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一例泛发性发疹性组织细胞增多症。

A case of generalized eruptive histiocytosis.

作者信息

Fernández-Jorge Beatriz, Goday-Buján Jaime, Del Pozo Losada Jesús, Alvarez-Rodríguez Roberto, Fonseca Eduardo

机构信息

Department of Dermatology, Hospital Juan Canalejo, A Coruña, Spain.

出版信息

Acta Derm Venereol. 2007;87(6):533-6. doi: 10.2340/00015555-0212.

Abstract

Histiocytoses are a heterogeneous group of diseases, characterized by the accumulation of reactive or neoplastic histiocytes in various tissues. Generalized eruptive histiocytosis belongs to cutaneous non-Langerhans' cell histiocytoses and is a rare, generalized, self-healing disorder that usually follows a benign clinical course. Herein, we report a case of generalized eruptive histiocytosis in a 41-year-old woman with peculiar clinical and histological features. Clinically, the papules showed a marked distribution into the seborrhoeic areas of the trunk, with a great tendency to coalesce. Furthermore, immunohistochemical labelling demonstrated that the histiocytes were positive for CD68, but negative for CD34, S100, CD1a and XIIIa factor. This is the second report of generalized eruptive histiocytosis with a negative XIIIa factor. We discuss the differential diagnoses of the clinical picture and emphasize that this benign cutaneous disorder should be subjected to close follow-up, owing to the possibility of evolution to a more severe type of histiocytosis or the association with underlying diseases. Spontaneous regression was observed in this actual case.

摘要

组织细胞增多症是一组异质性疾病,其特征是反应性或肿瘤性组织细胞在各种组织中积聚。泛发性发疹性组织细胞增多症属于皮肤非朗格汉斯细胞组织细胞增多症,是一种罕见的、全身性的、可自愈的疾病,通常呈良性临床经过。在此,我们报告一例41岁女性泛发性发疹性组织细胞增多症,具有独特的临床和组织学特征。临床上,丘疹在躯干的脂溢性区域有明显分布,极易融合。此外,免疫组化标记显示组织细胞CD68阳性,但CD34、S100、CD1a和XIIIa因子阴性。这是第二例XIIIa因子阴性的泛发性发疹性组织细胞增多症报告。我们讨论了该临床症状的鉴别诊断,并强调由于可能演变为更严重的组织细胞增多症类型或与潜在疾病相关,这种良性皮肤疾病应密切随访。在本实际病例中观察到了自发消退。

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