Perianal Lesion as the Leading Presentation of Langerhans Cell Histiocytosis: Report of a Case

以肛門周邊病灶爲主要表現之蘭格罕細胞組織球增生症：病例報告

蔡家騏(Chia-Chi Tsai) ； 陳自諒(William Tzu-Liang Chen) ； 丁文謙(Wen-Chien Ting) ； 江驊哲(Hua-Che Chiang) ； 張伸吉(Sheng-Chi Chang) ； 陳冬英(Tung-Ying Chen) ； 黃思銘(Ssu-Ming Huang)

《Formosan Journal of Surgery》 42卷3期 (2009/06) Pp. 165-170

https://doi.org/10.29833/FJS.200906.0006

摘要

蘭格罕細胞組織球增生症是一種增生性的血液疾病以多種不同型態表現。再此，我們報告一罕見病例：一位無任何特殊疾病史的年輕男性病人，以肛門周邊病灶爲主要表現之蘭格罕細胞組織球增生症。此17歲病人發現有肛門周圍紅斑性結節兩個月，經局部塗抹藥膏無改善。切片組織病理檢查顯示爲蘭格罕細胞組織球增生症。進一步檢查發現頭骨左後顯區及左脛骨近端內側亦受侵犯。然而這些骨骼侵犯並無任何臨床症狀。在非特異性肛門周邊病灶，蘭格罕細胞組織球增生症需列爲鑑別診斷。對於任何可疑的肛門周圍腫塊，切片檢查即足以診斷。

並列摘要

Langerhans cell histiocytosis is a proliferative hematological disease with pleomorphic characters. Here, we present a very unusual case of Langerhans cell histiocytosis in a young male patient without a specific previous medical history, who had a perianal lesion as the leading symptom. This 17-year-old patient presented with perianal erythematous nodules for two months, unresponsive to topicals. Langerhans cell histiocytosis was suggested by histopathological examination after excisional biopsy. Thorough investigations revealed additional involvement of the left posterior temporal region of the cranium, and the left medial proximal tibia. However, these bone lesions caused no symptoms. Langerhans cell histiocytosis should always be included in the differential diagnosis list for patients with nonspecific perianal lesions. For any perianal mass in doubt, a simple biopsy examination is necessary.

並列關鍵字

langerhans cell histiocytosis ； immunohistology ； perianal lesion ； CD1a ； S-100