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The British Journal of Dermatology Dec 2017Port-wine stain (PWS) is a vascular malformation characterized by progressive dilatation of postcapillary venules, but the molecular pathogenesis remains obscure.
BACKGROUND
Port-wine stain (PWS) is a vascular malformation characterized by progressive dilatation of postcapillary venules, but the molecular pathogenesis remains obscure.
OBJECTIVES
To illustrate that PWS endothelial cells (ECs) present a unique molecular phenotype that leads to pathoanatomical PWS vasculatures.
METHODS
Immunohistochemistry and transmission electron microscopy were used to characterize the ultrastructure and molecular phenotypes of PWS blood vessels. Primary culture of human dermal microvascular endothelial cells and in vitro tube formation assay were used for confirmative functional studies.
RESULTS
Multiple clinicopathological features of PWS blood vessels during the development and progression of the disease were shown. There were no normal arterioles and venules observed phenotypically and morphologically in PWS skin; arterioles and venules both showed differentiation impairments, resulting in a reduction of arteriole-like vasculatures and defects in capillary loop formation in PWS lesions. PWS ECs showed stemness properties with expression of endothelial progenitor cell markers CD133 and CD166 in non-nodular lesions. They also expressed dual venous/arterial identities, Eph receptor B1 (EphB1) and ephrin B2 (EfnB2). Co-expression of EphB1 and EfnB2 in normal human dermal microvascular ECs led to the formation of PWS-like vasculatures in vitro, for example larger-diameter and thick-walled capillaries.
CONCLUSIONS
PWS ECs are differentiation-impaired, late-stage endothelial progenitor cells with a specific phenotype of CD133 /CD166 /EphB1 /EfnB2 , which form immature venule-like pathoanatomical vasculatures. The disruption of normal EC-EC interactions by coexistence of EphB1 and EfnB2 contributes to progressive dilatation of PWS vasculatures.
Topics: Adolescent; Adult; Arterioles; Cells, Cultured; Child; Child, Preschool; Dilatation, Pathologic; Endothelial Progenitor Cells; Female; Humans; Infant; Infant, Newborn; Male; Middle Aged; Port-Wine Stain; Receptor, EphB1; Receptor, EphB2; Skin; Skin Diseases, Vascular; Venules; Young Adult
PubMed: 28599054
DOI: 10.1111/bjd.15716 -
Indian Dermatology Online Journal Jan 2012Hemangiomas are neoplastic proliferations of endothelial cells, characterized by a period of growth after birth, and eventual spontaneous involution. The course can be...
Hemangiomas are neoplastic proliferations of endothelial cells, characterized by a period of growth after birth, and eventual spontaneous involution. The course can be uneventful with spontaneous resolution; or it may be marked by complications such as infection, bleeding, ulceration, visual defects and feeding difficulties. Apart from these, rare life-threatening complications such as congestive heart failure and consumption coagulopathy may also be seen. Although hemangiomas commonly occur in the head and neck region, intraoral occurrence is relatively rare. A port wine stain is defined as a macular telangiectatic patch which is present at birth and remains throughout life. They may be localized or extensive, affecting a whole limb. This article reports a rare case of co-occurrence of port wine stain with intraoral hemangioma.
PubMed: 23130257
DOI: 10.4103/2229-5178.93493 -
Cureus May 2020Klippel-Trenaunay Syndrome (KTS) is a rare and sporadic congenital disorder, characterized by the classical triad of port-wine stains, varicosities along with bone and... (Review)
Review
Klippel-Trenaunay Syndrome (KTS) is a rare and sporadic congenital disorder, characterized by the classical triad of port-wine stains, varicosities along with bone and soft tissue hypertrophy. Symptoms of Klippel-Trenaunay Syndrome include pain, swelling, lymphedema, bleeding, superficial thrombophlebitis, and deep vein thrombosis. The etiology remains indistinct and has been attributed to both genetic and environmental factors. In most cases, a thorough history and clinical examination is enough for the diagnosis of Klippel Trenaunay Syndrome. However, when certain complications are present, noninvasive imaging techniques are used for the diagnosis and evaluation of the disease in patients. Due to the diversity of presentation, a multidisciplinary approach is essential for the proper management of such patients. At present, there is no cure for the disease; rather, symptomatic treatment is employed in order to improve the patients' quality of life. In this review, we provide a brief overview of the clinicopathological profile and management of Klippel-Trenaunay Syndrome.
PubMed: 32528762
DOI: 10.7759/cureus.8023 -
BioMed Research International 2020This study aims at exploring the clinical efficacy and sonographic changes of photodynamic therapy (PDT) using Hematoporphyrin Monomethyl Ether (HMME) for the treatment... (Observational Study)
Observational Study
This study aims at exploring the clinical efficacy and sonographic changes of photodynamic therapy (PDT) using Hematoporphyrin Monomethyl Ether (HMME) for the treatment of port-wine stains (PWS). Forty-five patients with PWS were recruited between March 2017 and June 2018 from the Department of Dermatology of The Third Affiliated Hospital of Soochow University. Five cases were of the pink type, thirty-nine cases were of the purple-red type, and one case was of the thickened type. All patients received three treatment sessions of PDT. After covering normal skin outside the treated area, patients received an intravenous injection of 5 mg/kg HMME within 20 minutes. The affected areas were exposed to a 532 nm LED light and were kept vertically at a distance of 10 cm. The irradiation energy density was set between 80 and 110 J/cm in 15-minute sessions. Intermittent power density adjustment was performed at a rate of 5 mW/cm, and the treatment was withheld when the endpoint reaction appeared. Three follow-ups were performed before and after treatment, respectively, and the efficacy, thickness, and density of skin before and after treatment were evaluated with high-frequency ultrasound. The overall efficacy rate was 97.78% in forty-five cases after treatment for three sessions. Efficacy was related to age ( = 0.029) and lesion severity ( < 0.001). There were significant differences in the efficacy between the groups of <18 years old, 18-29 years old, and >29 years old ( = 0.029). A marked decrease in the numbers of distorted enlarged blood vessels per unit of the lesion was observed under high-frequency ultrasound. There were significant differences in skin thickness and skin density before and after treatment ( = 14.528, 5.428, < 0.001). The swelling was reported to varying degrees in the treated areas in 23 patients with cheek lesion and in 6 frontal lesions. Hyperpigmentation after inflammation was observed in four patients that faded spontaneously after two months. In conclusion, photodynamic therapy for the treatment of PWS using HMME is effective and safe with few adverse reactions. Moreover, monitoring the changes in skin thickness and density of lesion tissue using high-frequency ultrasound can objectively evaluate the clinical efficacy of HMME photodynamic therapy and provide the basis for the formulation of individualized photodynamic therapy.
Topics: Adolescent; Adult; Female; Hematoporphyrins; Humans; Male; Photochemotherapy; Photosensitizing Agents; Port-Wine Stain; Ultrasonography
PubMed: 32802859
DOI: 10.1155/2020/6030581 -
Journal of the American Academy of... Aug 2012Port wine stains (PWS) are the most common vascular malformation of the skin, occurring in 0.3% to 0.5% of the population. Noninvasive laser irradiation with... (Review)
Review
Port wine stains (PWS) are the most common vascular malformation of the skin, occurring in 0.3% to 0.5% of the population. Noninvasive laser irradiation with flashlamp-pumped pulsed dye lasers (selective photothermolysis) currently comprises the gold standard treatment of PWS; however, the majority of PWS fail to clear completely after selective photothermolysis. In this review, the clinically used PWS treatment modalities (pulsed dye lasers, alexandrite lasers, neodymium:yttrium-aluminum-garnet lasers, and intense pulsed light) and techniques (combination approaches, multiple passes, and epidermal cooling) are discussed. Retrospective analysis of clinical studies published between 1990 and 2011 was performed to determine therapeutic efficacies for each clinically used modality/technique. In addition, factors that have resulted in the high degree of therapeutic recalcitrance are identified, and emerging experimental treatment strategies are addressed, including the use of photodynamic therapy, immunomodulators, angiogenesis inhibitors, hypobaric pressure, and site-specific pharmaco-laser therapy.
Topics: Angiogenesis Inhibitors; Humans; Immunosuppressive Agents; Low-Level Light Therapy; Port-Wine Stain
PubMed: 22305042
DOI: 10.1016/j.jaad.2011.11.938 -
Annals of Translational Medicine Dec 2019This study aimed to assess the different types of port-wine stain (PWS) skin lesions quantitatively using high-frequency ultrasound (US) and shear wave elastography...
BACKGROUND
This study aimed to assess the different types of port-wine stain (PWS) skin lesions quantitatively using high-frequency ultrasound (US) and shear wave elastography (SWE) before and after treatment, and investigate the feasibility and application value of high-frequency US and SWE in PWSs.
METHODS
A total of 195 PWS patients with 238 skin lesions before treatment and 72 follow-up PWS patients with 90 skin lesions were assessed using high-frequency US and SWE. The skin lesions were divided into four groups: pink-type, purple-type, thickened-type, and nodular-type PWSs. Gray-scale US was used to observe normal skin, observe the skin changes of lesions, and assess the skin thickness. The thickened skin was calculated. Power Doppler (PD) signal grades were used to assess the skin blood signals. SW velocity (in m/s) and Young's elastic modulus (in kPa) were used to assess the stiffness of normal skin and skin lesions. The heightened SWE was also calculated.
RESULTS
The dermis hypoechogenicity, thickness of thickened skin, and skin PD signal grades were significantly higher in all PWS-type groups compared with the normal-skin group (all P<0.05). The thickened skin and skin PD signal grades in the nodular-type and thickened-type group were significantly thicker and higher than those in the pink-type and purple-type group (all P<0.05). The PD signal grades in the purple-type was significantly higher than that in the pink-type group (P<0.05). All SWE values of PWS lesions were significantly higher in the transverse section than those in the longitudinal section (all P<0.05). The differences in heightened E, E, C, and C between each PWS group and the normal-skin group were not significant. The heightened E and C in the nodular-type PWS group was significantly higher than those in the normal-skin group and pink-type, and purple-type PWS groups (all P<0.05). The heightened E and C were significantly higher in the thickened-type PWS group than those in the normal-skin group (all P<0.05). In the evaluation of therapeutic effects, the ratio of dermis hypoechogenicity in pink-type lesions significantly decreased, and thickened skins in all groups were significantly thinned (all P<0.05). The differences of PD signal grades, heightened E, and C in all groups between pre-treatment and post-treatment showed no significance.
CONCLUSIONS
High-frequency US and SWE show feasibility and application values assessing PWS skin lesions. Their features include dermis hypoechogenicity, thicker skin, higher PD signal grades, higher E, and higher C. Thicker skin is thus the best feature for assessing therapeutic effect.
PubMed: 32042819
DOI: 10.21037/atm.2019.12.57 -
Journal of the American Academy of... Apr 2012Pulsed dye laser (PDL) is the gold standard for treatment of port-wine stain (PWS) birthmarks but multiple treatments are required and complete resolution is often not... (Randomized Controlled Trial)
Randomized Controlled Trial
BACKGROUND
Pulsed dye laser (PDL) is the gold standard for treatment of port-wine stain (PWS) birthmarks but multiple treatments are required and complete resolution is often not achieved. Posttreatment vessel recurrence is thought to be a factor that limits efficacy of PDL treatment of PWS. Imiquimod 5% cream is an immunomodulator with antiangiogenic effects.
OBJECTIVE
We sought to determine if application of imiquimod 5% cream after PDL improves treatment outcome.
METHODS
Healthy individuals with PWS (n = 24) were treated with PDL and then randomized to apply posttreatment placebo or imiquimod 5% cream for 8 weeks. Chromameter measurements (Commission Internationale de l'Eclairage L∗a∗b∗ colorspace) for 57 PWS sites (multiple sites per patient) were taken at baseline and compared with measurements taken 8 weeks posttreatment. The Δa∗ (change in erythema) and ΔE (difference in color between normal-appearing skin and PWS skin) were measured to quantify treatment outcome.
RESULTS
Two patients developed minor skin irritation. Other adverse effects were not noted. Average ∆a∗ was 0.43 for PDL + placebo sites (n = 25) and 1.27 for PDL + imiquimod sites (n = 32) (P value = .0294) indicating a greater reduction in erythema with imiquimod. Average ∆E was 2.59 for PDL + placebo and 4.08 for PDL + imiquimod (P value = .0363), again indicating a greater color improvement with imiquimod.
LIMITATIONS
Effects were evaluated after a single treatment and duration of effect is unknown.
CONCLUSION
Combined selective photothermolysis and antiangiogenic therapy may enhance PWS treatment efficacy.
Topics: Adjuvants, Immunologic; Adolescent; Adult; Aged; Aminoquinolines; Combined Modality Therapy; Female; Humans; Imiquimod; Lasers, Dye; Male; Middle Aged; Port-Wine Stain; Single-Blind Method; Young Adult
PubMed: 22244840
DOI: 10.1016/j.jaad.2011.11.958 -
Australian Family Physician May 2012Birthmarks are common in newborns, and their presence can cause much anxiety in new parents.
BACKGROUND
Birthmarks are common in newborns, and their presence can cause much anxiety in new parents.
OBJECTIVE
This article provides an update on common birthmarks and identifies those complex subtypes that may indicate potentially important associations or outcomes.
DISCUSSION
Birthmarks encompass a range of lesions presenting at birth or soon after. They can be divided into vascular, epidermal, pigmented and other subtypes. This article focuses on common birthmarks to help identify patients requiring specific intervention and explores recent developments in management. A minority of higher risk birthmarks have complications or systemic associations that need identification and further management. Birthmarks are common, and in most cases parents can be reassured they are only of cosmetic significance and that the appearance will improve over time.
Topics: Cafe-au-Lait Spots; Female; Hemangioma; Humans; Infant; Infant, Newborn; Male; Nevus; Port-Wine Stain; Skin Neoplasms
PubMed: 22558616
DOI: No ID Found -
Ugeskrift For Laeger Sep 2018The classification of vascular anomalies has been revised, as physicians and researchers have recognised an increasing number of vascular anomalies. The International... (Review)
Review
The classification of vascular anomalies has been revised, as physicians and researchers have recognised an increasing number of vascular anomalies. The International Society for the Study of Vascular Anomalies presents a classification distinguishing between tumours and malformations. Over the years, an inaccurate application of the term haemangioma has been used, which has led to confusion among physicians. By using the classification and combining it with a thorough history and objective examination a classification of the most common vascular anomalies should be possible.
Topics: Hemangioma; Humans; Neoplasms, Vascular Tissue; Port-Wine Stain; Vascular Malformations
PubMed: 30187855
DOI: No ID Found -
American Family Physician Jan 2008Birthmarks in newborns are common sources of parental concern. Although most treatment recommendations are based on expert opinion, limited evidence exists to guide... (Review)
Review
Birthmarks in newborns are common sources of parental concern. Although most treatment recommendations are based on expert opinion, limited evidence exists to guide management of these conditions. Large congenital melanocytic nevi require evaluation for removal, whereas smaller nevi may be observed for malignant changes. With few exceptions, benign birthmarks (e.g., dermal melanosis, hemangioma of infancy, port-wine stain, nevus simplex) do not require treatment; however, effective cosmetic laser treatments exist. Supernumerary nipples are common and benign; they are occasionally mistaken for congenital melanocytic nevi. High- and intermediate-risk skin markers of spinal dysraphism (e.g., dermal sinuses, tails, atypical dimples, multiple lesions of any type) require evaluation with magnetic resonance imaging or ultrasonography. Family physicians should be familiar with various birthmarks and comfortable discussing disease prevention and cosmetic strategies.
Topics: Hemangioma; Humans; Infant, Newborn; Nevus; Prognosis; Skin Neoplasms
PubMed: 18236823
DOI: No ID Found