Treatment Update of Port-Wine Stain: A Narrative Review.

BACKGROUND: Port-wine stain (PWS) is a congenital vascular malformation affecting 0.3–0.5% of normal population. These characteristic lesions arise due to the interplay of vascular, neural, and genetic factors. Treatment options include lasers, cosmetic tattooing, electrotherapy, cryosurgery, derma-abrasion, and skin grafting; however, none of these treatment alternatives appears to be satisfactory and is unable to provide consistent, satisfactory responses or even complete cures. Currently, laser is the treatment of choice, as it is comparatively safe and more effective than other procedures. The most commonly used modality is pulsed dye laser (PDL). The literature research includes peer-reviewed articles (clinical trials or scientific reviews). Studies were identified by searching electronic databases (MEDLINE and PubMed) to January 2020 and reference lists of respective articles. Only articles published in English language were included. J Drugs Dermatol. 20(5): doi:10.36849/JDD.5005.

Quality of life in adults with facial port-wine stains.

BACKGROUND: Facial port-wine stains (PWS) are considered by some an aesthetic skin problem, yet impact on quality of life (QoL) has not been objectively documented. OBJECTIVE: We sought to (1) characterize the effect of PWS on QoL in adults, (2) to identify the clinical and demographic factors that affect QoL, and (3) to compare our results with QoL studies in other skin conditions. METHODS: In total, 244 adults with facial PWS completed an online QoL survey, which included the Skindex-29 instrument. RESULTS: QoL in adults with facial PWS was diminished, especially from an emotional perspective. Variables associated with reduced QoL in all Skindex-29 subdomains included comorbid depression, limited facial mobility, and presence of other skin conditions. Persons with hypertrophy had more emotional and symptomatic impairment. The composite dermatologic-specific QoL scores were similar to those of cutaneous T-cell lymphoma, rosacea, alopecia, and vitiligo. LIMITATIONS: Selection bias was a potential limitation, as participants were primarily recruited from patient support groups. CONCLUSION: Our analysis demonstrates that the presence of a facial PWS has a significant negative impact on QoL. Dermatologists caring for patients with PWS should inquire about QoL, provide appropriate support and resources, and consider QoL when discussing treatment options and obtaining authorization for these procedures.

Stigmatization predicts psychological adjustment and quality of life in children and adolescents with a facial difference.

OBJECTIVES: This cross-sectional study assessed psychological adjustment and health-related quality of life (HRQOL) in children and adolescents with congenital or acquired facial differences and identified potential predictors of adjustment. METHODS: Data were obtained from 88 children, ages 9 months to 16 years, by means of parent questionnaires (n = 86) and standardized interviews with children ≥7 years old (n = 31). Evaluation measures included the Child Behavior Checklist (CBCL), KIDSCREEN-27, TNO-AZL Preschool Quality of Life Questionnaire (TAPQOL), and Perceived Stigmatization Questionnaire. RESULTS: Psychological adjustment, as measured by the CBCL, was within norms. Parent-reported HRQOL was good in preschool children. Parent- and self-reported HRQOL of participants 7-16 years old was impaired in several dimensions, including psychological well-being. Psychological adjustment (especially internalizing behavior problems) and HRQOL were predicted primarily by perceived stigmatization. CONCLUSIONS: Identification of stigma experiences and appropriate support may be crucial to enhancing psychological adjustment and quality of life in children with facial disfigurement.

Treating pediatric port-wine stains in aesthetics.

A port-wine stain (PWS) is a vascular birthmark present in 0.3% to 0.5% of newborns. If untreated, this erythematous patch will grow proportionally with the child to thicken and darken with age. PWSs have implications for the child's quality of life for many years, with cosmetic, medical, and psychosocial disability. Controversy exists in many aspects surrounding laser treatment of these birthmarks in the pediatric population. We have reviewed the clinical features as well as the historic and current laser treatment of PWS. We have also examined the current hot topics of debate surrounding the treatment of PWS in the pediatric population. These controversies include the patient age of treatment initiation, the long-term psychologic impact, the use of general anesthesia, the application of eye shields, and alternative treatments for recalcitrant PWS. We have concluded with a discussion on the future directions of management and treatment.

Consensus Statement for the Management and Treatment of Port-Wine Birthmarks in Sturge-Weber Syndrome.

Importance: Sturge-Weber syndrome (SWS) is a neurocutaneous syndrome involving the skin, brain, and eyes. Consensus recommendations for management are lacking. Objective: To consolidate the current literature with expert opinion to make recommendations that will guide treatment and referral for patients with port-wine birthmarks (PWBs). Evidence Review: In this consensus statement, 12 nationally peer-recognized experts in dermatology with experience treating patients with SWS were assembled. Key topics and questions were formulated for each group and included risk stratification, optimum treatment strategies, and recommendations regarding light-based therapies. A systematic PubMed search was performed of English-language articles published between December 1, 2008, and December 1, 2018, as well as other pertinent studies identified by the expert panel. Clinical practice guidelines were recommended. Findings: Treatment of PWBs is indicated to minimize the psychosocial impact and diminish nodularity and potentially tissue hypertrophy. Better outcomes may be attained if treatments are started at an earlier age. In the US, pulsed dye laser is the standard for all PWBs regardless of the lesion size, location, or color. When performed by experienced physicians, laser treatment can be safe for patients of all ages. The choice of using general anesthesia in young patients is a complex decision that must be considered on a case-by-case basis. Conclusions and Relevance: These recommendations are intended to help guide clinical practice and decision-making for patients with SWS and those with isolated PWBs and may improve patient outcomes.

Pulsed dye laser vs. intense pulsed light for port-wine stains: a randomized side-by-side trial with blinded response evaluation.

BACKGROUND: Pulsed dye lasers (PDLs) are considered the treatment of choice for port-wine stains (PWS). Studies have suggested broadband intense pulsed light (IPL) to be efficient as well. So far, no studies have directly compared the PDL with IPL in a randomized clinical trial. OBJECTIVES: To compare efficacy and adverse events of PDL and IPL in an intraindividual randomized clinical trial. METHODS: Twenty patients with PWS (face, trunk, extremities; pink, red and purple colours; skin types I-III) received one side-by-side treatment with PDL (V-beam Perfecta, 595 nm, 0.45-1.5 ms; Candela Laser Corporation, Wayland, MA, U.S.A.) and IPL (StarLux, Lux G prototype handpiece, 500-670 and 870-1400 nm, 5-10 ms; Palomar Medical Technologies, Burlington, MA, U.S.A.). Settings depended on the preoperative lesional colour. Treatment outcome was evaluated by blinded, clinical evaluations and by skin reflectance measurements. RESULTS: Both PDL and IPL lightened PWS. Median clinical improvements were significantly better for PDL (65%) than IPL (30%) (P = 0.0004). A higher proportion of patients obtained good or excellent clearance rates with the PDL (75%) compared with IPL (30%) (P = 0.0104). Skin reflectance also documented better results after PDL (33% lightening) than IPL (12% lightening) (P = 0.002). Eighteen of 20 patients preferred to receive continued treatments with PDL (P = 0.0004). No adverse events were observed with PDL or IPL. CONCLUSIONS: Both the specific PDL and IPL types of equipment used in this study lightened PWS and both were safe with no adverse events. However, the PDL conveyed the advantages of better efficacy and higher patient preference.

Autism with facial port-wine stain: a new syndrome?

The hallmark of Sturge-Weber syndrome is leptomeningeal angiomatosis. Over 15 years, four children were identified (2 boys, age 2.9-6 years) with unilateral facial port-wine stain, referred for presumable Sturge-Weber syndrome but who were also autistic. Computed tomography and magnetic resonance imaging scans failed to show evidence of leptomeningeal angioma in all four children. Three of the children had a history of seizures. Detailed neuropsychologic testing of three children revealed a similar presentation, characterized by developmental disturbance, particularly involving delayed onset of language, and early-emerging social atypicality. Positron emission tomography scanning of cerebral glucose metabolism revealed hypometabolism in the bilateral medial temporal regions, anterior cingulate gyrus, frontal cortex, right temporal cortex, and cerebellum. The pattern of glucose hypometabolism differed from that of 12 children with infantile autism (age 2.7-7.9 years) who had mild left medial temporal but more severe right temporal cortical hypometabolism and showed a reversal of normal frontotemporal asymmetry of glucose metabolism. Unilateral facial port-wine stain and autism with no intracranial angioma on conventional imaging may represent a rare clinical entity distinct from both infantile autism and previously described variants of Sturge-Weber syndrome.

Analysis of quality of life and influencing factors in 197 Chinese patients with port-wine stains.

Port-wine stains (PWS) are congenital capillary malformations, usually occurring on the face, neck, and other exposed parts of the skin, that have serious psychological and social impact on the patient. Most researchers focus on the treatment of PWS, but the quality of life (QoL) of PWS patients is seldom researched. The objective of this study is to evaluate the QoL of patients with PWS on exposed parts and explore the factors influencing the QoL of PWS patients. The QoL of 197 cases with PWS on exposed parts were prospectively studied using the Dermatology Life Quality Index questionnaire (DLQI), and the factors influencing the patients' QoL were analyzed by single-factor analysis and multiple-factor logistic regression analysis. The reliability and validity of the QoL of PWS patients were then assessed by DLQI. A total of 197 valid questionnaires were collected. The DLQI scores in PWS cases ranged from 2 to 16, with 2 to 5 in 52.29% (103/197), 6 to 10 in 42.13% (83/197), and 11 to 20 in 5.58% (11/197). The main score elements of the DLQI focused on symptoms and feelings, daily activities, and social entertainment. Single-factor analysis and multiple-factor logistic regression analysis showed that the main influencing factors were female sex, skin hypertrophy, and lesion area >30 cm. The inter-item correlation averaged 47.46% and the Cronbach α was 0.740, indicating high internal consistency. Correlation of the 6 dimensions of the DLQI questionnaires with the total scores showed that the Spearman correlation coefficient r ranged from 0.550 to 0.782 (P < .001), with symptoms and feelings having a correlation coefficient of 0.782 and a high correlation with total scores. This study shows that PWS has mild to moderate influence on the QoL of most patients, mainly on daily activities, social entertainment, and feelings.

Characterization of portwine stain disfigurement.

Portwine stain disfigurement is caused by several factors. To what extent and in which proportion these factors influence the overall perceived disfigurement is incompletely understood. In this study, the contribution of seven portwine stain characteristics to overall portwine stain disfigurement was assessed. Color slides were taken from 90 patients with untreated portwine stains in the head/neck area. From these slides, overall portwine stain disfigurement was judged by a panel of 16 lay persons. The reliability of the average ratings of this panel was established with weighted kappa analysis (kappa = 0.51) and by calculating the Cronbach alpha coefficient (0.99). Using a previously tested multi-item questionnaire, the following portwine stain characteristics were rated quantitatively by a panel of five professionals: color, patchiness, boundary, size, shape, surface structure, and hypertrophy of the underlying tissue. By means of multiple linear regression analysis, the ratings for overall portwine stain disfigurement (panel of lay persons) were compared with the ratings for the individual portwine stain characteristics (panel of professionals). From the results of this analysis, the percentual contribution of each of the characteristics to overall portwine stain disfigurement was calculated. Size turned out to be the most important portwine stain characteristic, being responsible for almost half of the overall disfigurement. Color and boundary are the next two most important characteristics, contributing 18.7 and 12.4 percent, respectively. The other four characteristics together account for 10 percent. In our model, 13 percent of overall portwine stain disfigurement remains unexplained. We expect patient features to account for this. We feel that these results may have consequences for laser treatment of portwine stains. Reducing the size and fading out the boundary of the stain probably reduce overall portwine stain disfigurement more effectively than primarily trying to lighten the often persistent center of the stain.

Facial disfigurement: a self-psychological perspective on the "hide-and-seek" fantasy of an avoidant personality.

The author presents the self-psychological treatment of a patient who was born with a facial disfigurement. At the onset of treatment, the patient met the diagnostic criteria for avoidant personality disorder. The disfigurement predisposed the patient to perceive herself as defective and flawed. This sense of being flawed was exacerbated by early losses and selfobject failures. The formulation presented focuses on the patient's "hide-and-seek" fantasy, which expresses both archaic grandiosity and omnipotence. The patient's emotional hiding and seeking is an apt description of her avoidant behavior, in general, and the transference, more specifically.

Undisclosed port-wine stain--anesthetic implications and psychosocial considerations: a case report.

Port-wine stains (nevus flammeus, port-wine nevus) are congenital vascular lesions that have psychological and physiological implications for patient care. The location and size of these lesions can lead to complications related to anesthetic management. Facial lesions have the most profound psychological effect on the patient with respect to behavior alterations and reluctance to disclose the lesion's presence. Covering makeup has become increasingly effective not only in concealing the lesion, but also in being nearly undetectable during routine examination. Issues such as agent choice, surgical position, frequency of positioning evaluation, and choice of intravenous fluid should be considered when caring for a patient with a port-wine stain. In this case study the authors describe the anesthesia implications and outcome in a patient who refused to disclose her port-wine lesion in the preoperative interview and who ignored preoperative instructions to remove all makeup before presenting for surgery.

Port wine stains: laser treatment and nursing management.

In 1960, an American physicist Theodore Maiman observed the first laser in action. Since then laser technology has progressed at an extraordinary rate. This article shows how one type of laser has touched the lives of thousands of people. The Candela tunable dye laser has given hope to people with port wine stain birthmarks. It also shows the duties and responsibilities of the nurse working with lasers and the possibility of the extended role of the nurse in the field of skin laser therapy.

Tratamiento con Pulsed Dye Laser de mancha de vino oporto: seguimiento a 10 años / Treatment with Pulsed Dye Laser of wine stain Porto: 10-year follow-up

Las manchas de vino de oporto son un tipo de malformación capilar que afecta del 0,3 al 0,5% de la población. Están presentes desde el nacimiento como máculas o placas eritematosas en la piel o mucosas. Sin tratamiento, las lesiones tienden a oscurecerse con la edad, tornándose rojizas o color púrpura, y pueden desarrollar engrosamiento nodular o un granuloma piógeno asociado. Los tratamientos con láser proporcionan mejoría mediante la destrucción selectiva de la vasculatura. Se han utilizado una variedad de láseres vasculares selectivos, pero el tratamiento de primera elección es el láser de colorante pulsado. Los mejores resultados se obtienen cuando el tratamiento es instaurado tempranamente. (AU)

Port wine stains are a type of vascular malformation that affects 0.3% to 0.5% of the population. They are present from birth as erythematous macules or plaques on the skin or mucous membranes. Without treatment, these lesions tend to darken with age, becoming reddish or purple and may develop nodular thickening or an associated pyogenic granuloma. Laser treatments might provide an improvement by selective destruction of the vasculature. A variety of selective vascular lásers may be employed, with the pulsed dye laser as the gold standard treatment. Better results are obtained when the treatment is established early. (AU)

Willingness to pay and time trade-off: useful utility indicators for the assessment of quality of life and patient satisfaction in patients with port wine stains.

BACKGROUND: For cost utility analyses in health economic research it is necessary to assess quality of life for content validation. Previously, both quality of life questionnaires and utility indicators such as willingness to pay and time trade-off have been used successfully in patients with chronic skin diseases, such as psoriasis vulgaris or atopic eczema. OBJECTIVE: For the first time to assess willingness to pay and time trade-off in patients with port wine stains, and to study possible correlations between these indicators, patient income, quality of life questionnaires and subjective or objective outcome measures. METHODS: The results of a survey on 36 patients with facial lesions who had completed laser treatment were used for assessments. The 'Chronic Skin Disease Questionnaire' (CSDQ) and the 'Short Form-36 Health Survey' (SF-36) were used as quality of life questionnaires. Special questions were used as a basis for calculating the utility indicators, willingness to pay and time trade-off. RESULTS: Completed questionnaires were sent back by 25 patients (69%). Willingness to pay and time trade-off were used as instruments for the assessment of quality of life [questions answered by 23 (92% of respondents)]; patients were willing to pay 11.8% of their monthly income and would offer a mean value of 1.2 h per day for an imaginary therapy leading to complete cure of the skin problem. Statistical correlation analyses were not performed because this pilot study included only a small number of patients. Frequency distributions show no apparent correlation between willingness to pay and patients' profession (used as a surrogate for income). Possible correlations were seen between willingness to pay, time trade-off, the scales 'anxiety/avoidance' and 'helplessness' of the CSDQ as well as 'social function' of SF-36 and subjective or objective efficacy. Twenty-nine patients (80%) were willing to pay for the treatment, a surrogate measure for patients' satisfaction with treatment modality. Overall, patients would pay an average of 16.0 euros per single treatment and 192.0 euros for the whole course of treatments. In contrast with patients who evaluated treatment modality as excellent or very good (willingness to pay for single treatment, euro 22.0; for whole treatment, 270.0 euros), patients who judged treatment good (8.0 euros, 145.0 euros), moderate or bad (12.0 euros, 146 .0 euros) were willing to pay markedly less. CONCLUSIONS: (i) The questions for willingness to pay and time trade-off were understood by most patients and produced meaningful answers. (ii) There were apparent correlations between the utility indicators, subjective or objective outcome and the scales of the quality of life questionnaires. (iii) The results show that these indicators have a high potential for use in this dermatological condition, especially as a basis for health economic evaluations.

[Coping with illness and quality of life of patients with port-wine stains treated with laser therapy]. / Krankheitsbewältigung und Lebensqualität bei Patienten mit Feuermalen unter Laser-Therapie.

Port wine stains (PWS) can lead to considerable emotional distress. The present study evaluated a) the coping with illness, the quality of life and the body image of patients with PWS and b) the effects of dye laser treatment on psychosocial parameters. Seventy PWS patients undergoing treatment with the flashlamp-pumped pulsed dye laser (FPDL) were assessed with questionnaires regarding coping with skin disease, quality of life and body image. Major clinical criterium was the lightening of PWS under treatment. PWS patients showed significant social phobia and avoidance similar to patient suffering from chronic skin diseases. The anxiety correlated with size and darkness of the PWS. In terms of helplessness and depressive mood, PWS patients were less affected than the comparison group. Also, PWS patients had reductions in quality of life and in body image. The coping strategies had a differential effect on the body image. Since there is a correlation between lightening of the PWS and reduction of emotional distress, FDPL therapy can be considered an effective treatment of PWS also in psychosocial terms.

Patients with port-wine stains and their psychosocial reactions after photothermolytic treatment.

BACKGROUND: Since the pulsed dye laser (PDL) was introduced in the late 1980s the question has been raised whether one should treat congenital capillary malformations, commonly called port-wine stains (PWSs), early or not. OBJECTIVE: There are pros and cons, but studies have mainly emphasized the clinical treatment results. The psychosocial importance of an early treatment needs also to be clarified. METHODS: This retrospective study includes 163 patients with PWS treated with the PDL until there was either total clearing or until there was no further improvement. When a period from a few months to 8 years had elapsed after the treatments, the patients were given a questionnaire, which had been developed in cooperation with a psychiatrist, in order to evaluate the psychosocial implications of the PWS and the consequences of treatment. RESULTS: . Eighty percent of the patients more than 7 years of age had not fully accepted their PWS with increasing age and 80% thought that their life would change for the better if their PWS could be eliminated. Eighty-five percent thought their PWS influenced their life in some negative way. Forty-five percent of the patients considered themselves to have a lower self-esteem than their own age group. The majority of the patients considered themselves negatively influenced by their PWS. Most psychosocial parameters, such as self-esteem, contact with the opposite sex, social relationships, school contacts, the need to cover their PWS with make-up or clothes, and meeting new people, improved significantly after PDL treatment. Older patients had higher negative scores than the younger ones, indicating the advantage of early treatment. Patients who were treated earlier with other methods producing less favorable results scored unfavorably compared to previously untreated patients. CONCLUSION: Patients with PWS treated at our clinic consider their psychosocial status to be improved after treatment. An early treatment seems to be favorable for these patients.