以鞭笞样皮损为表现的成人Still病1例并文献回顾

Adult-Onset Still’s Disease Presenting with Flagellate Lesions:a Case Report and Literature Review

目的 探讨成人Still病皮损的特点及皮损组织病理学对诊断的作用。方法 回顾性分析1例以不典型皮肤损害(皮损)诊断为成人Still病患者的临床资料,并复习相关文献。结果 患者,女,18岁,因反复发热半月伴咽痛、皮疹入院,胸部及上腹部可见对称性分布的鞭笞样皮损。皮损组织病理示:角质层内及棘层中上部可见大量坏死角质细胞形成,真皮浅层血管周围淋巴组织细胞,个别中性粒细胞浸润,诊断为成人Still病。予以托珠单抗400 mg(8 mg·kg^(-1))静脉滴注1次等治疗病情好转,出院后口服甲泼尼龙、甲氨蝶呤等治疗,门诊随访4周,患者无发热,皮疹及关节酸痛逐渐消退,逐渐减量至停药,病情无复发。结论 典型的成人Still病有一过性特异性皮疹,体温升高时出现,热退时消失;以发热为首诊的患者,可出现不典型皮损,早期临床表现不满足诊断标准时,应仔细评估患者的皮损并行组织病理学检查,有助于该病的诊断。

Objective To investigate the characteristics of skin lesions in adult-onset Still’s disease and the role of skin histopathology in the diagnosis.Methods The clinical data of a case of adult-onset Still’s disease with atypical skin lesions were retrospectively analyzed,and the related literature were reviewed.Results The 18-year-old female patient was admitted to the hospital due to recurrent fever for half a month with sore throat and rash.In addition,the patient had symmetrical distribution of flagellate lesions on the chest and upper abdomen.Histopathology of the skin lesions showed that a large number of necrotic keratinocytes were formed in the stratum corneum and the middle and upper part of the spinous layer.Furthermore,lymphocytes were observed in the superficial dermis around blood vessels,with some infiltrated neutrophils.Therefore,the patient was diagnosed as having adult-onset Still’s disease,and was treated with intravenous drip infusion of Tocilizumab(400 mg,8 mg·kg^(-1)) once.Moreover,Methylprednisolone and Methotrexate were taken orally after discharge.During the follow-up in the outpatient department for 4 weeks,no fever occurred and the rash and joint pain gradually subsided.The dosages were gradually reduced until the drugs were stopped,and no recurrence was found in this patient.ConclusionThe typical adult-onset Still’s disease has a transient specific rash that appears with elevated body temperature and disappears with fever abatement.Patients with fever as the first diagnosis may have atypical skin lesions.When the early clinical manifestations do not meet the Yamaguchi’s criteria,careful evaluation and histopathological examination of the skin lesions should be carried out to facilitate the diagnosis of adult-onset Still’s disease.