- 期刊
A Review of 10 Cases with Solitary Cutaneous Juvenile Xanthogranuloma-Clinical and Histological Presentation
10位年少型黃肉芽腫病人之臨床回顧分析
摘要
年少型黃肉芽腫是一罕見，多發生於小孩的組織球異常病變。其表現主要為皮膚上紅色或黃色的小丘疹及結節。一般沒有症狀。好發的部位為頭頸部及上肢。大多數的病灶不去處理亦會在幾年內消失。根據文獻記載，有少部分的病人會有其他器官的侵犯，如眼睛、肺臟、肝臟、骨頭、中樞神經等，而造成較嚴重的後果。年少型黃肉芽腫與年少型慢性骨髓白血病及第一型神經纖維瘤的相關性也曾被提出。病理組織特徵為真皮層不同型態的組織球浸潤及數量不一的Touton巨細胞。組織免疫染色HAM56，CD68及factor XⅢa呈現陽性反應但S100及CD1a為陰性反應。本文回顧10位年少型黃肉芽腫病患的臨床表現及治療經驗：這10位病患在系統評估後皆為單一皮膚病灶，他們均接受外科切除，在術後平均5年的追蹤僅有一例的復發但沒有其他器官的侵犯。我們認為外科切除獲得確切的病理診斷以和其他疾病作鑑診斷及排除其他器官的侵犯是很重要的。若懷疑有其他器官的侵犯，應照會眼科、腫瘤科、神經科等，予以適當的治療。
關鍵字
無資料並列摘要
Juvenile xanthogranuloma (JXG) is one of the most common forms of non-Langerhans' cell histiocytosis in infancy and early childhood. It is presented principally as a solitary cutaneous lesion with predilection for the head, neck and upper trunk region. Cutaneous lesions usually resolve and most patients have an otherwise unremarkable course. Systemic (extracutaneous) involvement is rare but significant morbidity and occasional deaths may occur. A rare association between juvenile xanthogranuloma and neurofibromatosis Ⅰ or juvenile chronic myelogenous leukemia has been identified. This study included 10 patients with a solitary cutaneous form of juvenile xanthogranuloma at our institute in past 20 years. The patient age, gender, lesion size, location, associated diseases, clinical, management, follow-up and histiologic characteristic are summarized and the literatures are reviewed.