EADV Task Force Pruritus White Paper on chronic pruritus and chronic prurigo: Current challenges and future solutions.

Chronic pruritus (CP) is frequent in general medicine and the most common complaint in general dermatology. The prevalence of CP is expected to rise in the future due to the ageing population. The clinical presentation, underlying aetiology and treatment strategy of CP are heterogeneous. Also, individual treatment aims and physical, psychic and economic burdens of patients might vary. Chronic prurigo (CPG) is the most severe disease in the chronic pruritus spectrum, being associated with long-standing scratch-induced skin lesions and a therapy refractory itch-scratch-cycle. It is thus important to raise disease awareness for CP and CPG in the general public and among decision-makers in the health system. Further, there is a need to support a rational clinical framework to optimize both diagnostics and therapeutics. Currently, there is still a shortcoming regarding approved therapies and understanding CP/CPG as severe medical conditions. Therefore, the EADV Task Force Pruritus decided to publish this white paper based on several consensus meetings. The group consented on the following goals: (a) ensure that CP is recognized as a serious condition, (b) increase public awareness and understanding of CP and CPG as chronic and burdensome diseases that can greatly affect a person's quality of life, (c) clarify that in most cases CP and CPG are non-communicable and not caused by a psychiatric disease, (d) improve the support and treatment given to patients with CP to help them manage their disease and (e) publicize existing therapies including current guidelines. We aim to point to necessary improvements in access and quality of care directed to decision-makers in health policy, among payers and administrations as well as in practical care.

Pruritus Intensity Scales across Europe: a prospective validation study.

BACKGROUND: Chronic pruritus (CP) is a subjective symptom, and it is necessary to assess its intensity with validated patient-reported outcome tools in order to allow determination of the treatment course. OBJECTIVES: So far, the itch intensity scales were validated in small cohorts and in single languages. Here, we report the validation of the numerical rating scale, the verbal rating scale and the visual analogue scale for the worst and average pruritus intensity in the last 24h in several languages across Europe and across different pruritic dermatoses. METHODS: After professional translation, the intensity scales were digitized for use as a tablet computer application. Validation was performed in clinics for Dermatology in Austria, France, Germany, Italy, Poland, Russia, Spain, Switzerland and Turkey. RESULTS: A total of 547 patients with contact dermatitis, chronic nodular prurigo, psoriasis vulgaris, lichen planus or cutaneous T-cell lymphoma were included. The intensity scales showed a high level of reproducibility and inter-correlations with each other. The correlation with the Dermatology Life Quality Index was weak to strong in nearly all countries and dermatoses with the exception of France and patients with chronic nodular prurigo, for which no statistically significant correlations were found. CONCLUSIONS: The numerical rating scale, the verbal rating scale und the visual analogue scales are valid instruments with good reproducibility and internal consistency in German (Germany, Austria, Switzerland), French, Italian, Polish, Russian, Spanish and Turkish for different pruritic dermatoses. VAS worst was the best reproducible and consistent measuring instrument in all countries.

Google search trends for itch in Europe: a retrospective longitudinal study.

BACKGROUND: Itch is a common symptom in the general population. Affected individuals often do not seek medical consultation and rely on Internet searches to obtain information regarding their itch. OBJECTIVES: The aim of this study was to attain insights into common concerns of the general population regarding itch can by analysing itch-related Internet search behaviour. METHODS: Google AdWords Keyword Planner was used to assess search volumes for itch-related terms in 15 European countries between September 2014 and August 2018. All identified keywords were qualitatively categorized. Itch-related terms were descriptively analysed and are shown as number of searches/100 000 inhabitants. RESULTS: The search volume for the keyword 'itch' per 100 000 inhabitants was highest in Northern Europe, followed by Eastern, Central and Southern Europe. In 4/15 countries, itch was searched for more often in the autumn/winter months compared to in the spring/summer months. Most itch-related terms were related to dermatological conditions such as inflammatory skin diseases (e.g. psoriasis, atopic dermatitis), allergic or immunologic conditions (e.g. urticaria), and infectious diseases or infestations (e.g. scabies). In terms of body location, genitoanal itch dominated the searches. Symptoms and signs related to itch, possible non-dermatological aetiologies, and treatment options were also among the most searched terms. CONCLUSIONS: These analyses provided for the first time insights into the search behaviour patterns related to itch across Europe. People from Northern and Eastern Europe are more likely to seek online information regarding itch. Causes for the itch, especially dermatological conditions, and genitoanal itch are the most important concerns for Internet users. This unconventional and inexpensive method identifies medical needs of people beyond the medical setting, including people who do not seek medical consultation. Accordingly, the data could be used to guide public health interventions and manage respective inhabitants' medical needs.

Chronic nodular prurigo: clinical profile and burden. A European cross-sectional study.

BACKGROUND: Chronic nodular prurigo (CNPG) is a condition characterized by chronic itch, a prolonged scratching behaviour and the presence of pruriginous nodules. A comprehensive understanding of this condition, especially regarding its clinical characteristics and impact on quality of life is still lacking. OBJECTIVES: Aim of this pan-European multicentre cross-sectional study was to establish the clinical profile of CNPG, including its associated burden. METHODS: Fifteen centres from 12 European countries recruited CNPG patients presenting at the centre or using the centres' own databases. Patients were asked to complete a questionnaire in paper or electronic format. Demography, current co-morbidities, underlying disease, itch intensity, additional sensory symptoms, quality of life, highest burden and emotional experience of itch were assessed. RESULTS: A total of 509 patients (210 male, median age: 64 years [52; 72]) were enrolled. Of these, 406 reported itch and CNPG lesions in the previous 7 days and qualified to complete the whole questionnaire. We recorded moderate to severe worst itch intensity scores in the previous 24 h. Scores were higher in patients with lower educational levels and those coming from Eastern or Southern Europe. Most patients experience itch often or always (71%) and report that their everyday life is negatively affected (53%). Itch intensity was considered to be the most burdensome aspect of the disease by 49% of the patients, followed by the visibility of skin lesions (21%) and bleeding of lesions (21%). The majority of patients was unaware of an underlying condition contributing to CNPG (64%), while psychiatric diseases were the conditions most often mentioned in association with CNPG (19%). CONCLUSIONS: This multicentre cross-sectional study shows that itch is the dominant symptom in CNPG and reveals that the profile of the disease is similar throughout Europe.

Cross-European validation of the ItchyQoL in pruritic dermatoses.

BACKGROUND: Chronic pruritus (CP) is a frequently occurring symptom in inflammatory dermatoses, causing a high burden and limitations to health-related quality of life (HRQoL). OBJECTIVE: The ItchyQoL was developed to assess the impairment to HRQoL in patients with CP. However, it has only been validated in English and German. Here, we report the validation in several languages across Europe. METHODS: After professional translation, the versions of ItchyQoL were digitized for use as a tablet application. Validation was performed in clinics for dermatology in Austria, France, Germany, Italy, Poland, Russia, Spain, Switzerland and Turkey. RESULTS: Five hundred and thirty-five patients with either contact dermatitis, chronic prurigo - nodular type, psoriasis vulgaris, lichen planus or mycosis fungoides/Sézary syndrome and with CP ≥ 3 on the numerical rating scale were included. ItchyQoL showed a high level of consistency (Cronbach's-α, all: 0.95) and test-retest reliability (intraclass correlation: 0.91). It strongly correlated with the Dermatology Life Quality Index (r = 0.72, P < 0.001) and moderately correlated with itch intensity scales in the study population (visual analogue scale r = 0.46; numerical rating scale r = 0.51; verbal rating scale r = 0.51, for all: P < 0.001). CONCLUSION: ItchyQoL is now also validated in French, Italian, Polish, Russian, Spanish and Turkish and can be used in clinical trials in countries speaking these languages.

European academy of dermatology and venereology European prurigo project: expert consensus on the definition, classification and terminology of chronic prurigo.

BACKGROUND: The term prurigo has been used for many decades in dermatology without clear definition, and currently used terminology of prurigo is inconsistent and confusing. Especially, itch-related prurigo remains unexplored regarding the epidemiology, clinical profile, natural course, underlying causes, available treatments and economic burden, although burdensome and difficult to treat. OBJECTIVE: To address these issues, the multicentre European Prurigo Project (EPP) was designed to increase knowledge on chronic prurigo (CPG). In the first step, European experts of the EADV Task Force Pruritus (TFP) aimed to achieve a consensus on the definition, classification and terminology of CPG. Additionally, procedures of the cross-sectional EPP were discussed and agreed upon. METHODS: Discussions and surveys between members of the TFP served as basis for a consensus conference. Using the Delphi method, consensus was defined as an agreement ≥75% among the present members. RESULTS: Twenty-four members of the TFP participated in the consensus conference. Experts consented that CPG should be used as an umbrella term for the range of clinical manifestations (e.g. papular, nodular, plaque or umbilicated types). CPG is considered a distinct disease defined by the presence of chronic pruritus for ≥6 weeks, history and/or signs of repeated scratching and multiple localized/generalized pruriginous skin lesions (whitish or pink papules, nodules and/or plaques). CPG occurs due to a neuronal sensitization to itch and the development of an itch-scratch cycle. CONCLUSION: This new definition and terminology of CPG should be implemented in dermatology to harmonize communication in the clinical routine, clinical trials and scientific literature. Acute/subacute forms of prurigo are separated entities, which need to be differentiated from CPG and will be discussed in a next step. In the near future, the cross-sectional EPP will provide relevant clinical data on various aspects of CPG leading to new directions in the scientific investigation of CGP.

European EADV network on assessment of severity and burden of Pruritus (PruNet): first meeting on outcome tools.

BACKGROUND: Chronic pruritus is a frequently occurring symptom of various dermatoses that causes a high burden and impaired quality of life. An effective anti pruritic therapy is important for the patient, but its effectiveness is difficult to evaluate. Diverse methods and interpretations of pruritic metrics are utilized in clinical trials and the daily clinical practice in different countries, resulting in difficulties comparing collected data. METHODS: We founded a European Network on Assessment of Severity and Burden of Pruritus (PruNet) that is supported by the EADV. PruNet consists of 28 experts from 15 EU countries (21 dermatologists, 5 medical informaticists, 2 psychologists) and aims to unify the assessment of itch in routine dermatological care. Following a preliminary survey, a consensus conference was held in order to agree upon the prioritization of patient-reported outcome tools. RESULTS: Through utilizing the Delphi method, it was agreed that tools for measuring itch intensity (ex. the visual analogue scale) and quality of life (ex. ItchyQoL) are of primary importance and should urgently be foremost validated. CONCLUSION: The validation and harmonization of standards are needed for the improvement of quality care for patients suffering from pruritic dermatoses. This summer, the first validation studies in several EADV member countries already began.

Social problem-solving, perceived stress, negative life events, depression and life satisfaction in psoriasis.

BACKGROUND: Psoriasis is a chronic dermatosis which may cause significant impairment of the patient's quality of life. OBJECTIVE: The purpose of this study was to investigate the social problem-solving skills, perceived stress, negative life events, depression and life satisfaction in psoriasis patients. METHODS: Data were gathered by means of questionnaires and clinical evaluations from 51 psoriatic patients and 51 matched healthy controls. RESULTS: Average disease duration was 16.47 years and average Psoriasis Area and Severity Index score was 3.67. Compared with the controls, the patients displayed lower social problem-solving skills. They displayed higher negative problem orientation and impulsive-careless problem-solving style scores than the controls. Patients tended also to show more avoidant problem-solving style and lower life satisfaction than controls. There was no difference between psoriatic patients and controls in terms of depression, perceived stress and negative life events. Higher social problem-solving skills were associated with lower depression, perceived stress and fewer numbers of negative life events but higher level of life satisfaction. LIMITATIONS: The patient group largely included mild and moderate psoriatic cases. CONCLUSION: The findings of the study suggest that problem-solving training or therapy may be a suitable option for alleviating levels of psychological distress in patients suffering from psoriasis.

Resistant oral mucosal lesions in pemphigus vulgaris responsive to double filtration plasmapheresis: First case report from Turkey.

BACKGROUND: Adjuvant therapeutic methods are employed when pemphigus vulgaris (PV) fails to be controlled by conventional corticosteroid treatment. OBJECTIVE: The efficacy of double filtration plasmapheresis (DFPP) was investigated in a PV patient with severe, refractory mucosal disease. METHODS: A total of 3 DFPP cycles, each cycle consisting of 5 double filtration sessions conducted on alternate days was completed. RESULTS: DFPP provided immediate clinical relief of symptoms as well as a significant decrease in anti-desmoglein antibody levels and allowed for a much lower corticosteroid dose. CONCLUSION: DFPP was an effective and safe adjuvant therapy in our patient with PV and it offers a valid treatment option in PV patients with recalcitrant disease.

Adiponectin levels, insulin resistance and their relationship with serum levels of inflammatory cytokines in patients with Behçet's disease.

AIM: Increased frequency of cardiovascular disease and its possible relations with insulin resistance have been reported in patients with inflammatory diseases. The aim of our study was to investigate insulin resistance and serum adiponectin levels as cardiovascular risk markers in patients with Behçet's disease. METHOD: Study population consisted of 40 patients with Behçet's disease (BD) and a control group composed of age, gender, body mass index-matched 46 healthy individuals. All patients were examined for signs of Behçet's disease. Body mass index, waist and hip circumference were measured. Insulin resistance was evaluated using the homeostasis model assessment-insulin resistance method. Erythrocyte sedimentation rate (ESR), lipid profile, high sensitive CRP (hsCRP), adiponectin, TNF-α, IL-6 and IL-8 levels were measured. RESULTS: Erythrocyte sedimentation rate, serum hsCRP and IL-6 levels were significantly higher in patients with BD than those in the controls (P=0.001, P=0.001, P=0.001, respectively). Fasting plasma glucose, insulin levels and lipid profile were not different between the two groups. Insulin resistance and decreased levels of the serum adiponectin were not detected in the patients. There was no relationship between insulin resistance, adiponectin levels and inflammatory markers. Active and inactive patients did not differ in respect of any parameters. CONCLUSION: Being a systemic vasculitis, BD may cause cardiovascular involvement. In this study, dyslipidemia, insulin resistance and low adiponectin levels were not detected among our patients with Behçet's disease. Our results suggest that there exists no increased risk for atherosclerotic cardiovascular disease associated with adiponectin levels and insulin resistance in patients with Behçet's disease.

Acne: prevalence, perceptions and effects on psychological health among adolescents in Aydin, Turkey.

BACKGROUND: Acne vulgaris is a common disease among adolescents and known to have adverse effects on psychological status. METHODS: A cross-sectional study was conducted on 600 participants by means of a questionnaire designed for this study in high school students. In addition, an objective evaluation of acne in participants was undertaken. The questionnaire consisted of questions about acne, General Health Questionnaire (GHQ) and Rosenberg Self-Esteem Scale (RSES). RESULTS: Five hundred and sixty-three questionnaires out of 600 were answered and 550 adolescents who gave permission for examination were evaluated. The study population consisted of 303 girls and 260 boys between the ages 13 and 19, and the mean age was 15.24 +/- 1.05 years. Acne prevalence was 63.6% with 29.2% non-inflammatory and 34.4% inflammatory acne. It was more prevalent and severe in boys than in girls. Not the objective but the subjective severity of acne and opinion that one could benefit from acne treatment was found to be related to anxiety, depression and self-esteem. Factors implicated among causes of acne were food, bad skin hygiene and hormones in decreasing frequency. Forty-eight per cent of adolescents expect a maximum duration of 4 weeks for treatment. CONCLUSION: Despite the high prevalence of acne, there is still much deficiency of knowledge and wrong beliefs about acne. This indicates that there is an urgent need for education about etiopathogenesis, potential complications and importance of effective treatment for acne. Effective treatment may make significant contributions for the mental health of adolescent and as well as adult populations.

Cutaneous basosquamous carcinoma infiltrating cerebral tissue.

Basosquamous carcinoma of the skin is a rare malignancy with specific histopathological features of both basal cell carcinoma and squamous cell carcinoma. Some authors believe that basosquamous carcinoma is a variant of basal cell carcinoma, while others suggest that this tumour may behave more aggressively. We present a 44-year-old female patient who was diagnosed with a basosquamous carcinoma histopathologically. She had extensive ulcero-vegetative lesions, involving the anterior half of the scalp, the left orbit and the left side of the face. With this case we aim to emphasize the aggressive nature of basosquamous carcinoma and review the literature.

Immunohistochemical findings in notalgia paresthetica.

BACKGROUND: Notalgia paresthetica (NP) is a sensory neuropathy the pathogenesis of which is not yet completely elucidated. OBJECTIVE: The aim of this study was to investigate the histopathological changes in NP with special emphasis on cutaneous innervation. METHODS: Along with site-matched biopsies from 5 healthy individuals, lesional skin biopsies from 14 cases of NP and biopsies from contralateral nonlesional skin in 9 of these cases were stained with hematoxylin-eosin and Congo red. For immunohistochemical analysis, all samples were stained with two general neural markers (S-100 protein and protein gene product 9.5) and two neuropeptides (vasoactive intestinal polypeptide and substance P). RESULTS: Light microscopy was compatible with postinflammatory hyperpigmentation. Immunohistochemistry did not reveal a significant difference in the staining pattern of lesional skin and control tissue (p > 0.05). Although not reaching statistical significance, the percentage of cases which showed no staining was higher in the group of patients with more chronic NP. CONCLUSION: The finding of less immunohistochemical staining in cases with more chronicity could be of clinical importance and is worth investigating further.

Open pilot study on oxcarbazepine for the treatment of notalgia paresthetica.

Notalgia paresthetica is a sensory neuropathy typically presenting with pruritus on the back. There is currently no effective treatment for this disorder. We describe a single patient who responded to oxcarbazepine, and a subsequent trial in another 4 who also responded. Although the number of patients in our study is small, we believe further trials are warranted to determine the efficacy of oxcarbazepine in this disorder.

Power Doppler ultrasonography for the evaluation of skin tumors other than malignant melanoma.

The purpose of this prospective study was to evaluate the value of power Doppler ultrasonography (PDUS) in assessing the vascularity of skin tumors other than malignant melanoma, and to investigate possible diagnostic criteria to help to distinguish malignant from benign tumors preoperatively. Seventy-one patients with the clinical diagnosis of a skin tumor were initially evaluated by ultrasonography. Then PDUS was performed and the presence and type of vascularity (peripheral or mixed) were investigated. Of the 19 benign and 52 malignant lesions, 3 could not be visualized with US. Forty-nine lesions were found to have vascularity (21 peripheral, 28 mixed-type) on PDUS. Of the malignant lesions, 26 showed mixed and 17 showed peripheral-type vascularity; for benign lesions these numbers were 2 and 4 respectively. The presence of vascularity correlated well with pathological behavior (malignant versus benign) (P = 0.00002), width (P = 0.0001), thickness (P = 0.001), dermal disruption (P = 0.0018), and subdermal extension (P = 0.002) of the tumor; however, the type of vascularity correlated only with thickness (P = 0.014). In relation to malignancy, the sensitivity of the presence of vascularity on PDUS was 88% and the specificity was 63%. These values were 93% and 40% respectively, for the mixed type of vascularity when it was accepted as a feature of malignancy. PDUS may help to distinguish malignant from benign skin tumors during preoperative evaluation.

Notalgia paresthetica: a study on pathogenesis.

BACKGROUND: Notalgia paresthetica is a sensory neuropathy involving the dorsal spinal nerves. The characteristic symptom is pruritus on the back, occasionally accompanied by pain, paresthesia, and/or hyperesthesia, which results in a well-circumscribed hyperpigmented patch in the symptomatic area. The etiology of this condition has not yet been completely defined. OBJECTIVE: Possible mechanisms that could explain the pathogenesis of notalgia paresthetica were investigated through clinical examination and various diagnostic tests. METHODS: Ten cases of notalgia paresthetica underwent dermatologic, neurologic, and orthopedic examination. This was followed by skin biopsy, electrodiagnostic investigation, and radiography of the spine. RESULTS: All patients had a typical symptomatology and dermatologic picture. Neurologic examination and standard electrodiagnostic investigation results were normal in all cases. Histopathology was compatible with postinflammatory hyperpigmentation; there were no amyloid deposits. In seven cases, degenerative changes in the vertebrae were observed and, in all of these cases, these changes were most prominent in the vertebrae which corresponded to the dermatome of the cutaneous lesion. CONCLUSIONS: The striking correlation of notalgia paresthetica localization with degenerative changes in the spine suggests that spinal nerve impingement may contribute to the pathogenesis of this entity.