Port-wine stains. A disease of altered neural modulation of blood vessels?

Smoller, BR

doi:10.1001/archderm.122.2.177

Cited by 142 publications

(116 citation statements)

References 11 publications

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“…16 In contrast, studies using antibodies against neuronal cytoplasmic protein (PGP 9.5), neuronspecific enolase (NSE), and S100 protein have demonstrated that the density of cutaneous nerves is significantly decreased in CM. 17,18 These findings support the hypothesis that the gradual dilatation of the dermal vessels is the result of abnormal neural regulation of blood flow. 17 However, the decreased density of cutaneous nerves can be a secondary effect due to reduced blood flow and chronic ischemia in the lesions.…”

Section: Introductionsupporting

confidence: 78%

“…17,18 These findings support the hypothesis that the gradual dilatation of the dermal vessels is the result of abnormal neural regulation of blood flow. 17 However, the decreased density of cutaneous nerves can be a secondary effect due to reduced blood flow and chronic ischemia in the lesions. 19 In an effort to understand the genetic mechanisms that underlie CM, and are likely to control cutaneous vascular morphogenesis, we performed genome-wide linkage analysis on six families with inherited CM (families A -F, Figure 2).…”

Section: Introductionsupporting

confidence: 78%

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Locus for susceptibility for familial capillary malformation (‘port-wine stain’) maps to 5q

et al. 2002

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Capillary malformation (CM; 'port-wine stain'), is a common vascular malformation affecting cutaneous capillary vessels in 0.3% of newborns. Increased incidence of lesions in first-degree relatives of these patients and several reported familial cases suggest that genetic factors may play a role in the pathogenesis of CM. We report the first genome-wide linkage analysis of familial CM. In the non-parametric linkage analysis, strong evidence of linkage (peak Z-score 6.72, P-value 0.000136) was obtained in an interval of 69 cM between markers D5S407 and D5S2098, corresponding to 5q11 -5q23. Parametric linkage analysis gave a maximum combined HLOD score of 4.84 (a-value 0.67) at marker D5S2044 on 5q15, and analysis using only the linked families, defined a smaller, statistically significant locus CMC1 of 23 cM (peak LOD score 7.22) between markers D5S1962 and D5S652 corresponding to 5q13 -5q15. Interesting candidate genes implicated in vascular and neural development, such as MEF2C, RASA1, and THBS4, are in this locus.

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Section: Introductionsupporting

confidence: 78%

Section: Introductionsupporting

confidence: 78%

Locus for susceptibility for familial capillary malformation (‘port-wine stain’) maps to 5q

et al. 2002

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“…The pathogenesis of this vascular dilatation is unknown but previous studies have proposed a reduction in neural innervation in areas of skin with port wine stain involvement. [5][6][7] Our confocal images confirm a significant decrease in nerve density from samples of both untreated and treated (po0.01) port wine stain skin compared to uninvolved skin. Our findings add support to the theory that port wine stain vascular ectasia may result from decreased neural innervation.…”

Section: Discussionsupporting

confidence: 61%

“…4 Previous studies proposed that the pathogenesis of port wine stain is related to a reduction of neural innervation around the ectatic blood vessels. [5][6][7][8][9] The neural defect is likely to be autonomic in nature because there is no sensory loss within port wine stain. Blood flow in the absence of tonic modulation is thought to produce port wine stain vascular ectasia.…”

Section: Mona M Selim Md Kristen M Kelly Md J Stuart Nelson Mmentioning

confidence: 99%

Confocal Microscopy Study of Nerves and Blood Vessels in Untreated and Treated Port Wine Stains: Preliminary Observations

et al. 2004

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Background and Objective. Vascular ectasia in port wine stain birthmarks (PWS) might result from reduced innervation with loss of autonomic stimulation. We investigated this theory and evaluated nerve and blood vessel density, and mean blood vessel size in untreated and treated PWS skin. Methods. Skin biopsy specimens were obtained from uninvolved skin, untreated PWS, PWS with a good response to laser treatment and PWS with a poor response to laser treatment. Confocal microscopy was performed to determine nerve and blood vessel density, and mean blood vessel size. Results. Nerve density was significantly decreased in all PWS sites compared to uninvolved skin. Mean blood vessel diameter was larger in untreated compared to treated PWS. PWS with a good response to treatment had decreased nerve density but blood vessel density and mean diameter was relatively normal. PWS with a poor response to treatment had decreased nerve density but increased blood vessel density and mean blood vessel diameter compared to normal skin. Conclusion. Nerve density was decreased in all evaluated PWS sites and this may be a factor in lesion pathogenesis. PWS blood vessel size correlated with pulsed dye laser response and may prove to be a useful prognostic indicator of therapeutic outcome.

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“…The causes of PWS are not well understood, but in the late 1980s it was proposed that one cause could be the reduction of neural innervation around the vessels [17]. A recent article used a confocal microscope to determine nerve and blood vessel density and mean vessel size in PWSs [18].…”

Section: Recent Advances In Understanding the Causes For Port-wine Stmentioning

confidence: 99%

Selected Laser-based Therapies in Otolaryngology

Mioc

Mycek

2005

Otolaryngologic Clinics of North America

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Port-wine stains. A disease of altered neural modulation of blood vessels?

Cited by 142 publications

References 11 publications

Locus for susceptibility for familial capillary malformation (‘port-wine stain’) maps to 5q

Locus for susceptibility for familial capillary malformation (‘port-wine stain’) maps to 5q

Confocal Microscopy Study of Nerves and Blood Vessels in Untreated and Treated Port Wine Stains: Preliminary Observations

Selected Laser-based Therapies in Otolaryngology

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