双语病例血管性水肿表现为系统性红斑狼

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医脉通导读

血管性水肿/血管神经性水肿是一种界限清楚的局限性水肿，病变累及皮肤深层（包括皮下组织）。荨麻疹的皮肤病变会出现瘙痒症状，也能累及头皮到脚心等各个部位，而血管性水肿与荨麻疹不同，最常见的累及部位是眶周组织和嘴唇。血管性水肿由多种病因，或者遗传所致。获得性血管性水肿（AAE）的特点是获得性C1抑制剂缺陷（C1-INH），人类补体经典途径的高度激活，因接触激肽系统不适当激活而释放的缓激肽介导的血管性水肿症状。关于获得性过敏性血管神经性水肿继发于系统性红斑狼疮（SLE）的报道较少，下文所述为一例血管性水肿作为初始症状最终诊断为SLE。

CaseReport

A32-year-oldKashmirifemalewaspresentedwithswellingofdorsumofhands,lipsandrightperiorbitalregionofrecentorigin.Shehadaprevioushistoryofprimaryhypothyroidismforlastsixmonths.Shewastaking75μgoflevothyroxineperday.Shedeniedanyrecentexposurestodrugs,food,insectvenomstingsandphysicalfactorspriortotheonsetoffacialandhandsswelling.Shehadnoprevioushistoryofsimilarattacks,andnofamilyhistoryofangioedema.Herpastmedicalhistorywasotherwiseunremarkable.

患者，女，32岁。因手背、嘴唇、右眼眶周肿胀入院。既往病史：原发性甲状腺功能减退症，持续6个月。用药左旋甲状腺素75μg/d。否认在面部和手肿胀前接触过药物、食物、昆虫毒液和物理因素。既往无类似疾病，无血管性水肿家族史。既往病史不明显。

Ongeneralphysicalexamination,shewastall,thinbuiltandafebrile.Shehaderythematousswellingofdorsumofhandsinvolvingallfingerswithouttendernessandinduration.Thiswasassociatedwithunilateralperiorbitalswellinganderythema,(Figure1)forwhichprovisionaldiagnosisofangioneuroticedemawithunderlyingallergicdisorderwasentertained.Therewasnopedalorsacraledema,nolymphadenopathyorhepatosplenomegalyonclinicalexamination.Shehadnofeaturesofvasculitisonphysicalexamination.Laboratoryinvestigationshowedbicytopeniawithhemoglobinof11.4g/dL(normalrange10.7–14.9),awhitebloodcellcountof2.88x/μg/L(normalrange3.30x–9.66x),anabsolutelymphocytecountof0.9x/μL(normalrange1.04x–2.86x/μL)andaplateletcountof46x/μL(normalrangex–x/μL).HerESRmeasured30mm/h(normalrange0–20mm/h).ShewasfurtherinvestigatedbyestimatinglevelsofC1–esteraseinhibitorprotein(C1–INH),foundlowmg/L(normalrange–mg/L).Bonemarrowexaminationandplasmaelectrophoresiswasnormal.Imagingtechniquesnamely,ultrasonography,magneticresonanceimaging(MRI)and

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