Introduction
Port-wine stain (PWS), or nevus flammeus, is the most prevalent type of congenital capillary malformation which appears most commonly on the face and neck, but it can be localized anywhere on the body. The name is related to its characteristic appearance that reminds of red wine spilled or splashed on the skin [1]. Disease occurs in about 0.3–0.9% of infants, equally in males and females [2]. Initially, a lesion occurs as a pink or red macule that grows relatively to the age, can form papules or nodules, as well as develop into hypertrophic PWS [2, 3]. The colour of the birthmarks which is induced by haemoglobin pigment might progressively darken to the deep reddish purple or even violaceous [4]. The lesions do not involute spontaneously during the life, which might cause significant psychosocial burden especially when it is located on visible parts of the body like the face or neck. Nevus flammeus might occur separately or be a part of clinical picture in some disorders such as Sturge-Weber syndrome (SWS) or Klippel-Trenaunay syndrome (KTS) [5–7]. Recently, somatic mosaic mutations in genes that control cell-cycle regulation, including GNAQ, GNA11, PiK3CA, and others that are also implicated in cell-cycle signalling, have been reported as the main cause of PWS. The detailed pathomechanism is still elusive [7].
The golden standard method in the treatment of the PWS is non-invasive pulsed dye laser [5, 8]. In this method laser beams aim light-absorbing targets in the lesion such as oxyhaemoglobin, deoxyhaemoglobin or methaemoglobin, heat them and damage with a minimal injury to the surrounding structures [9]. It is essential to start laser therapy from an early infancy to achieve better response to treatment [1]. In a significant number of patients, the effect of the therapy might not be satisfactory, so other options of treatment are needed [10].
The aim of the PWS therapy is not only to achieve a reduction of the symptoms, but also to provide patients with proper mental care and satisfying quality of life (QoL). It is especially crucial in patients with visible skin lesions which might cause considerable anxiety disorders of social rejection as well as fear of negative evaluation by the society [11]. Despite the fact that port-wine stains are considered a benign and asymptomatic disorder, it is reported that it can cause significant decrease in patients’ quality of life as well as stigmatization. In clinical practice there are special tools to measure psychosocial influence of dermatological diseases (DLQI, Skindex-29, PSQ, DCQ, HADS), which were applied in our study. Dermatology Life Quality Index (DLQI) and Skindex-29, which were previously used in other research concerning PWS, are the most common instruments to evaluate the impact of dermatological disorders on patients’ quality of life [12–14].
DLQI is a standardized scale that consists of 10 questions about patients’ everyday life in the previous week [12]. The number of received points represents the impact of skin disease on patients’ quality of life. Skindex-29 is a validated, skin specific instrument that combines 29 items divided into three different domains: symptoms (seven items), emotions (ten items), functioning (twelve items) [15–18] and 1 additional question about side-effects of the treatment that is not scored as part of the instrument. Scores are given on a 5-point scale and are later computed in order to transform results to the linear scale – from 0 to 100. The results reflect patients’ QoL in the previous 4 weeks. The Perceived Stigmatization Questionnaire (PSQ) is a useful tool to assess stigma of different diseases. It consists of 21 items, divided into three sections – eight of them pertain to “absence of healthy behaviour”, another eight concern “confusing/staring behaviour” and five refer to “hostile behaviour” [19, 20]. The Hospital Anxiety and Depression Scale (HADS) is a questionnaire comprising 14 questions used for the purpose of measuring anxiety and depression in patients with various disorders [21–24]. The results are taken separately for both disorders. The Dysmorphic Concern Questionnaire (DCQ) is a scale assessing body dysmorphic disorder (BDD), a condition characterized by negative perception of the body [11, 25, 26]. The DCQ contains seven items allowing participants to express their concern about their physical appearance relative to others. Interpretation of the results of the scales mentioned above and range of points in each of them vary between the scales (Table 1).
Table 1
Five scales used in our research
| Scale | Description | Minimum score [points] | Maximum score [points] | Interpretation |
|---|---|---|---|---|
| DLQI | Dermatology Life Quality Index | 0 | 30 | 0–1 – no effect 2–5 – small effect 6–10 – moderate effect 11–20 – very large effect 21–30 – extremely large effect |
| Skindex-29 | Skindex-29 | 0 | 100 | Higher number indicates lower QoL (Table 4) |
| PSQ | Perceived Stigmatization Questionnaire | 21 | 105 | Higher score means stronger sense of stigmatization behaviour towards the patient. |
| DCQ | Dysmorphic Concern Questionnaire | 0 | 21 | ≥ 9 – clinically significant dysmorphic concern |
| HADS | Hospital Anxiety and Depression Scale | 0 | 21 | 0–7 – normal 8–10 – borderline abnormal 11–21 – abnormal – anxiety or/and depression |
Prior studies on that topic have demonstrated impaired QoL in patients with PWS. HADS, DCQ and PSQ have not been commonly used to assess psychosocial aspects of PWS in other studies yet [2, 3]. The data about psychosociological influence of PWS are limited, so there is still a need for further investigation.
Aim
The aim of the study was to investigate psychosocial well-being of patients with vascular malformations as well as to raise the awareness of possible psychosocial problems in patients with port wine stains and the need of an interdisciplinary approach to patients with visible skin lesions.
Material and methods
The questionnaire used in this research was created by the authors. It was divided into four parts: 8 questions about demographic characteristics (sex, age, personal relationship status, place of residence, education and current employment), 6 questions concerning the characteristics of the patient’s disease and the phototype, 12 original questions of PDL treatment history (six items included in this part were ranged by four-point scale 1 – definitely yes, 2 – rather yes, 3 – rather no, 4 – definitely no). The last section was a combination of five standardized tests, namely DLQI, Skindex-29, PSQ, DCQ, HADS [11–14, 20, 21, 26–28]. The questionnaires were filled out by patients after various forms of PWS treatment including different amounts of pulsed dye laser sessions.
The study group consisted of 90 adult patients with a diagnosis of PWS who were previously hospitalized in the dermatological ward. Patients were reached through telephone calls and informed about the aims and methods of the study. Afterwards the survey was emailed to the patients, who gave consent to fill out the form, and sent back by via Google Forms. The exclusion criterion was the lack of consent from the patients. The data were collected and subjected to analysis to determine the intensity of the studied characteristics in the sample. Multivariate linear regression analysis was undertaken to identify significant predictors associated with change in DLQI.
Results
Out of 90 patients who received a questionnaire, 59 filled it out and sent back (response rate 65.6%). Most of participants were women (n = 51; 86.4%). The dominant skin phototype among the patients was phototype II in Fitzpatrick classification. At the time of the study the mean age of participants was 32.12 years (range: 18–59 years). Forty-three patients presented malformations in only one location on the body from which the face, the lower limb and the upper limb were the most common. Twelve patients (20.3%) had a positive family history of PWS (Table 2).
Table 2
Demographic data and lesions characteristics
DLQI
According to the results of DLQI questionnaire, quality of life was decreased in 52.5% of patients (n = 31). Out of the patients whose QoL was affected by this condition the majority received between 2 and 5 points. Those results are classified to a “small effect” category (Table 3). Mean DLQI score for all the patients was 4.0 ±5.3 points. Those values were increased separately in patients with lesions located on the face and in patients whose lesions were present in more than one location. According to median and mean DLQI scores in our study group, patients with PWS located on the face experienced worse QoL than the ones without any face lesions. Women received higher results than men which indicates lower QoL. Older patients’ QoL was generally more affected than in younger patients.
Table 3
Dermatology Life Quality Index scores
Skindex-29
The results of Skindex-29 in our study group imply that emotions subscale was the most significantly influenced according to the mean results (Table 4). The least affected subscale was symptoms. Due to the differences in the cut-off values of emotions and functioning subscales [27] we considered the distribution of patients’ scores in each of those subscales. In both subscales mentioned, the results were distributed similarly. The mean Skindex-29 total score being 22.9 ±17.1 points is an indication of a moderate negative influence of PWS on patients.
Table 4
Skindex-29 results
HADS
According to the results of HADS, anxiety (including abnormal and borderline abnormal scores) was affecting 45.8% of patients (n = 27) and it was more frequently observed than depression (including abnormal and borderline abnormal scores) which occurred in 22.0% of the participants (n = 13). One patient received scores classified as abnormal both of anxiety and depression simultaneously (Table 5).
Table 5
Hospital Anxiety and Depression Scale results and Perceived Stigmatization Questionnaire results
DCQ
55.9% of patients obtained a score equal to or greater than 9 in the Dysmorphic Concern Questionnaire (DCQ) (n = 33) with the highest score of 20 out of 21 points.
PSQ
The mean results and standard deviation of PSQ were similar in each subscale. The highest score was obtained in “confusing/staring behaviour” (4.5 points) and the lowest was the same in each subscale (1.0 point) (Table 5).
Multivariate linear regression
To test whether DLQI is influenced by other standardized predictors, a multivariate linear regression including DLQI as a response variable and Skindex-29, HADS, DCQ and PSQ as explanatory variables was employed. It was noted that mentioned factors have a significant effect on DLQI (F(4,54) = 17.56, p < 0.0001), with R2 = 0.533 suggesting that 53.3% of the variation is predicted by the factors listed. The predicted DLQI is equal to 0.20*SKINDEX-29 + 1.15*PSQ + 0.03*DCQ-0.03*HADS-2.96, per one unit increase in each factor. SKINDEX-29 was found to be the highest predictor of DLQI (p = 0.0002) while other factors (PSQ, DCQ, HADS) did not have a significant contribution to changes in DLQI. Detailed results are presented in Table 6.
Table 6
ANOVA table for multiple linear regression model and multiple linear regression model coefficients
Discussion
Patients’ QoL can be significantly influenced by PWS which is the topic of research by various scientists [2]. DLQI is one of the most commonly used tools in assessing patients’ QoL with many dermatological conditions including PWS [3, 29]. Factors like presence of face lesions and presence of lesions in more than one location seemed to be associated with lower QoL. Gender might also have an influence on that issue. In our study women seemed to have lower QoL than men which was also observed in studies by Wang et al. and Zhao et al. [3, 30]. The cause of that might be found in women’s concentration on physical appearance and greater aesthetic orientation [30]. In the research by Wanitphakdeedecha et al. those issues do not resolve with advancing age; on the contrary older patients in our study presented even lower quality of life than those earlier in life [2]. Causes of that phenomenon remain unknown. Our research and others seem to advocate the thesis that PWS lesions cause negative impact on patients’ QoL. Comparing PWS to other dermatological diseases and conditions, DLQI results in psoriasis, hidradenitis suppurativa and pemphigus [31] are higher than achieved in our study, which is in accordance with the current knowledge of the severity of those diseases. Results in patients with morphea [31, 32] were generally lower than in patients with PWS. Similar results were observed in patients with cutaneous T-cell lymphoma (CTCL), rosacea, alopecia, and vitiligo [33].
Patients’ QoL was also assessed using Skindex-29 scale. Results achieved in our study are similar to the ones presented by Hagen et al. and Akbar TM et al. [33, 34]. This scale is designed to assess complex look into patients’ perspective on their QoL by dividing their answers into three categories – emotions, symptoms, and functioning. High mean score for the emotion subscale might be deceptive and confusing because of the various distribution of points in different categories [27]. In both emotions and functioning subscales, patients’ results were distributed similarly. Both of those life aspects were similarly negatively influenced by PWS lesions in the presented group of patients. Symptoms subscale was the least influenced in patients with PWS, which remains in agreement with results achieved in other studies [33, 34].
HADS, DCQ and PSQ that were used in our research, are not commonly used tools in studies regarding patients with PWS. According to the results of the HADS, 45.8% of patients were affected by anxiety and 22% by depression. Only 1 patient was suffering from both conditions, which suggests that in the majority of cases co-occurrence of anxiety and depression was not observed. Despite the fact that our research was focused on the impact of PWS on patients’ life, it should be emphasized that there might have been other factors affecting patients’ psychosocial state which were not included in our study. Results of the DCQ scale suggest that in more than half of the patients, their perception of their bodies was disturbed due to the skin condition. Scores achieved in the PSQ indicate that patients with PWS deal with stigmatization that patients notice in all types of behaviours mentioned in the scale. Assessment of perceived stigmatization might be used as a tool to predict future issues with psychosocial adjustment such as internalizing behaviour [35].
Achieved results are alarming and are a proof that patients’ mental health in different areas of life is impaired due to the presence of visible skin lesions. Our study provides an important perspective on the topic but there are several limitations that need to be acknowledged. The study group consisted of a relatively small number of patients. The majority of patients were women which might not be an appropriate portrayal of the population of patients with PWS. Phototype II in Fitzpatrick’s classification was the dominant type in our study as it is the most common among Polish citizens. There were no patients with phototypes V and VI, which gives us no insight on QoL of patients of colour. Additionally, only adults who were able to fill the online survey and were interested in taking part in our research were included, which might not be representative of the whole population with PWS and might result in selection bias.
Conclusions
PWS is a dermatological condition that can affect psychosocial well-being of the patients including their quality of life, self-perception of the body or social stigmatization. Use of standardized questionnaires plays an important role in an appropriate assessment of patients’ feelings and well-being. Patients with PWS should be provided with psychological, or if necessary psychiatric, care.
HADS copyright © R.P. Snaith and A.S. Zigmond, 1983, 1992, 1994. Record form items originally published in Acta Psychiatrica Scandinavica 67, 361-70, copyright © Munksgaard International Publishers Ltd, Copenhagen, 1983. Published by GL Assessment Limited, 1st Floor Vantage London, Great West Road, London TW8 9AG, UK. All rights reserved. GL Assessment is part of the GL Education Group.
Skindex29 © MM Chren, 1996.
PSQ Copyright © 2006, American Psychological Association. Reproduced with permission [19].
