A case of urticaria pigmentosamisdiagnosed as chronic urticaria

案例報告：誤診為慢性尋痲疹之色素性尋痲疹個案

薛如婷(Ju-Ting Hsueh) ； 黃靖富(Ching-Fu Huang)

《中華職業醫學雜誌》 23卷4期 (2016/10) Pp. 291-297

摘要

肥大細胞增多症其特徵為全身各器官內有大量的肥大細胞聚集，主要侵犯皮膚，但也可能侵犯皮膚以外的器官。故根據肥大細胞侵犯的位置不同可將其區分為皮膚性肥大細胞增多症及全身型肥大細胞增多症。皮膚性肥大細胞增多症，顧名思義，肥大細胞聚集處僅限於皮膚處，而根據世界衛生組織(World Health Organization, WHO)在2008年更新肥大細胞增生症的分類與診斷標準中，皮膚性肥大細胞增生症又可被分為四種亞型，其中色素性蕁麻疹是皮膚性肥大細胞增生症最常見的亞型。在全身性肥大細胞增生症中，肥大細胞會侵犯皮膚以外的器官，但也有可能合併有皮膚的侵犯。全身性肥大細胞增生症的病患有較高的機率會產生惡性變化，本案例被誤診為慢性尋痲疹多年，故多年來僅發作時自行服藥緩解症狀，未曾接受過其他檢查已排除惡性變化的可能，更遑論定期回診追蹤，所以臨床上當看到病人皮膚病灶並懷疑可能為皮膚性肥大細胞增生症時，需安排進一步檢查以排除全身性肥大細胞增生症的可能。

關鍵字

色素性蕁麻疹 ； 肥大細胞增多症 ； 達里埃氏徵象(Darier sign)

並列摘要

Mastocytosis is a rare disorder characterized by marked mast cells infiltrate in the skin and/ or other organs. It can be subdivided into two subtypes: cutaneous mastocytosis and systemic mastocytosis. Cutaneous mastocytosis is limited to skin and it includes four subtypes among which urticaria pigmentosa is the most common. Systemic mastocytosis is characterized by mast-cell infiltrationin multiple extracutaneous organs with or without skin involvement and ittends to have a greater risk of malignant transformation. Most patients, clinically suspected as mastocytosis with skin lesions, should undergo examination to exclude the possibility of systemic mastocytosis. We present a clinicalcase of a 22-year-old patient with urticaria pigmentosa.

並列關鍵字

Mastocytosis ； Urticaria pigmentosa ； Darier sign