Papi M, Didona B, De Pità O, Frezzolini A, Di Giulio S, De Matteis W, Del Principe D, Cavalieri R
Department of Dermatology, Istituto Dermopatico Immacolata, Istituto Ricovero e Cura a Carattere Scientifico, Rome, Italy.
Arch Dermatol. 1998 Apr;134(4):447-52. doi: 10.1001/archderm.134.4.447.
To assess the role of platelets and lymphocyte-related immunological mechanisms in livedo vasculopathy (LV) and cutaneous small vessel vasculitis (CSVV). Livedo vasculopathy is thought to be related to the thrombotic occlusion of small and medium-sized dermal vessels. Cutaneous small vessel vasculitis comprises a heterogeneous group of disorders in which the main pathogenetic events could be modulated by circulating cytokines.
Case series study of 2 groups of patients affected respectively with LV and CSVV.
A large clinical and research institute for the study and treatment of cutaneous diseases.
Consecutive patients with clinically and histologically proved idiopathic LV (n = 8) and CSVV (n = 20) were studied and compared with healthy donors (n = 20). Patients with potentially correlated systemic diseases were excluded.
Surface expression of platelet P-selectin and circulating level of interleukin (IL) 1beta, tumor necrosis factor alpha (TNF-alpha), IL-8, IL-2, and soluble IL-2 receptor.
The IL-2 and soluble IL-2 receptor levels were significantly higher in serum samples from patients with both LV (1.24 +/- 0.46 IU/mL [mean +/- SD] vs 0.46 +/- 0.24 IU/mL, P<.001; 899 +/- 368 IU/mL vs 628 +/- 132 IU/mL, P<.02) and CSVV (0.91 +/- 0.57 IU/mL, P<.02; 1087 +/- 451 IU/mL, P<.001) than in those from the healthy controls. The serum levels of IL-1beta, TNF-alpha, and IL-8 were higher in patients with CSVV than in controls (7.53 +/- 6.7 pg/mL vs 4.58 +/- 2.72 pg/mL; 23.7 +/- 12.6 pg/mL vs 10.82 +/- 2.46 pg/mL, P<.001; 37.8 +/- 46 pg/mL vs 8.25 +/- 3.53 pg/mL, P<.02, respectively). No significant difference in the serum levels of IL-1beta (7.2 +/- 4.9 pg/mL), TNF-alpha (12.9 +/- 3.47 pg/mL), and IL-8 (5.9 +/- 4.13 pg/mL) was observed in patients with LV compared with controls. An increased expression of platelet P-selectin was also detected in patients with LV in comparison with controls and patients with CSVV. The mean +/- SD percentage of positive cells for P-selectin was 43% +/- 5% in the patients with LV, 5.1% +/- 2% in the controls (P<.001), and 5.3% +/- 2% in the patients with CSVV (P<.001).
Taken together, these data demonstrate that different pathogenetic mechanisms are operative in LV and CSVV. In fact, platelet and lymphocyte activation is present in LV, whereas the levels of inflammatory mediators are in a normal range. In CSVV, the high serum levels of proinflammatory cytokines suggest that they are actively involved in the pathogenesis of cutaneous vasculitis.
评估血小板及淋巴细胞相关免疫机制在网状青斑血管病（LV）和皮肤小血管炎（CSVV）中的作用。网状青斑血管病被认为与中小真皮血管的血栓性闭塞有关。皮肤小血管炎是一组异质性疾病，其主要发病机制可能受循环细胞因子的调节。
对分别患有LV和CSVV的两组患者进行病例系列研究。
一家大型皮肤病研究与治疗临床及研究机构。
对临床及组织学确诊的特发性LV患者（n = 8）和CSVV患者（n = 20）进行连续研究，并与健康供者（n = 20）进行比较。排除患有潜在相关系统性疾病的患者。
血小板P-选择素的表面表达以及白细胞介素（IL）-1β、肿瘤坏死因子α（TNF-α）、IL-8、IL-2和可溶性IL-2受体的循环水平。
LV患者（1.24±0.46 IU/mL[平均值±标准差]对0.46±0.24 IU/mL，P<0.001；899±368 IU/mL对628±132 IU/mL，P<0.02）和CSVV患者（0.91±0.57 IU/mL，P<0.02；1087±451 IU/mL，P<0.001）血清样本中的IL-2和可溶性IL-2受体水平均显著高于健康对照组。CSVV患者血清中的IL-1β、TNF-α和IL-8水平高于对照组（分别为7.53±6.7 pg/mL对4.58±2.72 pg/mL；23.7±12.6 pg/mL对10.82±2.46 pg/mL，P<0.001；37.8±46 pg/mL对8.25±3.53 pg/mL，P<0.02）。与对照组相比，LV患者血清中的IL-1β（7.2±4.9 pg/mL）、TNF-α（12.9±3.47 pg/mL）和IL-8（5.9±4.13 pg/mL）水平无显著差异。与对照组及CSVV患者相比，LV患者血小板P-选择素的表达也增加。LV患者P-选择素阳性细胞的平均±标准差百分比为43%±5%，对照组为5.1%±2%（P<0.001），CSVV患者为5.3%±2%（P<0.001）。
总体而言，这些数据表明LV和CSVV存在不同的发病机制。事实上，LV中存在血小板和淋巴细胞激活，而炎症介质水平处于正常范围。在CSVV中，促炎细胞因子的高血清水平表明它们积极参与皮肤血管炎的发病机制。
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