- 期刊
Familial Amyloidosis Cutis Dyschromica-A Case Report and Review of the Literature
家族性色素異常性皮膚澱粉樣變性症－病例報告及文獻回顧
摘要
色素異常性皮膚澱粉樣變性症是原發性皮膚澱粉樣變性症的一個罕見而獨特的亞型，其特徵是在皮膚出現廣泛的色素沉著及色素脫失的斑點；我們在此報告一位25歲男性自八歲起在全身皮膚出現廣泛的色素沉著及色素脫失約斑點，病灶並未侵犯其臉部、手掌、及腳掌。他的姊姊亦於相近的年齡時在皮膚出現類似的色素異常。色素沉著的病灶其組織病理顯示在基底層有黑色素的增加，乳突狀真皮出現色素失調和均質的嗜伊紅性塊狀物質沉積，且以剛果紅染色呈陽性反應。配合臨床及病理發現診斷爲色素異常性皮膚澱粉樣變性症。我們報告此一家族性病例並回顧相關文獻。
關鍵字
無資料並列摘要
Amyloidosis cutis dyschromica is a rare distinct type of primary cutaneous amyloidosis characterized by the presence of widespread hypopigmented as well as hyperpigmented macules. We herein report a 25-year-old male showing diffuse hyperpigmentation as with hypopigmented spots over his whole body but sparing his lace, hands and feet from the age of 8 years. His elder sister had also des eloped a similar skin pigmentary defect since about the same age. Histopathology of the hyperpigmented lesion revealed increase of melanin in the basal layer, pigment incontinence and amorphous eosinophilic masses stained positive as with Congo red in the papillary dermis. Amyloidosis cutis dyschromica was diagnosed. We report the familial case and the literature was reviewed.