EuroGuiderm guideline on lichen sclerosus-introduction into lichen sclerosus.

INTRODUCTION: Lichen sclerosus (LS) is an inflammatory skin disease affecting all ages. LS typically involves the anogenital site where it causes itching and soreness. It may lead to sexual and urinary dysfunction in females and males; however, it may be asymptomatic. First signs of LS are redness and oedema, typically followed by whitening of the genital skin; sometimes fissuring, scarring, shrinkage and fusion of structures may follow in its course. LS is associated with an increased risk of genital cancer. LS has a huge impact on the quality of life of affected patients, and it is important to raise more awareness of this not uncommon disease in order to diagnose and treat it early. OBJECTIVES: The guideline intends to provide guidance on the diagnostic of LS, highlight important aspects in the care of LS patients (part 1), generate recommendations and treatment algorithms (part 2) on topical, interventional and surgical therapy, based on the latest evidence, provide guidance in the management of LS patients during pregnancy, provide guidance for the follow-up of patients with LS and inform about new developments and potential research aspects. MATERIALS AND METHODS: The guideline was developed in accordance with the EuroGuiDerm Methods Manual v1.3 https://www.edf.one/de/home/Guidelines/EDF-EuroGuiDerm.html. The wording of the recommendations was standardized (as suggested by the GRADE Working Group). The guideline development group is comprised of 34 experts from 16 countries, including 5 patient representatives. RESULTS: Ultrapotent or potent topical corticosteroids in females and males, adults and children remain gold standard of care for genital LS; co-treatment with emollients is recommended. If standard treatment fails in males, a surgical intervention is recommended, complete circumcision may cure LS in males. UV light treatment is recommended for extragenital LS; however, there is limited scientific evidence. Topical calcineurin inhibitors are second line treatment. Laser treatment, using various wave lengths, is under investigation, and it can currently not be recommended for the treatment of LS. Treatment with biologics is only reported in single cases. CONCLUSIONS: LS has to be diagnosed and treated as early as possible in order to minimize sequelae like scarring and cancer development. Topical potent and ultrapotent corticosteroids are the gold standard of care; genital LS is often a lifelong disease and needs to be treated long-term.

EuroGuiderm guideline on lichen sclerosus-Treatment of lichen sclerosus.

INTRODUCTION: Lichen sclerosus (LS) is an inflammatory skin disease affecting all ages. LS typically involves the anogenital site where it causes itching and soreness; it may lead to sexual and urinary dysfunction in females and males; however, it may be asymptomatic. First signs of LS are usually a whitening of the genital skin, sometimes preceded by redness and oedema; fissuring, scarring, shrinkage and fusion of structures may follow in its course. LS is associated with an increased risk of genital cancer. LS has a huge impact on the quality of life of affected patients, and it is important to raise more awareness of this not uncommon disease in order to diagnose and treat it early. OBJECTIVES: The guideline intends to provide guidance on the diagnostic of LS (part 1), highlight important aspects in the care of LS patients, generate recommendations and treatment algorithms (part 2) on topical, interventional and surgical therapy, based on the latest evidence, provide guidance in the management of LS patients during pregnancy, provide guidance for the follow-up of patients with LS and inform about new developments and potential research aspects. MATERIALS AND METHODS: The guideline was developed in accordance with the EuroGuiDerm Methods Manual v1.3 https://www.edf.one/de/home/Guidelines/EDF-EuroGuiDerm.html. The wording of the recommendations was standardized (as suggested by the GRADE Working Group). The guideline development group is comprised of 34 experts from 16 countries, including 5 patient representatives. RESULTS: Ultrapotent or potent topical corticosteroids in females and males, adults and children remain gold standard of care for genital LS; co-treatment with emollients is recommended. If standard treatment fails in males, a surgical intervention is recommended, complete circumcision may cure LS in males. UV light treatment is recommended for extragenital LS; however, there is limited scientific evidence. Topical calcineurin inhibitors are second line treatment. Laser treatment, using various wave lengths, is under investigation, and it can currently not be recommended for the treatment of LS. Treatment with biologics is only reported in single cases. CONCLUSIONS: LS has to be diagnosed and treated as early as possible in order to minimize sequelae like scarring and cancer development. Topical potent and ultrapotent corticosteroids are the gold standard of care; genital LS is often a lifelong disease and needs to be treated long-term.

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.

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.

European guideline for the management of scabies.

Scabies is caused by Sarcoptes scabiei var. hominis. The disease can be sexually transmitted. Patients' main complaint is nocturnal itch. Disseminated, excoriated, erythematous papules are usually seen on the anterior trunk and limbs. Crusted scabies occurs in immunocompromised hosts and may be associated with reduced or absent pruritus. Recommended treatments are permethrin 5% cream, oral ivermectin and benzyl benzoate 25% lotion. Alternative treatments are malathion 0.5% aqueous lotion, ivermectin 1% lotion and sulphur 6-33% cream, ointment or lotion. Crusted scabies therapy requires a topical scabicide and oral ivermectin. Mass treatment of large populations with endemic disease can be performed with a single dose of ivermectin (200 micrograms/kg of bodyweight). Partner management needs a look-back period of 2 months. Screening for other STI is recommended. Patients and close contacts should avoid sexual contact until completion of treatment and should strictly observe personal hygiene rules when living in crowded spaces. Written information should be provided to suspected cases.

2016 European guideline for the management of vulval conditions.

Vulval conditions may present to a variety of clinicians, such as dermatologists, gynaecologists and general practitioners. Women with these conditions are best managed by a multidisciplinary approach, which includes clear referral pathways between disciplines or access to a specialist multidisciplinary vulval service. Informed consent is a prerequisite for all examinations, investigations and treatments. Consent is particularly important for intimate examinations of the anogenital area, and a chaperone should be offered in all cases. All efforts should be made to maintain a patient's dignity. Depending on symptoms and risk factors, screening for sexually transmitted infections (STI) should be considered. If the patient presents with vulval itch, particularly if also complaining of increased vaginal discharge, vulvaginal candidiasis should be excluded. Sexual dysfunction should be considered in all patients with vulval complaints, either as the cause of the symptoms or secondary to symptoms, and assessed if appropriate. This guideline covers several aspects, such as diagnosis and treatment, of the more common vulval conditions (relatively) often encountered at vulval clinics, i.e. vulval dermatitis (eczema), psoriasis, lichen simplex chronicus, lichen sclerosus, lichen planus, vulvodynia and vulval intraepithelial neoplasia (VIN).

Skin phototypes of a Maltese sample population.

BACKGROUND AND AIM: Skin phototyping is a clinical classification system based on a patient's historical reporting of the acute skin response to sunlight. We carried out a simple study to estimate the skin phototypes of the Maltese people in order to acquire more information on the relative risk of the general Maltese population to sun burning and to the more chronic effects of sun exposure, including photoaging and skin cancers. METHOD: A consultant dermatologist and dermatology trainees determined the skin phototype of patients seen with skin infections or skin tags at dermatology outpatients in Malta by carrying out a short interview. RESULTS: Data were collected on 756 patients. The commonest reported skin phototype in both sexes was type III (48.0% overall; 52.4% in males, 43.8% in females); this was followed by type IV in males (30.4%) and type II in females (32.3%). Only 1.2% of the total was of skin type I. CONCLUSION: Approximately one half of the Maltese population is at moderate risk of sun damage; about one quarter is at low risk; and the other quarter is at high risk, the latter group needing more thorough sun avoidance and better surveillance for skin tumours. Social desirability can influence responses to such questionnaires and might have contributed to the difference between males and females noted in our study.

Methotrexate for psoriasis: current European practice. A postal survey.

The aim of this survey was to determine current practice throughout Europe regarding use and monitoring of methotrexate therapy for psoriasis. A structured questionnaire with questions on methotrexate prescribing and monitoring was mailed to 150 dermatologists in 32 European countries in June 2002. Dermatologists' names were chosen at random from the 2001 European Academy of Dermatology and Venereology membership directory. A reply was received from 69 dermatologists of whom 59 prescribed methotrexate regularly. In those patients receiving systemic treatment for psoriasis, methotrexate was the most widely used drug (4.4 patients of every 10) followed by acitretin (3.2) and cyclosporin (1.6). Myelosuppression was the commonest reported fatal side-effect of methotrexate (eight of a total of 10 cases). None of the respondents routinely requested a baseline liver biopsy before starting methotrexate treatment and of every 10 of their patients on long-term methotrexate it was estimated that less than two had had a liver biopsy some time during treatment. Serum measurement of the amino-terminal peptide of type III procollagen (PIIINP) was used routinely to detect liver fibrosis by 12 (20%) of the 59 respondents who regularly prescribed methotrexate. This survey demonstrates that, despite the advent of new therapies, methotrexate retains a central role in the treatment of severe psoriasis in Europe. The responses demonstrated variation in, among others, the methotrexate dose regimen utilized and the use of folate supplementation, confirming the need for randomized controlled studies to address these issues.

Methotrexate for psoriasis.

Methotrexate is an effective antipsoriatic agent and has been widely used to treat severe psoriasis since the 1960s. It is especially useful in acute generalized pustular psoriasis, psoriatic erythroderma, psoriatic arthritis and for extensive chronic plaque psoriasis in patients who are inadequately controlled by topical therapy alone. It has not, however, been formally compared with other systemic treatments for severe psoriasis such as cyclosporin, retinoids or photochemotherapy with psoralen and UVA (PUVA), but in comparison with these other therapies it is inexpensive, with correct use, its safety profile is favourable. In summary, therefore, it can be used as a short-term option to gain control of unstable psoriasis such as pustular psoriasis or erythroderma before returning to other modes of treatment, or more often, as long-term maintenance treatment. The most important potential side-effect is acute myelosuppression, which is the cause of most of the rare deaths attributable to this therapy for psoriasis. Myelosuppression is more likely in the elderly, in patients with renal impairment and/or folate depletion, and with overdose or drug interactions. Long-term therapy carries with it a risk of liver fibrosis which is related to the dosage regimen employed, and is increased by exposure to other hepatic toxins, particularly alcohol. The correlation between the risk of development of liver fibrosis, cumulative lifetime dose and duration of treatment with methotrexate is not clear-cut, but may have been overstated in some studies.

Screening for corticosteroid contact hypersensitivity.

To evaluate which corticosteroids are most useful for the detection of corticosteroid contact allergy in our population, 2123 patients were patch tested with a series of 6 corticosteroids, in parallel with a standard series, and other relevant investigations. 127 patients (5.98%) were allergic to one or more corticosteroids; 96 to tixocortol pivalate, 51 to hydrocortisone butyrate, 47 to budesonide, 11 to betamethasone valerate, 11 to clobetasone butyrate and 8 to clobetasol propionate. 511 patients with negative patch tests to the limited corticosteroid series were in addition tested to a further 12 corticosteroids; only 1 of these patients reacted to a corticosteroid. A combination of tixocortol pivalate and budesonide thus detected 91.3% of corticosteroid-allergic subjects. We believe that both these allergens should be included in the standard series and that there may be a case for extending this further.

Allergic reactions to glyceryl trinitrate and isosorbide dinitrate demonstrating cross-sensitivity.

Glyceryl trinitrate (nitroglycerin) has been in use for relief of angina for over a hundred years, but allergic reactions to it or to other organic nitrates rarely feature in the medical literature. Most of the case reports describe reactions to transdermally applied nitroglycerin. We report a case of a localized allergic reaction to nitroglycerin transdermal patches that developed when these were worn for the first time after 7 days of uneventful treatment with intravenous isosorbide dinitrate. The reaction evolved into a severe and generalized maculopapular rash with facial swelling when intravenous isosorbide dinitrate was re-administered 10 days later. Subsequent patch testing with a transdermal nitroglycerin patch and a placebo nitroglycerin-free patch provoked a reaction to the nitroglycerin patch but not to the placebo, thus excluding allergy to other constituents of the nitroglycerin patch.

Hypertrichosis as the presenting feature of porphyria cutanea tarda.

We report a case of porphyria cutanea tarda in which the only cutaneous abnormality and initial presenting complaint was hypertrichosis. The patient had a history of excessive alcohol consumption. Alcohol avoidance and treatment with low-dose hydroxychloroquine resulted in resolution of the hypertrichosis and normalization of biochemical abnormalities.