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Neonatal Lupus Erythematosus: A Case Report

新生兒紅斑性狼瘡-病例報告

摘要


新生兒紅斑性狼瘡為一種荌見的疾病,乃母親的自體抗體經胎盤傳給胎兒所造成的,臨床特徵有先天性心臟傳阻斷,狼瘡性皮膚病變,或是兩者都有。其他症狀有血液學異常,肺炎,淋巴腺腫大,脾腫大及肝病等。本文報告一例由外表健康母親所生產的新生兒女嬰,出生時在臉部、頸部及軀幹上半部即散布有紅色斑丘疹,理學檢查包括心臟、肺部及腹部皆正常。血液學檢查有白血球減少及血小板減少的情形。母親及女嬰的血清抗核抗體,抗Ro/SSA及La/SSB抗體皆呈陽性反應。皮膚病於三週大時漸漸變成環狀,紅色並有輕微脫屑的現象。七週大時,作一皮膚切片,其病理變化與紅斑性狼瘡相符合。除了建議病嬰避免接觸陽光外,她沒有接受殊的治療。血液學異常於九週大時恢復正常。促骨紅斑於隨後的四個月中漸漸地消失,只留下色素變淡及微血管擴張的變化。

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並列摘要


Neonatal erthematosus is a syndrome characterized by congenital heart block and/or cutaneous lupus erythematosus in the presence of maternal autoantibodies. We report a female newborn with cutaneous lesions of the neonatal lupus erythematosus born to a clinically asymptomatic mother. She presented with erythematous maculopapules on the face, neck and upper trunk at birth. Complete blood cell count showed leucopenia and thrombocytopenia. Antinuclear antibodies, anti-Ro/SSA and anti-La/SSB antibodies were positive in the mother and infant. The skin lesions became annular, erythematous and slightly scaling three weeks later. A skin biopsy performed at seven weks of age showed edema of the dermis and perivascular mononuclear cell infiltration. Immunofluorescent studies showed immune complex deposition of IgG an IgM in the basal zone of epidermis. She received supportive treatment and sun avoidance. Hematologic abnormalities recovered by nine weeks of age. Skin lesions faded gradually during the following four months, leaving residual hypopigmentation and telangiectasia.

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