1/17. Naevus varicosus osteohypertrophicus. An early diagnostic approach.Naevus varicosus osteohypertrophicus (synonym Klippel-Trenaunay Syndrome KTS) is relatively rare circumscribed, usually quadrant-related gigantism with vascular hyperplasia or malformations based on the embryonic development. The authors observed an 18- and a 30-year-old female with a triad of symptoms: cutaneous nevus flammeus, varicose and dilated veins, and bony and soft tissue hypertrophy of the low limb. The second patient also had two venous ulcers as a dominant clinical feature--a rare manifestation of Klippel-Trenaunay syndrome. A diagnosis of Klippel-Trenaunay syndrome was made by clinical observations, laboratory findings, dermoscan, radiological examination of the bones of the limb, Doppler ultrasonography, photopletismography and venoscan. A bone isotope scan was also done to the first patient. Making an early diagnosis of this sporadic congenital disease with unknown aetiology is important in order to be able to provide early prophylactic and therapeutic measures. Klippel and Trenaunay in 1900 were the first to describe a patient with the simultaneous appearance of osteohypertrophy, hemangiomas and varicose veins involving one extremity [1]. In 1907 Parkes and Weber reported a similar syndrome--they described a patient who had dilated and pulsatile arteries in the affected region including the presence of arterio-venous communications. In 1918 they used the compromise term "haemangiectatic hypertrophy" to embrace all conditions which were associated with congenital vascular malformations including A-V anastomoses associated with bone and soft tissue hypertrophy. Most authors are agreed that Klippel-Trenaunay syndrome and the syndrome of multiple congenital arterio-venous fistulae are two separate features of the Parkes Weber hypertrophy. KTS is manifesting with a triad of symptoms: cutaneous vascular nevus (more frequently nevus flammeus type), superficial venous varicosities and hypertrophy of the affected limb. Usually one quadrant of the body is involved: quite often a leg, an arm, lateral side of the trunk, very rarely the face. More than one quadrant and bilateral involvement are rarely affected. Naevus flammeus appeared at birth. It is extremely variable both in extent and in color--the latter ranging from pale pink to deep purple. Veinous varicosities appear in childhood and adolescence. They are painful and may be complicated by superficial or deep venous thrombosis and rarely, ulceration. hypertrophy of the affected extremities is due to bone and soft tissue hypertrophy. KTS can be associated with other developmental anomalies such as: polydactyly, syndactyly, oligodactyly [2] macrocephaly, blue nevus, epidermal naevus, venous malformations.- - - - - - - - - - ranking = 1keywords = naevus (Clic here for more details about this article) |
2/17. Phacomatosis pigmentovascularis type IIa successfully treated with two types of laser therapy.We describe a 28-year-old Japanese woman with phacomatosis pigmentovascularis type IIa who was treated by 27 sessions of Q-switched ruby laser irradiation to the site of dermal melanosis on her face and three sessions of dye laser irradiation to the port-wine stain on her left cheek. This is the first report of the successful treatment of a cutaneous lesion in a patient with phacomatosis pigmentovascularis. As the outcome of the treatments was excellent, we conclude that phacomatosis pigmentovascularis type IIa can be treated successfully by the combination of the two types of laser therapy.- - - - - - - - - - ranking = 0.00011965249587898keywords = cell (Clic here for more details about this article) |
3/17. Acquired port-wine stains and antecedent trauma: case report and review of the literature.BACKGROUND: While cases of congenital port-wine stains (PWSs) are relatively common, cases of acquired PWSs are quite rare. Many of the reported cases of the acquired type have been reported to be related to previous trauma. OBSERVATIONS: We encountered a case of acquired PWSs in a 3-year-old girl. Her parents noted that the lesions appeared in areas of antecedent trauma. This prompted us to review all reported cases of acquired PWSs and to assess the relationship to trauma. Among the 59 cases reported, there was a slight female predominance (female-male ratio, 35:24). Seventeen (29%) of the cases were related to trauma. One report evaluated the effectiveness of lasers in the treatment of acquired PWSs and found that 54% of patients treated with pulsed dye lasers had an excellent response or complete clearance. CONCLUSIONS: Port-wine stains are not only congenital but can be acquired as well. Trauma can be one of the causes of acquired PWSs. To explain this phenomenon, various theories, including abnormal vascular repair and altered vascular innervation, have been proposed. lasers are the treatment of choice in all cases of PWSs and may be more effective in those that are acquired.- - - - - - - - - - ranking = 0.00011965249587898keywords = cell (Clic here for more details about this article) |
4/17. Basal cell carcinomata in port wine stains.The development of basal cell carcinoma (BCC) in a port wine stain is rare and the aetiology of these malignancies in this situation is the subject of some debate. Early treatment of port wine stains with ionising radiation has been implicated in the development of BCC. The use of the argon laser has not been implicated. We present four cases of BCC in port wine stains, one previously treated with an argon laser, and discuss the possible aetiological factors.- - - - - - - - - - ranking = 0.00059826247939489keywords = cell (Clic here for more details about this article) |
5/17. 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 = 4keywords = naevus (Clic here for more details about this article) |
6/17. Pyogenic granuloma appearing on port-wine stain: a case report.BACKGROUND: Pyogenic granuloma has been reported to be associated with hemangiomas and hamartomas, including port-wine stain. It has been suggested that the spontaneous development of pyogenic granuloma in port-wine stain might be associated with microscopic arteriovenous anastomoses in highly vascularized areas such as the fingers, hands, lips, tongue and face. CASE REPORT: A 25-year-old male patient presented with a history of a reddish, solitary nodule on the posterior cervical area for eight months. There had been an associated port-wine stain at the same localization since birth. physical examination revealed a solitary, strawberry-like dome-shaped papule, 12 mm in diameter, within a well-demarcated reddish colored surrounding patch which had been present on the posterior cervical area. The lesion was excised, considering pyogenic granuloma arising in a port-wine stain. Histopathologic examination showed a mass of capillaries with variable luminal diameters, infiltration of inflammatory cells, and immature endothelial proliferation in the upper dermis. The capillaries were organized into lobules separated by fibrous stroma and were surrounded by an epithelial collarette. Immunohistochemical staining for factor viii-related antigen supported all these findings of the port-wine stain. CONCLUSION: We present a pyogenic granuloma arising in port-wine stain on the posterior cervical area, since the posterior cervical area is not as highly vascular as the fingers, hands, lips, tongue and face. We believed that the collar of the patient's shirt continuously traumatized port-wine stain, and consequently led to the development of pyogenic granuloma.- - - - - - - - - - ranking = 0.00011965249587898keywords = cell (Clic here for more details about this article) |
7/17. Encephalotrigeminal angiomatosis.We report an unusual case of encephalotrigeminal angiomatosis in which the facial and oral angioma was bilateral, and several teeth were congenitally absent. The developmental nature of the anomaly is reviewed. Encephalotrigeminal angiomatosis is commonly referred to as the sturge-weber syndrome, after Sturge and Weber who first described this affliction in 1879. The main clinical features of this syndrome are 1. venous angiomatosis of the leptomeninges of the cerebral-cortex, usually unilaterally 2. ipsilateral facial angiomatosis that often follows in outline the distribution of the trigeminal nerve (Fig 1) 3. ipsilateral gyriform calcification of the cerebral cortex 4. epileptic convulsions (contralateral focus) or other seizures 5. ocular defects (choroidal angioma, glaucoma hemianopia) 6. mental retardation 7. contralateral hemiplegia 8. obesity 9. oral mucosal and gingival involvement. Other less typical features are 1. association with hypomelanosis of 1 to 10 2. leptomeningeal angioma contralateral to the facial nevus 3. leptomeningeal angioma without facial vascular naevus 4. association with gastro intestinal hemorrhage 5. paranasal sinus enlargement. This syndrome that affects males and females equally, is a rare congenital disorder, apparently hamartomatous in nature, from persistence of a primitive embryonal vascular plexus. During the sixth week of intra-uterine life this plexus develops around the cephalic portion of the neural tube and under the ectoderm in the region destined to become facial skin. In the sturge-weber syndrome, the vascular plexus fails to regress, as is normal during the ninth week, resulting in angiomatosis of the related tissues. Variation in the degree of persistence or regression of the vascular plexus accounts for unilaterality or bilaterality of involvement, and also for an incomplete syndrome in which the leptomeninges, but not the facial tissues are affected. Leptomeningeal angiomatosis is the primary abnormality of encephalotrigeminal angiomatosis, all other features of the syndrome probably being secondary to this. Calcification of the cortex is a poorly understood phenomenon which may result from stasis of blood in the angioma, associated with altered local metabolism. epilepsy and other neurological seizures, and mental retardation are probably, in their turn, secondary to the cortical calcification. The most striking clinical feature of the sturge-weber syndrome is the facial vascular naevus which generally follows the distribution of innervation of one or more divisions of the trigeminal nerve, whence the term encephalotrigeminal angiomatosis. However, the naevus may be more extensive, down the neck and even onto the chest. The oral tissues underlying the affected facial tissues are invariably also angiomatous and may be considerably enlarged as a result. Alterations in eruption of teeth have also been noted. Histologically, affected soft tissues are very vascular, resembling a pyogenic granuloma or a capillary, or cavernous hemangioma. Yukna, Cassingham and Carr noted that affected bone was partially replaced by a delicate fibrous tissue containing thin-walled vascular spaces. Neither inflammatory cells, nor fatty or haemopoietic marrow was noted.- - - - - - - - - - ranking = 3.0001196524959keywords = naevus, cell (Clic here for more details about this article) |
8/17. Widespread capillary malformation associated with global developmental delay and megalencephaly.Although port wine stains are seen in 0.3% births, widespread cutaneous capillary malformations are unusual and an association with static gliosis has not been previously reported. This is a report of a 3-year-old boy with a fixed widespread capillary naevus (port wine stain), megalencephaly and global developmental, and features of gliosis on brain magnetic resonance imaging (MRI).- - - - - - - - - - ranking = 1keywords = naevus (Clic here for more details about this article) |
9/17. Multifocal basal cell carcinoma developing in a facial port wine stain treated with argon and pulsed dye laser: a possible role for previous radiotherapy.BACKGROUND: Basal cell carcinoma occurring in port wine stain is extremely rare but has been reported after radiotherapy for port wine stain. OBJECTIVE: The objective was to present 55-year-old man with a facial port wine stain who had multiple treatment sessions with both the argon laser and the pulsed dye laser and subsequently developed a recurrent multifocal basal cell carcinoma and highlight the treatment carried out and its effects on the outcome. methods: We describe the history of the patient's port wine stain treatment, the development of skin cancer within it, and the different modalities of therapy carried out. RESULTS: The patient developed basal cell carcinoma in his port wine stain. The possibility of a causal link between laser treatment and this skin cancer was considered but it was discovered that he had had radiotherapy treatment of his birthmark at age 9. The basal cell carcinoma was successfully treated. CONCLUSION: This case of a clinically indistinct multifocal basal cell carcinoma arising within facial port wine stain was most likely due to previous radiotherapy treatment as a child, rather than laser treatment. Careful treatment history should identify such patients who should be followed-up for development of skin cancer.- - - - - - - - - - ranking = 0.0010768724629108keywords = cell (Clic here for more details about this article) |
10/17. Basal cell carcinoma arising in a port-wine stain.BACKGROUND: The occurrence of basal cell carcinoma within a port-wine stain or nevus flammeus is rare. Sixteen cases of basal cell carcinoma which developed in a port-wine stain or nevus flammeus have been reported. OBJECTIVE: The objective was to demonstrate a rare case of basal cell carcinoma occurring in a port-wine stain successfully treated with Mohs micrographic surgery. methods: This is a case report and literature review. RESULTS: An 87-year-old man presented with a basal cell carcinoma on the margin of a previously untreated port-wine stain on the left cheek. Histologic examination showed a nodular basal cell carcinoma. The basal cell carcinoma was completely excised with Mohs micrographic surgery and complex linear closure was used to repair the wound in layers. The postoperative course was complicated by a hematoma, which developed 24 hr postoperatively. The hematoma was drained and there was no further bleeding or evidence of recurrence of the tumor after 12 months. CONCLUSION: Basal cell carcinoma should be included in the differential diagnosis of a skin lesion occurring in a port-wine stain.- - - - - - - - - - ranking = 0.0013161774546688keywords = cell (Clic here for more details about this article) |
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