Pyogenic granuloma within unilateral dermatomal superficial telangiectasia
Pyogenic granuloma within unilateral dermatomal superficial telangiectasia
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单侧皮瘤浅表毛细血管扩张内化脓性肉芽肿
DOI：
10.1046/j.1365-2133.2003.05195.x
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发表时间：
2003
影响因子：
10.3
通讯作者：
J. Bonafé
中科院分区：
文献类型：
--
作者：
J. Mazereeuw;I. Thibaut;J. Bonafé
SIR，单侧皮区浅表毛细血管扩张（UDST）综合征的特征是先天性或获得性皮区分布的细小毛细血管扩张。我们报告了第一例先天性 UDST 内发生的化脓性肉芽肿 (PG) 病例。一名 20 岁的白人妇女的左肩胛骨上出现了自出生以来就有的无症状红色皮疹。该病变无症状且强度没有变化。她的家族史并不起眼。该患者总体健康状况良好。几个月前，她注意到这个先天性病变内出现了新的病变。这个新病灶生长迅速且无症状。她不记得有任何外伤或昆虫叮咬。她没有服用任何药物，也从未服用过口服避孕药。经检查，她的左肩胛骨上有许多细丝状的毛细血管扩张。其中有一个孤立的鲜红色丘疹（图 1a）。病变被切除。组织病理学显示，边界清楚的外生肿块附着在狭窄的茎上，由位于水肿基质内的增殖毛细血管聚集体组成（图1b）。我们的患者因先天性 UDST 出现 PG。 UDST 的特点是皮肤分布的无症状细毛细血管扩张。该病症是先天性的或后天性的。组织学表明主要累及真皮浅层血管。发病机制尚不清楚。除多形性日光疹外，尚未描述 UDST 与其他皮肤病变相关，多形性日光疹仅发生在获得性 UDST 区域。作者推测，毛细血管扩张皮肤中血流量的增加可能会通过允许增强的炎症细胞向上覆组织的运输来降低光敏性表达的阈值。据我们所知，我们的病例是首例先天性 UDST 内发生 PG 的报告。 PG是一种常见的皮肤和粘膜血管病变，通常发生在婴儿期或儿童期。诱发因素包括外伤、局部病毒感染、昆虫叮咬、疫苗接种和激光治疗。据报道，PG 与鲜红斑痣 (PWS) 有关。激素因素可能起作用，因为它在怀孕或激素治疗期间很常见。在我们的病例中，PG 和 UDST 之间的关联表明皮肤微血管系统具有致病作用，并且似乎与 PG 和 PWS 之间的关联相似。在发育过程中，动脉、静脉和毛细血管循环从原始的毛细血管网络分化出来。原始网络阶段发育停滞导致动静脉（AV）瘘。一些皮肤表现，如毛细血管瘤、PWS 和火焰痣，都是由许多动静脉瘘组成的。 PG 也可能在显微 AV 吻合部位发生，这解释了 PG 和 PWS 之间的关联。我们的病例表明，UDST 是皮肤微血管系统的一种病理过程，可能部分由不同大小、口径和深度的 AV 吻合组成。
SIR, Unilateral dermatomal superficial telangiectasia (UDST) syndrome is characterized by congenital or acquired dermatomally distributed fine telangiectases. We report the first case of pyogenic granuloma (PG) occurring within congenital UDST. A 20-year-old white woman presented with an asymptomatic red rash on her left shoulder blade that she had had since birth. This lesion was asymptomatic and did not vary in intensity. Her family history was unremarkable. The patient was in good general health. A few months before, she noted that a new lesion had appeared within this congenital lesion. This new lesion grew rapidly and was asymptomatic. She could not recall any trauma or insect bites. She was on no medication and had never taken oral contraceptives. On examination, she had numerous fine thread-like telangiectases on her left shoulder blade. Within these was a solitary bright red papule (Fig. 1a). The lesion was excised. Histopathology revealed a well-circumscribed, exophytic mass attached to a narrow stalk, consisting of aggregates of proliferating capillaries located within an oedematous matrix (Fig. 1b). Our patient presented with PG arising in congenital UDST. UDST is characterized by dermatomally distributed asymptomatic fine telangiectases. The condition is congenital or acquired. The histology indicates involvement predominantly of the superficial vessels of the dermis . The pathogenesis is still unknown. UDST has not been described in association with other cutaneous lesions except for polymorphic light eruption, which was described in one case, occurring solely on an area of acquired UDST. The authors hypothesized that increased blood flow in the telangiectatic skin may reduce the threshold for expression of photosensitivity by permitting enhanced inflammatory cell trafficking to the overlying tissue. To our knowledge, our case is the first report of PG occurring within congenital UDST. PG is a common vascular lesion of skin and mucous membranes that commonly occurs during infancy or childhood. Predisposing factors are trauma, localized viral infection, insect bites, vaccination and laser treatment. PG has been reported to be associated with portwine stains (PWS). Hormonal factors may have a role, as it is a common occurrence in pregnancy or during hormone therapy. In our case, the association between PG and UDST suggests a pathogenic role for the cutaneous microvasculature, and seems similar to the association between PG and PWS. During the process of development, the arterial, venous and capillary circulation differentiate from a primitive capillary network. An arrest in development during the primitive network stage gives rise to arteriovenous (AV) fistulae. Several cutaneous manifestations such as capillary haemangiomas, PWS and naevus flameus are composed of numerous AV fistulae. PG may also develop at the sites of microscopic AV anastomoses, which explains the association between PG and PWS. Our case suggests that UDST, which is a pathological process in the cutaneous microvasculature, probably consists, in part, of AV anastomoses of variable size, calibre and depth.