Croue A, Gardembas-Pain M, Verret J L, Boasson M, Rousselet M C, Saint Andre J P
Laboratoire d'Anatomie Pathologique, CHU, Angers.
Ann Pathol. 1993;13(2):128-30.
Erythromelalgia is a vascular disorder of the extremities and is sometimes related to myeloproliferative syndrome with thrombocythemia. We report the cutaneous histopathology in case of erythromelalgia that revealed a thrombocythemia vera. Small arteries were occluded by thrombi of different age and narrowing of the lumen occurred by intimal proliferation of smooth muscle cells. There was no involvement of venules or capillaries. These vascular changes are highly suggestive of erythromelalgia and have not to be confused with necrotizing and/or granulomatous angiitis because of absence of fibrinoid necrosis and sparse inflammatory cells.
红斑性肢痛症是一种四肢血管疾病,有时与伴有血小板增多症的骨髓增殖综合征有关。我们报告了一例红斑性肢痛症的皮肤组织病理学检查结果,显示为真性血小板增多症。小动脉被不同时期的血栓阻塞,管腔因平滑肌细胞内膜增生而狭窄。小静脉和毛细血管未受累。这些血管变化高度提示红斑性肢痛症,且不应与坏死性和/或肉芽肿性血管炎相混淆,因为不存在纤维蛋白样坏死且炎症细胞稀少。