Jansen W, Lentner A, Genzel I
Hautklinik der RWTH Aachen, Germany.
J Dermatol Sci. 1994 Feb;7(1):68-70. doi: 10.1016/0923-1811(94)90024-8.
We report on capillary changes on the Sulcus matricis unguis in a patient with an established angiokeratoma corporis diffusum Fabry. A fasciculated ramification of the capillary in 2-5 loops, which is not found in healthy individuals and that until now has solely been described in patients with vasculitis, was detected. The question now arises whether these are initial changes in the disease which later lead to manifested angiokeratomas. Further investigations will show if, and to what extent, these observations can contribute to further clarification of the developmental method of acroparaethesiae, which are part of the principal signs of Morbus Fabry.
我们报告了一例确诊为弥漫性躯体血管角质瘤(法布里病)患者甲床沟的毛细血管变化。检测到毛细血管呈束状分支,形成2 - 5个环,这在健康个体中未发现,迄今为止仅在血管炎患者中有所描述。现在的问题是,这些是否是该疾病的初始变化,随后会导致明显的血管角质瘤。进一步的研究将表明,这些观察结果能否以及在多大程度上有助于进一步阐明肢端感觉异常的发展方式,肢端感觉异常是法布里病主要体征的一部分。