Isolated Collagenoma: A Case Report with a Review of Connective Tissue Nevi of the Collagen Type

A 22-year-old male had had five skin colored tumorous growths on the mid-palm, ring and little fingers of the right hand for the last 15 years. Histopathological examination with hematoxylin and eosin and special stains confirmed the diagnosis of connective tissue nevi of collagen type. There were no associated cutaneous or systemic findings; thus the case was designated as isolated collagenoma. A review of connective tissue nevi of the collagen type is presented.     

发疹性皮肤胶原瘤1例

报告发疹性皮肤胶原瘤1例,患者男,38岁。背部,肩部出现多发性扁平丘疹,结节及斑块8年,皮损为正常肤色,直径数毫米至数厘米,部分融合成斑块,无自觉症状,无家族史,组织病理检查示,胶原纤维变厚,增殖,弹性纤维减少,诊断为发疹性皮肤胶原瘤。     

A case of eruptive collagenoma localized on the neck and shoulders

A 78-year-old woman, who had first noticed asymptomatic eruptions on her neck and shoulders eight years earlier, presented with papules and nodules 2 to 20 mm in diameter that had a normal to white hue and were flatly elevated. These lesions were scattered and multiple, some forming confluent plaques. Histopathologically, the epidermis was slightly atrophied, and collagen fibers in the dermis were coarse and proliferated. In addition, the number of elastic fibers was slightly decreased. No complications were evident. Based on these findings, the patient was given a diagnosis of mild eruptive collagenoma, a type of connective tissue nevus according to the classification of Uitto. This case is unique in that onset was at an advanced age and that distribution was localized on the neck and shoulders.     

席纹状胶原瘤

报告1例席纹状胶原瘤。患者男,46岁。背部肿物40年余,缓慢增长,无自觉症状。无系统性疾病病史。皮损组织病理检查示表皮轻度萎缩,真皮内可见一边界清楚的结节,无包膜,病变均匀、局限,由大量透明样变的胶原束组成,胶原束之间见许多裂隙,排列成席纹状。诊断:席纹状胶原瘤。     

Familial cutaneous collagenoma: new affected family with prepubertal onset

Two siblings presented with clinical and histopathological findings of familial cutaneous collagenoma which is a rare connective tissue nevus, inherited in an autosomal-dominant pattern. A 13-year-old girl had oval-round, soft, painless papules, 5-10 mm in size and a total of 9-10 on her abdomen and flanks. Skin biopsy demonstrated dense, coarse collagen fibers in the dermis and a decrease in elastic fibers. Doppler echocardiography indicated an atrioseptal defect of the secundum type. Her 9-year-old brother was also examined; four lesions were discovered on his back but he was otherwise normal. Our cases comprise the sixth affected family to be reported in the medical published work and all lesions had appeared prepubertally.     

Progress of an isolated collagenoma during pregnancy

Collagenomas are connective tissue naevi composed predominantly of collagen. Isolated collagenomas are usually localized to a single body region, acquired, and of rare occurrence. We describe a patient with an isolated collagenoma that showed an increase in size during pregnancy and regressed afterwards.     

毛囊痣

报告1例毛囊痣。患者女,15岁。右颈前肤色丘疹15年,无自觉症状。皮肤科检查：右颈前一直径0．3cm肤色圆柱形丘疹,质地中等。组织病理检查示真皮内成熟毳毛毛囊明显增多,周围纤维组织鞘增厚,部分毛囊周围见皮脂腺结构,未见明显脂肪细胞和软骨结构。     

Pacinian collagenoma

An unusual histological variant of collagenoma is described. A 36-year-old woman presented with a lump in the left hypothenar eminence. Histological examination revealed a well-delineated lesion composed of paucicellular collagen fibres arranged in concentric lamellations giving rise to an onion skin appearance. The overlying epidermis was thin and the lateral borders were demarcated by an epidermal collarette. Inflammation and xanthoma cells were absent and occasional capillaries were present. The lesion was positive for collagen stains, reticulin and CD34. This lesion represents an uncommon histological form of collagenoma or fibroma. It can be distinguished from histological look-alikes on the basis of the characteristic morphology and immunophenotype.     

Isolated plantar collagenoma not associated with Proteus syndrome

Isolated plantar cerebriform collagenomas are a relatively rare type of connective tissue nevus. They have been suggested to be pathognomonic of Proteus syndrome. However, their presence is now considered to be a major criterion of Proteus syndrome, but the diagnosis of Proteus syndrome also requires the presence of other minor criteria. We present an unusual case of an acquired plantar cerebriform collagenoma, which is not associated with Proteus syndrome. Collagenomas, or connective tissue nevi of the collagen type, represent a hamartomatous overgrowth of normal collagen. Isolated plantar collagenoma is rare, and most commonly presents in childhood. We report an interesting case of an isolated plantar cerebriform collagenoma in an adult.     

白色萎缩合并未分类结缔组织病1例

报告1例白色萎缩合并未分类结缔组织病.患者女,33岁,双小腿出现许多象牙白色萎缩性瘢痕,伴毛细血管扩张,色素沉着,溃疡.皮肤组织病理检查见真皮浅层小血管内皮细胞增生,少数管腔闭锁,血管壁有纤维蛋白样物质沉积和透明变性,给予扩血管,抗凝,血浆置换治疗后好转.     

多发性Spitz痣一例

患儿,男,5岁。8个月时左侧鼻部出现米粒大褐色丘疹,逐渐增大成为红褐色结节,类似皮疹于左侧面部不断发生,病理检查示：真表皮交界处及真皮内弥散性梭形痣细胞浸润,呈对称分布,痣细胞巢垂直于表皮。免疫组化示：Ki-67（〈2%-3%＋）,HMB45（＋）,S-100（＋）。     

Elastic Fibers in Dermis of Juvenile Elastoma

To explore the ultrastructure of elastic fibers in juvenile elastoma, three patients (two without osteopoikilosis and one under examination of bones and joints) were studied by routine electron microscopy. In addition to normal elastic fibers, all the patients also exhibited alterations in elastic fibers. The altered ultrastructures showed lucent, homogenous matrix without peripheral microfibrils. The homogenous matrix were seen in various extensions from the small protrusions of the normal elastic fibers to the complete replacement of the entire fibers. Collagen fibrils occasionally showed twisted figures. Normal shapes of dermal glycosaminoglycans were increased in number. It seems likely that the lucent, homogenous matrix without peripheral microfibrils are the characteristic changes of elastic fibers in juvenile elastoma. The alteration could be nevoid in nature.     

Pacinian collagenoma: A distinct form of sclerotic fibroma

Sclerotic fibroma (storiform collagenoma) is a rare benign skin tumor. A solitary tumor, as well as multifocal lesions, are found either sporadically, or associated with Cowden syndrome. The tumor usually presents as clinically asymptomatic, slowly growing papule or nodule on the skin of the head, neck, and upper extremities. Microscopically the lesion is sharply demarcated, composed of hyalinized bands of collagen with low cellularity and a distinctive irregularly whorled or storiform pattern. We describe a case of a unique variant of this tumor in the scalp of a 33-year-old male. The tumor was microscopically composed of concentrically arranged collagen bundles with prevailing type III collagen, which resembled an enlarged Vater-Pacini corpuscle, with low density of CD34-positive and glucose transporter 1-negative spindle shaped cells. The specific microscopic appearance is suggestive of the term “Pacinian collagenoma” for this unique benign tumor.     

Cerebriform connective tissue nevus of lumbar

Connective tissue nevi represents a kind of hamartoma, and coalescence of the lesions in a cerebriform mode in the lumbar region without Proteus syndrome is rarely seen. Here, we report a 26‐year‐old woman presenting with nodules and plaques in her left lumbar region of 26 years in duration. Histopathological examination and Masson‐trichrome stain showed increased dermal collagen bundles in a haphazard array. The diagnosis of connective tissue nevi was made. This is the first case report on cerebriform connective tissue nevi without Proteus syndrome in the lumbar region.