Köstler E
Dermatol Monatsschr. 1976 Jun;162(6):465-77.
The trophedema Nonne-Milroy-Meige has an exceptional position within the group of the primary lymphatic edemas (l.e.) because of its hereditary. Its frequency less than 1% of primary l.e. The trophedema is caused by a genetic determined defect of the morphogenese of parts of lymphatic system, which is mainly autosomal dominantly transmitted. It is morphologically and lymphografically characterized by a lack and reduction respectively of the number of lymphatic vessels. The trophedema results an emotional (cosmetic) and physical stress. Complicationes will rarely arise. In this paper it is described the case of the development of a cancer upon a trophedema, which seems to be the first case ever published. It will be shown, that an test-section have to be carried out in all cases of damages at a l.e. als soon as possible. The best conservative method at present used is the treatment with cortisone and hyaluronidase including bandage. However a real cure of the primary l.e. including the trophedema can not be attained by therapeutic methods presently used, because the defect of the lymphatic-vessels-system is hereditary. On the other hand therapeutic nihilism cannot be recommended.
诺内 - 米尔罗伊 - 梅热氏营养性水肿在原发性淋巴水肿组中具有特殊地位，因其具有遗传性。其发病率不到原发性淋巴水肿的1%。营养性水肿是由淋巴系统部分形态发生的基因决定性缺陷引起的，主要为常染色体显性遗传。其形态学和淋巴管造影特征分别为淋巴管数量缺乏和减少。营养性水肿会导致情感（美容方面）和身体上的压力。并发症很少出现。本文描述了一例营养性水肿发生癌变的病例，这似乎是有史以来首次发表的病例。结果表明，一旦原发性淋巴水肿出现损伤，应尽快进行活检。目前使用的最佳保守方法是使用可的松和透明质酸酶并结合绷带进行治疗。然而，由于淋巴管系统的缺陷是遗传性的，目前使用的治疗方法无法真正治愈包括营养性水肿在内的原发性淋巴水肿。另一方面，不建议采取治疗虚无主义态度。
