Congenital Multiple Glomangiomas of the Breast Arranged in a Segmental Pattern

P. Morais, L. Peralta, H. Bettencourt, A. Mota
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Abstract

An otherwise healthy 15-year-old female patient presented with multiple bluish skin papules and nodules located at right breast that had been present since birth. The lesions were slightly tender to palpation and had enlarged with body growth, especially after menarche. Her father presented several lesions with a similar appearance on the upper limbs and trunk. On physical examination, multiple soft, well-defined dark-blue and purple-red papules and nodules were observed, arranged in a segmental or band-like pattern, in the girl’s right breast and ipsilateral axilla (Figure 1a). Dermoscopy of lesions revealed reddish purple structureless areas, without specific features of any skin neoplasm (Figure 1b). A biopsy of a breast nodule was performed. The histological examination revealed a non encapsulated vascular tumor involving the mid and lower dermis. It was composed of multiple dilated, cavernous-like capillaries containing erythrocytes surrounded by endothelial cells, and one to several layers of uniform cuboidal cells with pale or faintly eosinophilic cytoplasm (glomus cells) (Figure 1c). These features were consistent with the diagnosis of glomangioma. Complete blood and platelet counts, coagulation tests and fecal occult blood test were within normal range. Informed about the nature of the lesions and treatment options the patient chose therapeutic abstention.
先天性乳腺多发性血管瘤呈节段性排列
一位健康的15岁女性患者,自出生以来右乳出现多发蓝色皮肤丘疹和结节。病变触诊轻微,随身体生长而增大,特别是在月经初潮后。她父亲的上肢和躯干出现了几处类似的病变。体格检查,女孩右乳及同侧腋窝可见多发软质、界限分明的深蓝色和紫红色丘疹和结节,呈节段或带状排列(图1a)。皮损镜显示为红紫色无结构区,无任何皮肤肿瘤的特异性特征(图1b)。对乳腺结节进行活组织检查。组织学检查显示一非包膜血管性肿瘤累及真皮中下部。它由多个扩张的海绵状毛细血管组成,毛细血管中含有被内皮细胞包围的红细胞,以及一到几层均匀的立方体细胞,细胞质苍白或略带嗜酸性(球囊细胞)(图1c)。这些特征与血管瘤的诊断一致。全血和血小板计数、凝血试验和粪便潜血试验均在正常范围内。在得知病变的性质和治疗方案后,患者选择了治疗性戒酒。
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