Post-acute phase and sequelae management of epidermal necrolysis: an international, multidisciplinary DELPHI-based consensus.

BACKGROUND: Long-term sequelae are frequent and often disabling after epidermal necrolysis (Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN)). However, consensus on the modalities of management of these sequelae is lacking. OBJECTIVES: We conducted an international multicentric DELPHI exercise to establish a multidisciplinary expert consensus to standardize recommendations regarding management of SJS/TEN sequelae. METHODS: Participants were sent a survey via the online tool "Survey Monkey" consisting of 54 statements organized into 8 topics: general recommendations, professionals involved, skin, oral mucosa and teeth, eyes, genital area, mental health, and allergy workup. Participants evaluated the level of appropriateness of each statement on a scale of 1 (extremely inappropriate) to 9 (extremely appropriate). Results were analyzed according to the RAND/UCLA Appropriateness Method. RESULTS: Fifty-two healthcare professionals participated. After the first round, a consensus was obtained for 100% of 54 initially proposed statements (disagreement index < 1). Among them, 50 statements were agreed upon as 'appropriate'; four statements were considered 'uncertain', and ultimately finally discarded. CONCLUSIONS: Our DELPHI-based expert consensus should help guide physicians in conducting a prolonged multidisciplinary follow-up of sequelae in SJS-TEN.

Supportive care in the acute phase of Stevens-Johnson syndrome and toxic epidermal necrolysis: an international, multidisciplinary Delphi-based consensus.

BACKGROUND: Supportive care is the cornerstone of management of adult and paediatric Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN). However, consensus on the modalities of supportive care is lacking. OBJECTIVES: Our aim in this international multicentric Delphi exercise was to establish a multidisciplinary expert consensus to standardize recommendations regarding supportive care in the acute phase of SJS/TEN. METHODS: Participants were sent a survey via the online tool SurveyMonkey, consisting of 103 statements organized into 11 topics: multidisciplinary team composition, suspect drug management, infection prevention, fluid resuscitation and prevention of hypothermia, nutritional support, pain and psychological distress management, management of acute respiratory failure, local skincare, ophthalmological management, management of other mucosa, and additional measures. Participants evaluated the level of appropriateness of each statement on a scale of 1 (extremely inappropriate) to 9 (extremely appropriate). The results were analysed according to the RAND/UCLA Appropriateness Method. RESULTS: Forty-five participants from 13 countries (on three continents) participated. After the first round, a consensus was obtained for 82.5% of the 103 initially proposed statements. After the second round, a final consensus was obtained for 102 statements. CONCLUSIONS: We have reached an international Delphi-based consensus on best supportive care practice for SJS/TEN. Our expert consensus should help guide physicians in treating patients with SJS/TEN and thereby improve short-term prognosis and the risk of sequelae.

2020 European guideline on the management of genital molluscum contagiosum.

Molluscum contagiosum is a benign viral epidermal infection associated with high risk of transmission. The guideline is focused on the sexually transmitted molluscum contagiosum. The diagnosis is clinical with characteristic individual lesions, termed 'mollusca', seen as dome-shaped, smooth-surfaced, pearly, firm, skin-coloured, pink, yellow or white papules, 2 - 5 mm in diameter with central umbilication. Dermoscopy may facilitate diagnosis. Therapeutic options are numerous, including physical treatments (cautery, curettage and cryotherapy), topical chemical treatments (e.g. podophyllotoxin and imiquimod) or waiting for spontaneous resolution in immunocompetent patients. In pregnancy, it is safe to use physical procedures (e.g. cryotherapy). Immunosuppressed patients develop severe and recalcitrant molluscum lesions that may require treatment with cidofovir, imiquimod or interferon. Patients with molluscum contagiosum infection should be offered to be screened for other sexually transmitted infections.

2020 European guideline on the management of syphilis.

The 2020 edition of the European guideline on the management of syphilis is an update of the 2014 edition. Main modifications and updates include: -The ongoing epidemics of early syphilis in Europe, particularly in men who have sex with men (MSM) -The development of dual treponemal and non-treponemal point-of-care (POC) tests -The progress in non-treponemal test (NTT) automatization -The regular episodic shortage of benzathine penicillin G (BPG) in some European countries -The exclusion of azithromycin as an alternative treatment at any stage of syphilis -The pre-exposure or immediate post-exposure prophylaxis with doxycycline in populations at high risk of acquiring syphilis.

[Treatment of pigmentation disorders in association with systemic diseases]. / Therapie von Pigmentstörungen in Zusammenhang mit systemischen Erkrankungen.

Pigmentation disorders are a frequent skin problem and incorporate a broad spectrum of diseases, caused by an abnormal melanin pigmentation or also non-melanin pigmentation of the skin. Both hypermelanosis and hypomelanosis can be hereditary or acquired. This article summarizes the treatment approaches that are used in the majority of acquired pigmentation disorders of the skin. The following forms of hypermelanosis are addressed: lentiginosis, hyperpigmentation due to endocrine disorders or other systemic diseases, drug-induced hyperpigmentation. Acquired hypomelanoses include postinflammatory hypomelanosis, chemical depigmentation, idiopathic guttate hypomelanosis and punctate leucoderma. With reference to non-melanin pigmentation, the exogenous pigmentation due to chemicals, metals and drug exposure are discussed. The treatment is primarily based on finding the cause of the alterations to the pigment. The affected area, age and ethnic origin are also important factors. The spectrum of therapeutic options is broad: topical agents, chemical peeling, systemic agents, laser and light-based treatment. As some of these treatment procedures can have side effects, the availability of a protocol that contains information on the drug concentration, dose, parameters for laser treatment and the number of sessions is important. For every disorder the specific dermatological treatment is presented even when some pigmentation alterations that occur in association with systemic diseases, are cured by the treatment of the primary disease. Most diseases are exacerbated by exposure to UV light. Therefore, sun protection is recommended and a cosmetic coverage is indicated.

Quality of life measurement in occupational skin diseases. Position paper of the European Academy of Dermatology and Venereology Task Forces on Quality of Life and Patient Oriented Outcomes and Occupational Skin Disease.

The European Academy of Dermatology and Venereology (EADV) has started the 'Healthy Skin @ Work' campaign aimed to raise awareness among the public and EU authorities on the frequency and impact of occupational skin diseases (OSDs). The EADV Task Forces (TFs) on Quality of Life and Patient Oriented Outcomes (QoL/PO) and on OSD present their mutual position statement on QoL assessment in OSDs. The EADV TFs recommend the use of the DLQI as a dermatology-specific instrument and SF-36 as a generic instrument in health-related (HR) QoL studies on OSDs. The OSD-specific questionnaire, LIOD, is not recommended for general use in its present form because of its three months recall period. The EADV TFs discourage the use of non-validated and of non-validated modifications of previously validated HRQoL instruments. The EADV TFs wish to encourage research into: the HRQoL impact of OSDs other than occupational contact dermatitis and hand eczema; comparisons between the effects of different treatments and other interventions on HRQoL in OSDs; and into the HRQoL impairment of patients with OSDs from different countries, and with different provoking factors, to predict if the results of successful therapeutic and educational interventions may be generalized across countries and between occupations.

Personal solar ultraviolet radiation dosimetry in an occupational setting across Europe.

BACKGROUND: Work-related solar ultraviolet radiation (UVR) is an important factor in the pathogenesis of non-melanoma skin cancer (NMSC). The World Health Organization, through the International Agency for Research on Cancer, has classified solar UVR as a group 1 carcinogen since 2012. The main problems encountered so far in the study of occupationally induced skin cancer include the lack of accurate occupational UVR dosimetry as well as insufficient distinction between occupational and leisure UVR exposure and underreporting of NMSC. OBJECTIVES: The aim of this study was to collect long-term individual UVR measurements in outdoor workers across European countries. METHODS: A prospective study was initiated through the European Academy of Dermatology and Venereology, Healthy Skin@Work Campaign, measuring UVR exposure doses at occupational settings of masons from five European countries. Measurements were performed for several consecutive months using the GENESIS-UV measurement system. RESULTS: The results identified alarming UVR exposure data. Average daily UVR doses ranged 148.40-680.48 J/m2 in Romania, 342.4-640.8 J/m2 in Italy, 165.5-466.2 J/m2 in Croatia, 41.8-473.8 J/m2 in Denmark and 88.15-400.22 J/m2 in Germany. Results showed an expected latitude dependence with increasing UVR yearly dosage from the north to the south of Europe. CONCLUSIONS: This study shows that outdoor workers from EU countries included in this study are exposed to high levels of occupational solar UVR, vastly exceeding the occupational exposure limits for solar UVR exposure, considered to be 1-1.33 SED/day in the period from May to September. This finding may serve as an evidence-based recommendation to authorities on implementing occupational skin cancer prevention strategies.

Validation of the dermatology-specific proxy instrument the Infants and Toddlers Dermatology Quality of Life.

BACKGROUND: The first dermatology-specific proxy health-related quality of life (HRQoL) instrument for children 0-4 years old with skin diseases, the Infants and Toddlers Dermatology Quality of Life (InToDermQoL), was recently developed. In order to avoid the problem of cross-cultural inequivalence focus groups work and pilot tests were organized simultaneously in all national centres of the project. The InToDermQoL showed good comprehensibility, clarity and acceptance. OBJECTIVE: To validate the InToDermQoL questionnaire during international field tests. METHODS: Internal consistency, test-retest reliability, convergent and discriminant validity of the InToDermQoL questionnaire were checked during international field tests. RESULTS: Parents of 473 children with skin diseases filled in the national language versions of the InToDermQoL questionnaire. All three age-specific versions of the InToDermQoL questionnaire with 10, 12 and 15 items, respectively, showed high internal consistency (Cronbach's α 0.90-0.93), good test-retest reliability (correlation coefficients > 0.9), significant correlations with the most widely used atopic dermatitis-specific proxy instrument, the Infants Dermatitis Quality of Life Index (correlation coefficients 0.68-0.79). The InToDermQoL versions for children <3 years old well correlated with the atopic dermatis severity measure Scoring of Atopic Dermatitis (correlation coefficients 0.66 and 0.86 for 10 and 12 items versions, respectively). The InToDermQoL questionnaire discriminated well among different diagnoses and disease severity levels. CONCLUSION: Our field tests confirmed internal consistency, test-retest reliability, convergent and discriminant validity of the InToDermQoL questionnaire. Development and validation of the InToDermQoL questionnaire make it possible to assess dermatology-specific aspects of HRQoL in youngest children with skin diseases. There are many reasons to assess HRQoL in dermatologic clinical practice, and we hope that our new instrument will be used internationally in paediatric dermatology for research and practical needs.

Epidermolysis bullosa-specific module of the Infants and Toddlers Dermatology Quality of Life (InToDermQoL) questionnaire.

BACKGROUND: Epidermolysis bullosa (EB) may have severe impact on different aspects of patients' life. Until now there was no EB-specific quality of life (QoL) instrument for young children. OBJECTIVE: To create EB-specific proxy module of the Infants and Toddlers Dermatology Quality of Life (InToDermQoL) questionnaire. METHODS: Focus groups with parents of children with EB were organized. Parents of EB children were interviewed by the project staff with regard to their perception of QoL issues of the skin disease of their children. RESULTS: Focus groups with parents of EB children in Ukraine and Romania were organized. Parents represented eight boys and 12 girls from 3 months to 4 years old with different EB types and disease severity. Based on the analysis of focus groups' results, two EB specific items that were not mentioned by parents of children with other skin diseases and therefore were not included to the dermatology-specific InToDermQoL questionnaire were developed: 'problems with defecation' and 'problems with shoes'. These problems were mentioned by 55% of all parents and 11.76% of parents that represented EB children older than 1 year, respectively. CONCLUSION: We want to invite other centres and EB related organizations to join our project starting from the pilot test. There are many different reasons why QoL measurement is important in dermatology clinical practice and our goal is practical use of the instrument in children with EB.

Creation and pilot test results of the dermatology-specific proxy instrument: the Infants and Toddlers Dermatology Quality of Life.

BACKGROUND: Until now, there was no validated dermatology-specific health-related quality of life (HRQoL) instrument to be used in youngest patients. OBJECTIVE: To create dermatology-specific proxy instrument for HRQoL assessment in children from birth to 4 years. METHODS: International focus groups, item selection and pilot tests were utilized. In order to avoid the problem of cross-cultural inequivalence, focus group work and pilot tests were planned simultaneously in all national centres of the project. Comprehensibility, clarity, acceptance and internal consistency of new instrument were checked. RESULTS: The title 'Infants and Toddlers Dermatology Quality of Life' was chosen for our new instrument with the proposed acronym 'InToDermQoL'. Focus group work was completed in seven national centres (Croatia, Germany, Greece, Malta, Poland, Romania and Ukraine). A total of 170 families of children with different skin diseases were interviewed, and a pilot version of the instrument was created. Centres from France, Denmark and Spain have joined the project at this stage. Parents of 125 children with skin diseases filled in the pilot versions of the instrument. Good comprehensibility, clarity, acceptance and internal consistency of the InToDermQoL were confirmed. The pilot test results showed that the InToDermQoL questionnaire well differentiates severity-dependent differences. It was also checked and confirmed during the pilot test that no significant information was missed in the questionnaire. Three age-specific versions of the InToDermQoL questionnaire with 10, 12 and 15 items, respectively, were approved for field tests. CONCLUSION: The pilot test results showed that the InToDermQoL questionnaire has good comprehensibility, clarity, acceptance and internal consistency and well differentiates severity-dependent differences. Further validation of the InToDermQoL during international field test will be performed.

The regular use of an emollient improves symptoms of atopic dermatitis in children: a randomized controlled study.

BACKGROUND: Emollients are considered as a first-line therapy for the treatment of atopic dermatitis (AD). However, evidence-based proof that the regular use of emollients reduces AD severity is lacking. OBJECTIVE: To assess whether the regular use of emollients results in a reduction in AD severity in children with AD. METHODS: In this multicentre randomized, parallel group, open-label study, children with mild-to-moderate AD were recruited during a flare. After flare resolution with a topical corticosteroid, patients were randomized to V0034CR emollient, reference emollient or no emollient (1:1:1 ratio), for 12 weeks. AD severity was assessed regularly by physicians [Scoring for Atopic Dermatitis (SCORAD) and subcomponents, IGA] and by parents (PO-SCORAD and POEM). RESULTS: A total of 335 patients were randomized to V0034CR (n = 111), reference emollient (n = 116) or no emollient (n = 108). After 12 weeks of treatment, SCORAD score was reduced by 5.28 points in the V0034CR group and by 3.36 points in the reference emollient group compared with the no emollient group (+4 points; P < 0.001 in both emollient groups vs. no emollient group). In a similar manner, PO-SCORAD score was reduced by 4.88 and 2.67 points in the V0034CR and reference emollient groups, respectively, but increased by 2.90 points in the no emollient group (P < 0.001). Similar results were observed for POEM. A continuous decrease in all scores was observed over the 12-week treatment period. At the end of the study, the percentage of patients in complete remission (i.e. without a new flare over the treatment period) was higher in the V0034CR (59.5%) and reference emollient (44.3%) groups than in the no emollient group (29.8%; P < 0.001). CONCLUSION: These results demonstrate that the regular use of emollients in children with mild-to-moderate AD reduces the severity of symptoms and, therefore, support their use as a first-line treatment for these patients.

[Light-based inflammatory acne treatments]. / Phototherapie der entzündlichen Akne.

Light-based acne treatments may represent a new emerging treatment for acne that does not increase the risk of bacterial resistance and they may be potentially effective with a favorable safety profile. Current data show that photodynamic therapy reduces inflammatory lesions and significantly improves acne. However, there is no consensus on the optimal implementation in the treatment of acne. In addition to topically applied photodynamic therapy, intense pulsed light, pulsed dye lasers, potassium-titanyl-phosphate lasers, infrared diode lasers, broad-spectrum continuous-wave light sources (red light, blue-red light) have been introduced as alternative treatments. Since well-designed studies to evaluate their efficacy versus traditional medical therapies are lacking and standardized regimens have not been agreed upon, procedures including laser, intense pulsed light, and photodynamic therapy should currently not be considered first-line treatment for inflammatory acne.

[Laser treatment of basal cell carcinoma]. / Lasertherapie des Basalzellkarzinoms.

With a clear increase in the incidence and a continuously earlier onset, the main risk factors for the development of basal cell carcinoma are still exposure to sunlight, fair skin, immunosuppression, carcinogens such as arsenic, chronic irritations and certain genodermatoses. Treatment options for localized resectionable basal cell carcinoma include micrographically controlled surgery, simple excision, curettage, laser ablation, cryosurgery, imiquimod, 5­fluorouracil, photodynamic treatment and radiotherapy. Non-surgical treatment options are more suited for cases in which surgical procedures lead to disfigurement or functional impairments or for patients with a high surgical risk. Laser treatment, ablative and non-ablative as monotherapy or in combination can represent a meaningful treatment option in selected cases. In recent years there has been an increase in knowledge about the indications and effects of laser treatment of basal cell carcinoma; nevertheless, further studies with a high level of evidence are necessary.

European guideline for the management of pediculosis pubis.

Pediculosis pubis is caused by Phthirus pubis. The disease can be sexually transmitted. Patients main complain is of itch in the pubic area. The parasite can be spotted with the naked eye and blue macules can be observed in the pubic area. First line therapy consists of permethrin or pyrethrins with piperonyl butoxide. Second line therapy contains phenothrin, malathion and oral ivermectin. Partner management needs a look-back period of time of 3 months. Pubic lice incidence is increased in populations groups living in crowded spaces with scarce sanitary conditions as in time of war or disaster.

The Eastern European experience on occupational skin diseases. Make underreporting an issue?

BACKGROUND: While legislation in most of the Eastern European countries is nowadays widely harmonized with the legal safety and health provisions of Western countries, there is still a sustained resistance to the notification of occupational skin diseases (OSD). OBJECTIVE: The aim of the study was to identify the main barriers in notification and recognition of OSD in 22 Eastern European countries. METHODS: An online survey was administered to key persons in the field of occupational safety and health in 22 Eastern European countries. Multiple variables of the notification system were studied, including clinical, organizational and educational issues. RESULTS: The main causes of underreporting OSD are ineffective enforcement of occupational safety and health legislation, contractual relationship employer-employee, long duration of the notifying process, restrictions of the notification systems in terms of who is entitled to notify an OSD, ineffective regulations in regards to the pre-employment and periodical medical examination, ineffective compensation schemes, restraints and hesitations, mainly from the doctors, inappropriate mentalities - fear of losing the jobs, fining of the employers by the authorities, stigmatization of the workers with OSD, additional costs for employers, stakeholders' lack of interest in notifying, lack of guidelines and protocols and lack of preventive programmes. CONCLUSIONS: The most valuable method for a proper recognition of OSD is to increase the awareness of physicians involved in the management of OSD (occupational physicians, GPs, dermatologists), as well as employers and workers. There is an urgent need to improve national legislation, to develop and promote adequate preventive programmes, emphasizing ethical, legal, economical and psychological aspects in order to achieve an increased recognition and a real reporting of OSD, and to enforce an international action plan for Eastern Europe in order to improve the notification of OSD.