特发性高嗜酸性粒细胞综合征

A case of idiopathic hypereosinophilia syndrome

患者男,46岁。全身泛发丘疹、结节伴瘙痒3年。皮肤科检查:双侧腋窝及腹股沟多发淋巴结肿大,全身皮肤弥漫性褐色斑,躯干及四肢见多发黄豆大丘疹及结节,头发及眉毛脱落,皮损受累面积超过90%。实验室检查:外周血中嗜酸性粒细胞7.71×10^(9)/L,血小板源性生长因子受体α(PDGFRA)、血小板源性生长因子受体β(PDGFRB)基因阴性。皮损组织病理示真皮乳头层及真皮浅层血管和皮肤附属器周围淋巴浆细胞及嗜酸性粒细胞浸润。骨髓涂片及组织病理示嗜酸性粒细胞计数及比例增高,诊断:特发性高嗜酸性粒细胞综合征。予甲泼尼龙及沙利度胺等治疗,嗜酸性粒细胞数降至正常,皮损及症状均改善。

A case of idiopathic hypereosinophilia syndrome is reported.A 46-year-old male patient presented with generalized papules and itchy nodules for 3 years.Physical examination showed multiple enlarged lymph nodes in bilateral axilla and groin,diffuse brownpigmentation on theskin of the whole body,multiplesoybeansizeof tough papules and noduleson the trunk and limbs,which involved more than 90%area of body with hair and eyebrow loss.Laboratory examination showed peripheral blood eosinophil was 771×10^(9)/L Gene detection showed negative results for PDGFRA and PDGFRB Histopathological examination showed infiltration of lymphocytes and eosinophils around the dermal papilla and superficial dermal vessels and skin appendages.Bone marrow biopsy showed the proportion of eosinophils increased.The patient was diagnosed withidiopathic hypereosinophilia syndrome.Aftermethylprednisolone,thalidomide and othertreatments,theeosinophilswerereduced to normal level,the skin rash was controlled,and the itching symptom was improved.