1/7. Localized birt-hogg-dube syndrome with prominent perivascular fibromas.The autosomal dominant birt-hogg-dube syndrome is a cutaneo-intestinal condition that manifests on the skin in the form of multiple, skin-colored small papules that, histologically, prove to be mantleomas (fibrofolliculomas and trichodiscomas). These cutaneous lesions usually appear in the region of the head, neck, and upper part of the trunk. To date, only a single report in the literature describes the localized occurrence of this syndrome. We now describe a localized form of the birt-hogg-dube syndrome in a man with multiple mantleomas that were confined to the left half of the face, and which, in part, were arranged in the form of plaques. Another striking finding in this patient was a conspicuous vascular component in the lesions, characterized by a pronounced, well-demarcated fibrosis in the region of cutaneous blood vessel proliferations. Because perivascular fibromas have already been observed in other patients with Birt-Hogg-Dube-syndrome, the perivascular fibroma, with fibrofolliculoma and trichodiscoma, must be included within this syndrome's spectrum of skin changes.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
2/7. nevus lipomatosus cutaneous superficialis with perifollicular fibrosis.nevus lipomatosus cutaneous superficialis (NLCS) is a rare hamartomatous skin lesion histopathologically characterized by the presence of mature fat tissue even within the upper dermis. Clinically, two types of NLCS can be distinguished; a multiple type and a solitary type. We here report a 10-month-old girl showing multiple type NLCS as a collection of a nodule and papules on her right abdomen. Histological examination revealed that the lesion was composed of a lobular proliferation of fat tissue throughout the dermis and immature hair follicle-like structures with perifollicular fibrosis. Histological alterations of the dermal connective tissue components were also seen, including thickening of collagen bundles and increased numbers of both fibroblasts and blood vessels. This is the first reported case of NLCS with perifollicular fibrosis.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
3/7. Pilomatrix carcinoma with lymph node metastases.Pilomatrix carcinoma is a rare skin tumor with an origin from hair matrix cells. The tumor is locally aggressive with a great tendency for recurrence, but the metastatic potential is limited. A pilomatrix carcinoma in 76-year-old female with lymph node metastases is presented. In addition to classical histopathological criteria and dna ploidy analysis, a broad panel of antibodies was used for evaluation of the metastatic potential. Both primary tumor and lymph node metastasis revealed extremely high proliferation and apoptotic rates. High constant expressions of CD44v6 and P-cadherin were also observed. In the metastasis, significant reduction of E-cadherin and beta-catenin was detected. The best approach for assessment of metastatic potential of pilomatrix carcinoma seems to be the complex evaluation of routine histological criteria like vessel invasion, mitotic index, apoptotic count, and new molecular markers of cell death and adhesion.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
4/7. Pilomatricoma with a bullous appearance.BACKGROUND: Pilomatricoma is a benign, cutaneous neoplasm with differentiation toward hair matrix. The tumor is usually a deep-seated, solitary, firm nodule with overlying normal epidermis. Pilomatricoma with a bullous appearance is very rare. methods: A 16-year-old Chinese girl with a soft, purplish, translucent bulla on the left shoulder for 6 months and a nodule underlying the bulla is described. RESULTS: The histopathologic findings were consistent with pilomatricoma. There was extraordinary dilation of lymphatic vessels in the overlying dermis, which explains the clinical bullous appearance. The surrounding dermis had an edematous appearance. A Verhoeff-van Gieson stain disclosed the marked reduction of the elastic fibers, and an alcian blue stain was negative. CONCLUSIONS: The patient is diagnosed as bullous variant of pilomatricoma.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
5/7. Papillary endothelial hyperplasia and dilated lymphatic vessels in bullous pilomatricoma.This is a report of papillary endothelial hyperplasia in a 9-year-old girl with a pilomatricoma showing bullous appearance. Histologically, papillary proliferation of endothelial cells was found within dilated lymphatic endothelium-lined vascular channels overlying a pilomatricoma. The endothelial cells covering the papillae were of a lymphatic endothelial cell nature proved by immunohistochemistry and electron microscopy. Abundant fibrous long-spacing collagen was observed in the connective tissue and fibroblasts within papillae.- - - - - - - - - - ranking = 4keywords = vessel (Clic here for more details about this article) |
6/7. Angilymphoid hyperplasia appearing as a pilar cyst.A case of angiolymphoid hyperplasia presenting as a pilar cyst is presented herein. The clinical appearance of the lesion is described and its relationship to the blood vessel in which it grew is noted. Previous reports have shown neither the appearance of such a solitary lesion nor its intravascular growth. A review of the literature is also presented.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
7/7. Pilomatricoma with a bullous appearance.A 15-year-old Japanese girl had an asymptomatic nodule on the right thigh of seven months' duration. The clinical appearance was similar to that of a bulla. There was a history of blunt trauma from dog scratch to the skin over the tumor shortly before tumor growth. Histopathological findings were consistent with pilomatricoma. In the overlying dermis, the collagen bundles were compressed to the tissue surrounding the tumor and the large space was seen. Around the tumor, some dilated endothelium-lined vascular channels were found, which were identified as lymphatic vessels.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |