以Sweet病样皮损为首发表现的SAPHO综合征

A case report of SAPHO syndrome with Sweet’s disease-like skin lesions as the first manifestation

报告1例以Sweet病样皮损为首发表现的SAPHO综合征。患者女,62岁。双手手掌红斑、结节伴发热1个月。皮肤科检查:双手手掌鱼际处可见暗红色结节,浸润明显,轻度压痛。右侧手掌皮损组织病理检查:表皮大致正常,真皮乳头层及网状层弥漫性中性粒细胞浸润,局灶性纤维素样坏死,乳头层轻度水肿;真皮深部血管壁未见纤维素沉积及中性粒细胞浸润。初步诊断:Sweet病。予静脉滴注甲泼尼龙,患者未再发热,10 d后双手手掌鱼际处皮损基本消退。后患者因多关节疼痛伴双手手掌新发皮损5 d,再次至滨州医学院附属医院诊治。皮肤科检查:双手手掌鱼际处可见片状淡红斑,散在分布脓疱,部分伴有结痂、脱屑,触痛不明显。全身骨PET-CT检查:左侧眉骨、右侧顶骨区、胸骨、左侧第7后肋、双侧股骨大转子区及双侧股骨上段可见显像剂浓聚灶;胸骨部位可见典型“牛头征”现象。右侧手掌皮损组织病理检查:表皮角化过度,表皮内脓疱,脓疱内大量中性粒细胞聚集;真皮血管周围炎性细胞浸润。最终诊断:SAPHO综合征。予口服塞来昔布及醋酸泼尼松,配合外用复方氟米松软膏治疗。2周后患者双手手掌皮损基本消退,关节疼痛缓解,出院后继续口服塞来昔布及醋酸泼尼松维持治疗,停用复方氟米松软膏。经6周治疗,患者双手手掌皮损及全身多发关节疼痛消失,停用塞来昔布及醋酸泼尼松。半年后随访,皮损未再复发。

A case of SAPHO syndrome with Sweet’s disease-like skin lesions as the first manifestation is reported.A 62-year-old female presented with erythema and nodules on both palms with fever for one month.Physical examination showed dark red nodules on the palm of thenar eminence,with obvious infiltration and mild tenderness.Histopathological examination of right palm showed generally normal epidermis,diffuse neutrophil infiltration in the papillary layer and reticular layer of the dermis,focal fibrinoid necrosis and mild edema in the papillary layer.No cellulose deposition and neutrophil infiltration were observed in the vascular wall of deep dermis.Initial diagnosis:Sweet’s disease.Methylprednisolone was given intravenously.The patient did not have a fever again,and the skin lesions of the palms basically subsided 10 days later.The patient was admitted to our department again due to multi-joint pain with new skin lesions of both palms for five days.Physical examination showed lamellar erythema and scattered pustules on the thenar of both palms with unobvious tenderness,some of which were accompanied with scabs and desquamation.Whole body bone PET-CT showed developer concentration was observed on the left brow bone,right parietal region,sternum,7th of the left posterior rib,bilateral greater trochanteric region and bilateral upper femur;the typical"bull head sign"was seen on the sternum.Histopathological examination of right palm showed hyperkeratosis of the epidermis,pustules in the epidermis,a large number of neutrophils in the pustules and inflammatory cell infiltration around dermal blood vessels.A diagnosis of SAPHO syndrome was established finally.Oral celecoxib and prednisone acetate were given in combination with compound flamethasone ointment for topical treatment.Two weeks later,the skin lesions of the palms subsided basically,and the joint pain was relieved.After being discharged from the hospital,oral celecoxib and prednisone acetate were continued as a consolidation therapy,and the compound flumethasone ointment was stopped.After six weeks of treatment,the skin lesions of the palms disappeared,and the pain in multiple joints of the whole body disappeared.Celecoxib and prednisone acetate were stopped.During the six months of follow-up,no recurrence was observed.