Basal cell nevus syndrome in a black patient: report of a case and review of the literature

An 80-year-old black man had multiple basal cell epitheliomas (BCEs), palmar pits, and mandibular cysts. This patient is unusual, not only because of multiple BCEs, but also becuase they developed when the patient was at age 77. Only three other black patients with multiple BCEs in association with basal cell nevus syndrome have been reported previously.     

Basal cell nevus syndrome

A 90-year-old female presented with multiple nevoid basal cell epitheliomas, skeletal defects and jaw cysts. The syndrome is briefly revisited.     

Basal cell nevus syndrome and webbed neck

A patient is reported with the basal cell nevus syndrome (BCNS) in combination with a webbed neck. This symptom might be considered as an expression of the BCNS.     

Becker's nevus syndrome: case report

Becker's nevus is a hyperpigmented macula that is predominantly located on the anterior trunk or on the scapular region, frequently associated with hypertrichosis. The association of Becker's nevus with other cutaneous, musculoskeletal or maxillofacial anomalies has been called Becker's nevus syndrome. We report a case of a 16-year-old girl with a hyperpigmented macula which spread from her right anterolateral trunk to the inner part of her right thigh accompanied by ipsilateral mammary hypoplasia. The skin lesion started when the patient was seven years old when it was also noticed ipsilateral mammary hypoplasia. The histological exam confirmed the clinical hypothesis of Becker's nevus.     

Basal cell nevus syndrome: From DNA to therapeutics

Basal cell nevus syndrome, also known as Gorlin syndrome, is a hereditary cancer syndrome associated with multiple basal cell carcinomas, congenital defects, and nondermatologic tumors. This disease is autosomal dominant with variable expressivity and is caused by abnormalities in the sonic hedgehog signaling pathway. Management requires a multidisciplinary approach and should include the biopsychosocial needs of patients and their families. Genetic testing is necessary to confirm an unclear diagnosis, evaluate at-risk relatives, and assist with family planning.     

Blue rubber-bleb nevus syndrome: a case report

Blue rubber-bleb nevus syndrome (BRBNS) is an uncommon systemic disorder characterized by cutaneous and visceral cavernous hemangiomas. The characteristic rubbery textured and easily compressible lesions usually present in childhood and predominate cutaneously over the trunk and extremities. Gastrointestinal foci appear most commonly in the small bowel, a site that appears to dominate visceral involvement. We review the case of a 23-year-old white woman, whose numerous lesions on her trunk, extremities, and oral mucosa had been present since childhood.     

Epidermolysis bullosa nevus: case report and literature review

Acquired melanocytic lesions resembling malignant melanoma have been described in all major categories of Epidermolysis bullosa and referred to as "Epidermolysis bullosa nevi'. They easily induce to diagnostic error, although no malignant transformation has been reported. We report the development of a large acquired melanocytic nevus at a site of recurrent blisters in a 5-year-old child with Epidermolysis bullosa simplex. The global dermoscopic pattern was suggestive of benignity, and the histopathological findings were compatible with a compound melanocytic nevus. This is the first published case of Epidermolysis bullosa nevi in Brazilian literature. Despite their benign behavior, we emphasize the importance of regular clinical and dermoscopic monitoring, since a malignant course still cannot be totally excluded.     

蓝橡皮疱样痣综合征1例

患者男,17岁,农民。临床表现为全身分布约百余个大小不一的蓝紫色、蓝青色乳头样隆起,有弱性,柔软可压缩的血管瘤,同时伴有胃肠血管瘤损害以及消化道出血、贫血。     

黏蛋白痣一例并文献复习

<正>临床资料患者,男,44岁。右股部外侧增生性斑块30余年。患者30余年前,右侧股部外侧无明显诱因出现褐色增生性斑块,皮损逐渐扩大,无明显自觉症状。既往体健,一般情况好。否认该部位的外伤史及类似疾病的家族史。皮肤科查体:右股部外侧散在大小不等、形状不规则的褐色增生性斑块、结节,质软,沿Blaschko线分布(图1,2)。皮肤镜检查:可见表面无明显鳞屑,增生物呈皮色,皮损间可见深沟纹及点球状凹陷,呈脑回样,凹陷处有灰褐色色素沉着(图3)。组     

线状皮脂腺痣综合征1例

报告1例线性皮脂腺痣综合征，患者男，23岁，头皮中线，右前额至鼻尖和左面颊，下颌至颈部两片大面积皮脂腺痣，双眼角结膜胬肉样改变及高度近视眼底，X线片示蝶鞍增大，提示鞍区占位性病变。     

蓝色橡皮-大疱性痣综合征1例报道

蓝色橡皮-大疱性痣综合征(blue rubber-bleb syndrome,BRBNS)为一罕见综合征,现将我院所见1例报告如下。病例介绍患者,女,39岁。全身反复发作紫色结节36年。自3岁起全身出现多发性紫红色结节,曾诊断为血管瘤,后逐年增加,多达200余个,在当地医院曾行手术、激光切除治疗20余次。2003年患者因胃出血在当地医院行胃大部切除术,术后组织病理示:胃多发性血管瘤。     

Periungual Basal cell carcinoma: a case report with review of literature

Basal cell carcinomas (BCCs) are considered to be uncommon around the nail plate. An indolent lesion of this region should arouse suspicion of potential malignancy and a skin biopsy should be undertaken without delay. Early diagnosis can enable the physician to render simpler nondestructive modalities of treatment. In this article, we describe such a case of longstanding BCC of this region mimicking a traumatic ulcer. The nature of the ailment was finally discovered on biopsy and the carcinoma was initially treated with topical Imiquimod.     

Basal cell nevus syndrome with squamous cell carcinoma of the larynx

We report on a 54-year-old patient suffering from basal cell nevus syndrome. At the age of 51, squamous cell carcinoma of the larynx had been diagnosed. The frequent occurrence of various kinds of benign and malignant neoplasms in patients with basal cell nevus syndrome illustrates the close relationship between this syndrome and phacomatoses.     

Basal cell carcinoma of the umbilicus: a case report and literature review

We report the case of a 43-year-old woman with basal cell carcinoma (BCC) of the umbilicus. Although BCC is a common skin tumor, only 2 cases of BCC arising within the umbilicus have been reported previously. Our review of the literature shows that truncal BCCs frequently develop in younger patients, often grow larger, and are associated with an increased risk for developing multiple nonmelanoma skin cancers. Therefore, we advocate a low threshold for performing biopsies on umbilical lesions that are atypical in appearance, course, or response to therapy.     

Malignant blue nevus: a case report and molecular analysis

Malignant blue nevus is a rare melanocytic tumor that is described by some authors as a variant of malignant melanoma, whereas others regard it as a distinct entity. To our knowledge no molecular studies of this tumor have been performed, although the molecular pathogenesis of conventional melanomas has been extensively described. We present a case of malignant blue nevus that developed in a 15-cm congenital blue nevus on the back of a 41-year-old man. Subsequent regional lymph node and lung metastases developed within 1 and 29 months, respectively. We performed a molecular analysis for loss of heterozygosity on microdissected samples from the spectrum of benign to malignant blue nevus, using a panel of eight genes (MTS1, MXI1, CMM1, p53, NF1, L-myc hOGG1, and MCC), many of which are commonly associated with conventional melanomas. No loss of heterozygosity was detected, despite informativeness in seven genes. We suggest that malignant blue nevus may represent a distinct entity with a different molecular pathway to tumorigenesis than that of conventional melanomas.