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.

Consensus statement on the diagnosis and treatment of sclerosing diseases of the skin, Part 1: Localized scleroderma, systemic sclerosis and overlap syndromes.

The term 'sclerosing diseases of the skin' comprises specific dermatological entities, which have fibrotic changes of the skin in common. These diseases mostly manifest in different clinical subtypes according to cutaneous and extracutaneous involvement and can sometimes be difficult to distinguish from each other. The present consensus provides an update to the 2017 European Dermatology Forum Guidelines, focusing on characteristic clinical and histopathological features, diagnostic scores and the serum autoantibodies most useful for differential diagnosis. In addition, updated strategies for the first- and advanced-line therapy of sclerosing skin diseases are addressed in detail. Part 1 of this consensus provides clinicians with an overview of the diagnosis and treatment of localized scleroderma (morphea), and systemic sclerosis including overlap syndromes.

Induction and exacerbation of subacute cutaneous lupus erythematosus following mRNA-based or adenoviral vector-based SARS-CoV-2 vaccination.

Evidence is accumulating that COVID-19 vaccines might induce or exacerbate autoimmune rheumatic diseases. The currently available COVID-19 vaccines include mRNA and recombinant adenoviral vector vaccines, both encoding SARS-CoV-2 spike protein production as the primary target for neutralizing antibodies. We report a case of subacute cutaneous lupus erythematosus (SCLE) following mRNA vaccination with the Pfizer mRNA vaccine BNT162b2, and summarize the current literature on CLE occurring after COVID-19 vaccination.

2021 European Guideline on the management of proctitis, proctocolitis and enteritis caused by sexually transmissible pathogens.

This guideline intents to offer guidance on the diagnosis and management of patients with gastrointestinal symptoms and a suspected sexually transmitted cause. Proctitis is defined as an inflammatory syndrome of the anal canal and/or the rectum. Infectious proctitis can be sexually transmitted via genital-anal mucosal contact, but some also via digital contact and toys. Neisseria gonorrhoeae, Chlamydia trachomatis (including lymphogranuloma venereum), Treponema pallidum and herpes simplex virus are the most common sexually transmitted anorectal pathogens. Shigellosis can be transferred via oral-anal contact and may lead to proctocolitis or enteritis. Although most studies on these infections have concentrated on men who have sex with men (MSM), women having anal intercourse may also be at risk. A presumptive clinical diagnosis of proctitis can be made when there are symptoms and signs, and a definitive diagnosis when the results of laboratory tests are available. The symptoms of proctitis include anorectal itching, pain, tenesmus, bleeding, constipation and discharge in and around the anal canal. The majority of rectal chlamydia and gonococcal infections are asymptomatic and can only be detected by laboratory tests. Therefore, especially when there is a history of receptive anal contact, exclusion of anorectal infections is generally indicated as part of standard screening for sexually transmitted infections (STIs). Condom use does not guarantee protection from STIs, which are often spread without penile penetration. New in this updated guideline is: (i) lymphogranuloma venereum proctitis is increasingly found in HIV-negative MSM, (ii) anorectal Mycoplasma genitalium infection should be considered in patients with symptomatic proctitis after exclusion of other common causations such N. gonorrhoeae, C. trachomatis, syphilis and herpes, (iii) intestinal spirochetosis incidentally found in colonic biopsies should not be confused with syphilis, and (iv) traumatic causes of proctitis should be considered in sexually active patients.

Human Papillomavirus-type distribution in anogenital lesions of prepubertal children.

BACKGROUND: In contrast to adults, only limited data are available on the human papillomavirus (HPV)-type spectrum in anogenital warts (AGW) of children. OBJECTIVE: This study aimed to evaluate the HPV-type spectrum in AGW of prepubertal children. MATERIALS & METHODS: In a retrospective German multicentre study, HPV genotyping was performed in AGW biopsies of 55 1- to 12-year-old children using HPV group-specific PCRs followed by hybridization with type-specific probes or sequence analysis. RESULTS: Human papillomavirus-DNA was found in 53 of the 55 AGW. In 58.5% (31/53) of the HPV-positive AGW, mucosal HPV types were detected. HPV6 (27/53, 50.9%) was the predominant type. 43.4% (23/53) of the lesions were induced by cutaneous HPV types (HPV2, HPV27, HPV57). Mucosal HPV types were significantly more common in children under 5 years of age than in children 5 years of age and older (22/25, 88.0% [95% CI: 70.0-95.8] vs. 9/28, 32.1% [95% CI: 17.9-50.7], P < 0.001). In contrast, cutaneous HPV types were significantly more prevalent in the 5- to 12-year age group (4/25, 16.0% [95% CI 6.4-34.7] vs. 19/28, 67.9% [95% CI 49.3-82.1], P < 0.001). CONCLUSION: Anogenital warts in 5- to 12-year-old children are frequently associated with cutaneous HPV types, possibly due to horizontal transmission. HPV typing, in addition to comprehensive clinical and psychosocial evaluation, can potentially help in the assessment of these cases.

[Paraneoplastic subacute cutaneous lupus erythematosus]. / Paraneoplastischer subakut kutaner Lupus erythematodes.

Subacute cutaneous lupus erythematosus (SCLE) is a subtype of cutaneous lupus erythematosus characterized by high photosensitivity, the occurrence of annular or papulosquamous skin lesions located in body regions exposed to UV light, the presence of anti-Ro/SS­A antibodies, and mild systemic involvement, such as arthralgia and myalgia. Similar to other subtypes of cutaneous lupus erythematosus, certain trigger factors exist for the development of SCLE, such as exposure to UV light, cigarette smoking and drugs. Rheumatic diseases, such as dermatomyositis, have been known as paraneoplastic syndromes for a long time. In recent years, there has been an accumulation of publications on the association of SCLE with malignant diseases. This article reports the case of a 78-year-old female patient who was diagnosed with the concomitant development of SCLE and gastric carcinoma. In all older patients with SCLE, patients with widespread skin affection outside the UV-exposed body areas or patients with B­symptoms, the presence of a paraneoplastic SCLE should be considered and appropriate diagnostic steps should be initiated to screen for an associated neoplastic disease.

[Anakinra therapy in a patient with multifocal pyoderma gangrenosum and hidradenitis suppurativa]. / Anakinra-Therapie eines multifokalen Pyoderma gangraenosum bei einem Patienten mit Hidradenitis suppurativa.

The case of a patient with pyoderma gangrenosum and exacerbation after an intense massage is reported. After different immunosuppressive therapies and former diagnosis of hidradenitis suppurativa in his medical history the patient underwent therapy with anakinra 100 mg per day and showed good improvement of his ulcers.

[Reticular erythematous mucinosis-A special subtype of cutaneous lupus erythematosus?] / Retikuläre erythematöse Muzinose ­ Sonderform eines kutanen Lupus erythematodes?

Reticular erythematous mucinosis (REM syndrome) is a rare skin disease that predominantly affects women. It is clinically characterized by flat, partly reticular, irregularly configurated pale erythema located in the region of the central chest and upper back. The skin alterations are usually asymptomatic or associated with slight pruritus or burning. Extracutaneous diseases and involvement of internal organs do not occur in REM syndrome. Histopathological features include perivascular and periadnexal lymphocytic inflammatory infiltrates as well as increased deposition of mucin in the dermis. Due to the similar histology to lupus erythematosus (LE) tumidus, it is discussed whether REM syndrome can be assigned to the spectrum of cutaneous LE or can be assessed as a lupus-like disease. Moreover, both conditions respond well to treatment with hydroxychloroquine.

[Diffuse hyperkeratotic papules of the lower abdomen and genital region in a 38-year old male patient]. / Diffuse hyperkeratotische Papeln am unteren Abdomen und im Genitalbereich bei einem 38­jährigen Patienten.

A patient with diffuse angiokeratomas of the lower abdomen and genital region was diagnosed with Fabry disease on the basis of genetic testing. Fabry disease is an X-linked lysosomal storage disease that can affect several organ systems including the heart or kidneys, resulting in reduced median survival. Pathogenetically, Fabry disease leads to a deficiency of the lysosomal enzyme α­galactosidase A (α-GAL A). Treatment options include lifelong enzyme replacement therapy or chaperone therapy.

[Reality of inpatient vasoactive treatment with prostacyclin derivatives in patients with acral circulation disorders due to systemic sclerosis in Germany]. / Versorgungsrealität der stationären vasoaktiven Therapie mit Prostazyklinderivaten bei Patienten mit akralen Durchblutungsstörungen bei systemischer Sklerose in Deutschland.

BACKGROUND: Raynaud's phenomenon and the frequently ensuing digital ulcerations represent an early and very distressing symptom in patients with systemic sclerosis (scleroderma, SSc) causing significant limitations in the ability to work and quality of life. The use of vasoactive drugs (especially intravenous prostacyclin derivatives) is recommended to reduce the risk of hypoxic tissue damage up to the loss of fingers. METHODS: In order to obtain information about the current state of treatment of patients with prostacyclin derivatives in routine clinical life in Germany, a survey was conducted among the centers affiliated to the German Network for Systemic Scleroderma (DNSS). In addition, a separate patient survey was conducted by the schleroderma self-help group (Sklerodermie Selbsthilfe e. V.), which only covered the symptoms Raynaud's syndrome, digital ulcers and the use of intravenous prostacyclin derivatives. RESULTS: Of the 433 patients surveyed 56% stated that they had already been treated with prostacyclin derivatives (iloprost/alprostadil) because of their illness and symptoms. A total of 61% received the treatment for severe Raynaud's phenomenon and 39% for digital ulcerations. Most respondents not only experienced an improvement in Raynaud's phenomenon and digital ulcers but also a significant improvement of limitations in everyday life. They also needed significantly less outside help and absenteeism from work was much lower. CONCLUSION: Patients consistently reported a positive effect of treatment with prostacyclin derivatives on Raynaud's phenomenon, acral ulcerations, pain and daily restrictions and felt well and safely cared for during inpatient treatment. These positive effects in the patients' perceptions provide crucial information supporting and confirming the current European and international treatment recommendations.