crusts and scales,giving a rough paperlike appearance,and the peripheral lesions were surrounded by a faint prunosus halo,which faded on pressure.Laboratory examination found hypothyroidation,reduced insulin secretion,abnormal calcium and phosphorus metabolism,and progressive thrombocytosis.Immunofixation electrophoresis showed that the serum was weakly positive for IgG type M protein.This patient was diagnosed with POEMS syndrome associated with multicentre Castleman's disease and thrombocytosis.After treatment with methylprednisolone and thalidomide,the main symptoms of POEMS syndrome were improved,whereas thrombocytosis progressively aggravated.