Merlen J F
Phlebologie. 1987 Apr-Jun;40(2):473-87.
Ecchymotic patches on the fingers and vascular purpura in Gardner-Diamond syndrome are two benign but recurrent clinical disorders occurring chiefly in young women. They involve superficial cutaneous hemorrhagic signs. Both disorders testify to microvascular fragility without perturbation of general hemostasis. Diagnosis is easy for those familiar with the disorders. Ecchymotic episodes accompanied by sharp pain begin at the roots of the fingers. Microtraumatism is soon seen to be involved. Ecchymosis develops at the flexor muscles of the fingers but "capillary" resistance is only reduced in one third of cases. The pathogenic explanation involves rupture of a small post-capillary vein due to disorders in the control system of kinins and local metabolites. The autoerythrocyte sensitization syndrome described in 1955 by Gardner and Diamond is characterized by painful and febrile episodes followed by purpuric and ecchymotic lesions of the skin on various parts of the body, but not necessarily on the legs. Dysneurotonic effects are frequent but diagnosis is based upon promotion of the disorder by intradermal or subcutaneous injection of a minimal quantity of autologous blood. Immunological effects are considered but the ailment involves microvascular control disorders, notably of the capillary-vein segment, in particular at Copley's endo-endothelial fibrin film. Imbalance in fibrin formation and lysis is associated with perturbation of the kinin and serotonin systems. In addition to the use of certain bioflavonoids and calcium inhibitors, prevention and treatment should involve consideration of drugs which affect plasticity in the vascular wall and pericapillary interstitial conjunctiva and the rheological properties of blood flow and interstitial tissue.
加德纳 - 戴蒙德综合征患者手指上的瘀斑和血管性紫癜是两种主要发生在年轻女性中的良性但复发性临床病症。它们表现为浅表性皮肤出血迹象。这两种病症均表明存在微血管脆性,但全身止血功能未受干扰。对于熟悉这些病症的人来说,诊断很容易。伴有剧痛的瘀斑发作始于手指根部。很快就会发现微创伤与之相关。瘀斑在手指屈肌处形成,但仅有三分之一的病例中“毛细血管”阻力降低。其发病机制解释为由于激肽和局部代谢产物控制系统紊乱导致小的毛细血管后静脉破裂。1955年加德纳和戴蒙德描述的自身红细胞致敏综合征的特征是疼痛和发热发作,随后身体各部位皮肤出现紫癜和瘀斑,但不一定出现在腿部。神经张力障碍效应很常见,但诊断基于皮内或皮下注射少量自体血可促使病症发作。虽然考虑到了免疫效应,但该病症涉及微血管控制紊乱,尤其是毛细血管 - 静脉段,特别是在科普利的内皮 - 内皮纤维蛋白膜处。纤维蛋白形成和溶解的失衡与激肽和血清素系统的紊乱有关。除了使用某些生物类黄酮和钙抑制剂外,预防和治疗应考虑使用影响血管壁和毛细血管周围间质结膜可塑性以及血流和间质组织流变学特性的药物。