1/8. Arteriovenous and lymphatic malformations, linear verrucous epidermal nevus and mild overgrowth: another hamartoneoplastic syndrome?We report a 22 year old female presenting with slowly progressive paraparesis, who appeared to have many (mainly subcutaneous) hamartomas. The neurological symptoms were caused by intraspinal masses and arteriovenous malformations. In addition, she had mild overgrowth of one leg and lymph vessel malformations. This combination of symptoms resembles proteus syndrome, but is different in symptomatology and progression and may be yet another hamartoneoplastic syndrome.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
2/8. Naevus anaemicus with teleangiectatic vessels.We describe a 20-year-old man with naevus anaemicus on the chest where, after dermabrasion of the epidermis, enlarged teleangiectatic dark-red vessels were seen within the previously pale area. They were clearly different from those seen on dermabrasion at this site in normal skin and in patients with vitiligo where the area is lighter red with only small punctual bleedings from arterial capillaries. The naevus anaemicus and a port-wine stain (naevus flammeus) in the same location is a phenomenon of vascular twin spotting, which was revealed when the epidermis was removed. The area was transplanted with thin epidermal grafts and healed within 2 weeks. One year later the naevus looked the same as before grafting. Much thicker grafts than those used by us will be needed, but they are not cosmetically acceptable.- - - - - - - - - - ranking = 5keywords = vessel (Clic here for more details about this article) |
3/8. Uncommon vascular naevi associated with focal acantholytic dyskeratosis.Cutis marmorata telangiectatica congenita and vascular twin naevi are rare vascular anomalies in which focal acantholytic dyskeratosis is usually not observed. We describe a 44-year-old-man who presented for evaluation of skin lesions that had been present since birth. physical examination revealed anaemic macules adjacent to a naevus telangiectaticus on the chest. Naevus anaemicus was also seen on the shoulders, arms, and left leg. There was bluish-reddish reticulate marking of the skin and cutaneous atrophy. Shortening and hypoplasia of the left leg was observed. Histologic examination of two biopsy specimens revealed focal acantholytic dyskeratosis. In vivo confocal laser scanning microscopy showed dilated capillaries and vessels of the upper dermal plexus in the telangiectatic and decreased capillary blood flow in the anaemic skin sites. The findings were consistent with a diagnosis of cutis marmorata telangiectatica congenita, vascular twin naevi, and incidental focal acantholytic dyskeratosis. The particularities of the present case are the following: firstly, the association of two rare vascular anomalies to which the genetic concept of mosaicism can be applied; secondly, the occurrence of incidental focal acantholytic dyskeratosis in sites of vascular naevi.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
4/8. 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) |
5/8. The pre-operative embolisation of vascular malformations.Large arteriovenous malformations frequently require surgical excision in order to prevent or treat the potentially serious problems they can cause ranging from haemorrhage and pain to cardiac failure. The surgery itself is usually difficult and often dangerous due to the serious risk of major intraoperative haemorrhage. Transcatheter arterial embolisation has greatly facilitated the management of small arteriovenous malformations but may only afford temporary relief of symptoms in very large lesions. Recanalisation of occluded vessels and revascularisation via previously insignificant collateral vessels means that large lesions cannot be effectively managed by this method alone. Previous surgery and ligation of feeding vessels may make effective embolisation difficult or impossible and a combined radiological and surgical approach to these lesions may permit definitive treatment. We present three cases in whom the pre-operative embolisation of buttock arteriovenous malformations facilitated successful surgical excision.- - - - - - - - - - ranking = 3keywords = vessel (Clic here for more details about this article) |
6/8. Unilateral nevoid telangiectasia and chronic liver disease. Report of a case and review of the literature.Unilateral nevoid telangiectasia was observed on the back of the left hand and wrist in a 19-year old man with chronic liver disease and portal hypertension. Moreover, abnormalities of the elastic fibers of the skin were found. Examination of the liver biopsy showed extensive portal fibrosis with many dilated blood vessels. No usual cause of juvenile cirrhosis was detected. In the comment, the authors suggest that unilateral nevoid telangiectasia and liver disease could be manifestations of a disease involving skin and liver vessels, as in hereditary hemorrhagic telangiectasia.- - - - - - - - - - ranking = 2keywords = vessel (Clic here for more details about this article) |
7/8. A case of apple-shaped pedunculated lipofibroma.In 1975, Mehregan et al (1) proposed the term pedunculated lipofibroma for the solitary form of NLCS in the view of its distinctive clinicopathologic features. Subsequently, Nogita et al reported and reviewed 32 cases of pedunculated lipofibroma in 1994. Pedunculated lipofibroma is a relatively rare form of benign connective tissue proliferation abnormality characterized by ectopic adipose tissue in the dermis. We observed a case of pedunculated lipofibroma with a prominent stalk containing feeding vessels, a history of congenital onset, a remarkable globular apple-shaped mass and an accompanying small sessile lesion. We report this case with a review of the literature.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
8/8. Ocular naevus and hyperaemia with dilation of the conjunctival vessels: an early manifestation of Crohn's disease?Two cases of male patients with Crohn's disease showing the same neonatal ocular abnormality, a sector hyperaemia with dilation of the vessels of the bulbar conjunctiva surrounding a naevus close to the limbus, are presented. In both cases, this manifestation worsened when Crohn's disease relapsed, and improved when the disease went into remission with steroid treatment. In Crohn's disease, eye involvement is reported in varying percentages, but the condition discussed here does not fit into any of the ocular patterns previously described in this disease, and could represent an early manifestation of Crohn's disease.- - - - - - - - - - ranking = 5keywords = vessel (Clic here for more details about this article) |