NEJMのイメージから：手掌線状黄色腫

手掌線状黄色腫

コレステロールが高いといろいろなところに沈着する。

私の知っている限りでは アキレス腱、眼瞼、消化管では胃カメラでよく黄色腫を見ます。 

今回ドイツから報告のこの手掌はちょっと特徴的である。この方痛みで栓が開けられなかったり大変だ。 原因は少し特殊な病態のようで治療はフェレーシスなのだとか。

 今回初診から10年目の報告であり、いまでも隔週のフェレーシスをやっているらしい。 手掌の黄色腫は消失し症状も軽快しているとのこと。

IMAGES IN CLINICAL MEDICINE

Xanthoma Striatum Palmare

• Viktoria F. Koehler, M.D., 
• and Klaus G. Parhofer, M.D.
• A 49-year-old man was referred to the metabolic clinic for evaluation of severe hypercholesterolemia and xanthomas, which were particularly prominent on the hands. The lesions were painful and affected the patient’s everyday life, making it difficult for him to open bottles or shake hands. He had a 2-year history of biliary cirrhosis due to ischemic cholangiopathy. Laboratory evaluations showed a total cholesterol level of 970 mg per deciliter (25.1 mmol per liter); a triglyceride level of 158 mg per deciliter (1.8 mmol per liter); a low-density lipoprotein (LDL) cholesterol level of 875 mg per deciliter (22.6 mmol per liter), with presence of lipoprotein-X; and a high-density lipoprotein cholesterol level of 64 mg per deciliter (1.7 mmol per liter). Treatment with LDL apheresis was started. Palmar xanthomas can also be seen in patients with type III hyperlipoproteinemia (familial dysbetalipoproteinemia). Xanthomas are rarely so severe that they interfere with activities of daily life. Within 3 months after the initiation of weekly LDL apheresis, the lesions had almost disappeared and the patient reported substantial relief from pain and improvement in function. At follow-up 10 years after presentation, the patient was in stable condition and was undergoing LDL apheresis every other week. Since the initiation of LDL apheresis, his LDL cholesterol level had decreased to 110 mg per deciliter (2.8 mmol per liter).

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  Viktoria F. Koehler, M.D.
  Klaus G. Parhofer, M.D.
  Klinikum der Universität München, Munich, Germany