European expert consensus statement on the systemic treatment of alopecia areata.

Alopecia areata is an autoimmune form of non-scarring hair loss. It is usually characterized by limited areas of hair loss. However, the disease may progress to complete scalp and body hair loss (alopecia totalis, alopecia universalis). In patients with alopecia areata hair loss significantly impacts the quality of life. Children and adolescents with alopecia areata often experience bullying, including physical aggression. The disease severity evaluation tools used in clinical practice are: the Severity of Alopecia Tool (SALT) score and the Alopecia Areata Scale (AAS). A SALT score equal to or greater than 20 constitutes a commonly accepted indication for systemic therapy in alopecia areata. When using the AAS, moderate to severe alopecia areata should be considered a medical indication for systemic treatment. Currently, the only two EMA-approved medications for alopecia areata are baricitinib (JAK 1/2 inhibitor) for adults and ritlecitinib (JAK 3/TEC inhibitor) for individuals aged 12 and older. Both are EMA-approved for patients with severe alopecia areata. Other systemic medications used off-label in alopecia areata include glucocorticosteroids, cyclosporine, methotrexate and azathioprine. Oral minoxidil is considered an adjuvant therapy with limited data confirming its possible efficacy. This consensus statement is to outline a systemic treatment algorithm for alopecia areata, indications for systemic treatment, available therapeutic options, their efficacy and safety, as well as the duration of the therapy.

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.

Trichoscopy may enhance the differential diagnosis of erythroderma.

There is a paucity of data concerning the usefulness of trichoscopy in patients with erythroderma. The aim of the study was to compare the trichoscopic features in erythroderma of various aetiologies. In total, 49 patients with a determined cause of erythroderma [including atopic dermatitis (AD), mycosis fungoides (MF), allergic contact eczema (ACE), psoriasis (Pso), Sézary syndrome (SS), drug reaction, pityriasis rubra pilaris (PRP), dermatomyositis (DM), actinic reticuloid (AR), crusted scabies (CS) and pemphigus foliaceus (PF)] were included in the study. Dotted vessels were present in patients with AD, PRP, MF, SS and Pso, and absent in DM, CS and PF (χ², P < 0.02). Spermatozoon-like vessels were observed only in MF and SS (P = 0.001). Whitish-pinkish structureless areas were described in all patients with DM, AR and CS (P < 0.03). The type of vessel and the presence of whitish-pinkish structureless areas under trichoscopy may indicate the cause of erythroderma.

Guidelines for clinical trials of frontal fibrosing alopecia: consensus recommendations from the International FFA Cooperative Group (IFFACG).

BACKGROUND: Frontal fibrosing alopecia (FFA) has become one of the most common causes of cicatricial alopecia worldwide. However, there is a lack of clear aetiology and robust clinical trial evidence for the efficacy and safety of agents currently used for treatment. OBJECTIVES: To enable data to be collected worldwide on FFA using common criteria and assessment methods. METHODS: A multicentre, international group of experts in hair loss was convened by email to create consensus recommendations for clinical trials. Consensus was defined at > 90% agreement on each recommended part of these guidelines. RESULTS: Standardized diagnostic criteria, severity rating, staging, and investigator and patient assessment of scalp hair loss and other clinical features of FFA were created. CONCLUSIONS: These guidelines should allow the collection of reliable aggregate data on FFA and advance efforts in both clinical and basic research to close knowledge gaps in this condition.

The significance of dermoscopy and trichoscopy in differentiation of erythroderma due to various dermatological disorders.

BACKGROUND: The diagnosis of a patient with erythroderma may be difficult and sometimes pose a challenge for both dermatologist and pathologist. The role of dermoscopy in this area seems to be poorly investigated. There are only a few reports, with limited number of patients, describing dermoscopic features in erythroderma of various origins. To the best of our knowledge, none of the previous studies had included trichoscopic examination. OBJECTIVES: Analysis of dermoscopic and trichoscopic patterns in series of patients with erythroderma. METHODS: We retrospectively analysed 28 adult patients who presented with erythroderma between May 2016 and August 2020. Demographic data, disease course and duration, previous treatment, as well as dermoscopic and trichoscopic features were analysed. RESULTS: There were 9 patients (32.1%) with the diagnosis of mycosis fungoides, 8 patients (28.5%) with atopic dermatitis, 3 patients (10.5%) with Sézary syndrome and 3 patients (10.5%) with pityriasis rubra pilaris. The others were diagnosed with allergic eczema (n = 1; 3.6%), dermatomyositis sine myositis (n = 1; 3.6%), psoriasis (n = 1; 3.6%), actinic reticuloid (n = 1; 3.6%) and crusted scabies (n = 1; 3.6%). Characteristic dermoscopic/trichoscopic patterns have been observed in erythroderma due to crusted scabies, psoriasis, dermatomyositis sine myositis, Sézary syndrome and pityriasis rubra pilaris. Differentiation of mycosis fungoides and long-standing atopic dermatitis based on dermoscopy is difficult, as the overlap of vessel morphology, background colour and scale colour exists. Similarly, differentiation between AD and AE based on dermoscopy/trichoscopy seems to be impossible, and clinical background is crucial. CONCLUSION: Dermoscopy and trichoscopy seem to provide additional clues in the assessment of erythrodermic patient. Depending on the underlying cause, trichoscopy or dermoscopy may be more useful.

Therapeutic management in paediatric alopecia areata: A systematic review.

Alopecia areata is the third most common cause of dermatology consultations in children but the treatment of paediatric alopecia areata remains challenging. A systematic review of the literature about the treatment of alopecia areata in children (≤18 years old) was performed on 11 May 2020 by searching the PubMed, Scopus and EBSCO databases. The terms used for the search were: 'alopecia areata', 'alopecia totalis' or 'alopecia universalis' combined with 'paediatric', 'children' or 'childhood'. A total of 89 articles were included in final evaluation. The most commonly assessed treatment options in paediatric alopecia areata were topical immunotherapy (response rate in monotherapy: 54%; 187/345) intralesional glucocorticosteroids (75%; 211/280), systemic glucocorticosteroids (73%; 102/140), and anthralin (42%; 31/74). Topical glucocorticosteroids (81%; 35/43), systemic Janus kinase (JAK) inhibitors (90%; 27/30), topical calcineurin inhibitors (42%; 8/19), topical JAK inhibitors (65%; 11/17), PUVA therapy (56%; 9/16) and 308-nm excimer laser (77%; 10/13) were also evaluated. Additionally, evaluation in smaller numbers of paediatric patients included methotrexate (100%; 10/10), topical minoxidil (44%; 4/9) and cyclosporine (83%; 5/6). There were limited data considering children with alopecia areata treated with azathioprine, hydroxychloroquine, topical sildenafil, topical prostaglandin analogues, fractional carbon dioxide laser, leflunomide, mesalazine, apremilast, dupilumab, ustekinumab, efalizumab, botulinum toxin, and compound glycyrrhizin. On the basis of the limited data available glucocorticosteroids (systemic, intralesional or topical) and JAK inhibitors (systemic or topical) may be considered the best documented and most effective treatment options in alopecia areata in children. There are no sufficient paediatric data to compare treatment safety and relapse rates in these therapeutic modalities.

Current controversies in trichology: a European expert consensus statement.

INTRODUCTION: Hair disorders are one of the most common conditions within dermatology practice but, although new diagnostic tools and therapeutic options have arisen, the management of these patients still represents a major clinical challenge. OBJECTIVE: This study aimed at gathering information and achieving consensus on relevant recommendations on the latest advances in alopecia, trichoscopy and hair dermocosmetics. METHODS: Experts of the steering committee consulted the available evidence on trichology-related areas from the past 5 years and formulated recommendations based on the evidence and their experience. A modified two-round Delphi procedure was performed among 45 European dermatologists experts in trichology to consult their degree of agreement on twenty recommendations, using a 4-point Likert scale. Consensus was defined as >80% of participants scoring either 1 (totally agree) or 2 (agree). RESULTS: In the first round of the Delphi questionnaire, 75% of the recommendations reached consensus. Those that were not agreed upon were reformulated by the steering committee and voted again after an online meeting, where consensus was achieved in all recommendations. CONCLUSIONS: All recommendations reached consensus after the two-round Delphi questionnaire and may be useful in clinical practice for dermatologists. The participants agreed that besides this consensus, further clinical studies are needed to assess the benefits of the emerging tools and treatments and to clarify the controversies that still exist in the field, aiming at improving patients' quality of life.

Standardization of dermoscopic terminology and basic dermoscopic parameters to evaluate in general dermatology (non-neoplastic dermatoses): an expert consensus on behalf of the International Dermoscopy Society.

BACKGROUND: Over the last few years, several articles on dermoscopy of non-neoplastic dermatoses have been published, yet there is poor consistency in the terminology among different studies. OBJECTIVES: We aimed to standardize the dermoscopic terminology and identify basic parameters to evaluate in non-neoplastic dermatoses through an expert consensus. METHODS: The modified Delphi method was followed, with two phases: (i) identification of a list of possible items based on a systematic literature review and (ii) selection of parameters by a panel of experts through a three-step iterative procedure (blinded e-mail interaction in rounds 1 and 3 and a face-to-face meeting in round 2). Initial panellists were recruited via e-mail from all over the world based on their expertise on dermoscopy of non-neoplastic dermatoses. RESULTS: Twenty-four international experts took part in all rounds of the consensus and 13 further international participants were also involved in round 2. Five standardized basic parameters were identified: (i) vessels (including morphology and distribution); (ii) scales (including colour and distribution); (iii) follicular findings; (iv) 'other structures' (including colour and morphology); and (v) 'specific clues'. For each of them, possible variables were selected, with a total of 31 different subitems reaching agreement at the end of the consensus (all of the 29 proposed initially plus two more added in the course of the consensus procedure). CONCLUSIONS: This expert consensus provides a set of standardized basic dermoscopic parameters to follow when evaluating inflammatory, infiltrative and infectious dermatoses. This tool, if adopted by clinicians and researchers in this field, is likely to enhance the reproducibility and comparability of existing and future research findings and uniformly expand the universal knowledge on dermoscopy in general dermatology. What's already known about this topic? Over the last few years, several papers have been published attempting to describe the dermoscopic features of non-neoplastic dermatoses, yet there is poor consistency in the terminology among different studies. What does this study add? The present expert consensus provides a set of standardized basic dermoscopic parameters to follow when evaluating inflammatory, infiltrative and infectious dermatoses. This consensus should enhance the reproducibility and comparability of existing and future research findings and uniformly expand the universal knowledge on dermoscopy in general dermatology.

Erosive pustular dermatosis of the scalp: a multicentre study.

BACKGROUND: Erosive pustular dermatosis of the scalp (EPDS) is characterized by crusted erosions or superficial ulcerations that lead to scarring alopecia. OBJECTIVES AND METHODS: We performed a multicentre retrospective clinical study including 56 patients (29 females and 27 males, mean age 62.7) with a confirmed EPDS in order to describe epidemiology, clinical findings and therapeutic choices of this disease. RESULTS: Mechanical/chemical trauma was reported in 28.6%, a previous infection in 10.7%, a previous cryotherapy in 5.4% androgenetic alopecia in 48.2% and severe actinic damage in 25%. Trichoscopy showed absence of follicular ostia, tufted and broken hair, crusts, serous exudate, dilated vessels, pustules and hyperkeratosis. Histopathology revealed three different features, depending on the disease duration. The most prescribed therapy was topical steroids (62.5%), followed by the combination of topical steroids and topical tacrolimus (8.9%), systemic steroids (7.1%) and topical tacrolimus (5.4%). A reduction of inflammatory signs was observed in 28 patients (50%) treated with topical steroids and in all three patients treated with topical tacrolimus. CONCLUSION: The relatively high number of patients collected allowed us to identify a better diagnostic approach, using trichoscopy and a more effective therapeutic strategy, with high-potency steroids or tacrolimus, which should be considered as first-line treatment.

Increased risk of severe course of pemphigus in patients with pemphigus-associated alopecia: a prospective observational study.

BACKGROUND: Pemphigus-associated alopecia is considered rare, and has not been studied in detail. AIM: To evaluate the clinical and immunological characteristics of patients with pemphigus-associated alopecia. METHODS: This prospective observational study included 80 consecutive patients with histopathologically and immunopathologically confirmed pemphigus, of whom 11 (13.8%) were found to have pemphigus-associated alopecia. Alopecia was observed in 11/52 patients with pemphigus and scalp involvement: [0/28 (35.7%) with pemphigus vulgaris and 1/24 (4.2%) with pemphigus foliaceus. The clinical and immunological characteristics of these patients were analysed. RESULTS: Patients with pemphigus-associated alopecia had a significantly higher Pemphigus Disease Area Index total activity score compared with patients who had no pemphigus-associated alopecia (21.8 ± 18.6 and 11.0 ± 20.5, respectively; P = 0.02). Mean serum anti-desmoglein (Dsg)1 antibody concentration was 141.8 ± 66.9 U/mL and 60.0 ± 52.6 U/mL, respectively (P = 0.03), and mean serum anti-Dsg3 concentration was 126.6 ± 36.7 U/mL and 67.4 ± 52.5 U/mL, respectively (P = 0.03). The values for achieving serological remission were 10% and 70%, respectively (P = 0.02). CONCLUSIONS: Pemphigus-associated alopecia is a marker of severe disease and a treatment-resistant disease course.

Dermoscopic features of psoriasis of the skin, scalp and nails - a systematic review.

Dermoscopy is a non-invasive in-office method, which enables the diagnosis of many dermatoses and reduces the need for performing biopsies. To date, no systematic review about the diagnostic usability of dermoscopy in psoriasis has been available. The objective of this article was to summarize and critically analyse literature data on the dermoscopy of skin, scalp and nail changes in psoriasis. A systematic search of three medical databases was performed. A total of 45 articles were included into the analysis. Cutaneous psoriatic lesions assessed in all studies at a low magnification showed regularly distributed red dots. At a 50-fold or higher magnification capillary bushes (glomerular vessels) with a diameter range of 50-146 µm were observed. The background colour was described as reddish or pinkish with white or yellowish scales. The most frequent dermoscopic (trichoscopic) feature of scalp psoriasis was the presence of red dots/globules and twisted red loops. Typical dermoscopic (onychoscopic) signs of nail psoriasis were onycholysis, salmon patches and splinter haemorrhages. There is an accumulating body of evidence that dermoscopy (both handheld and videodermoscopy) is a useful tool in differential diagnosis in doubtful cases of psoriasis of the skin, scalp, nails, palms, soles and genital regions.

Claudin-3 - a new intestinal integrity marker in patients with psoriasis: association with disease severity.

BACKGROUND: Gut dysbiosis and increased intestinal permeability play a significant role in the pathogenesis of psoriasis and its comorbidities. Claudin-3 is a key component of tight junctions, which may serve as marker of gut barrier integrity. OBJECTIVES: The aim of the study was to investigate circulating plasma claudin-3 in patients with psoriasis and to evaluate clinical and metabolic factors, which determine its concentration. METHODS: This cross-sectional study included 60 patients with psoriasis (39 men and 21 women, mean age: 45.6 ± 12.1 years) and 30 healthy controls (18 men and 12 women, mean age: 46.3 ± 15.5 years) age, sex and body mass index-matched. Plasma claudin-3 concentration was measured using an enzyme-linked immunosorbent assay. RESULTS: Plasma claudin-3 concentration was significantly higher in patients with psoriasis in comparison with healthy control [median (interquartile range), 50.7 ng/mL (47.3-54.2) vs. 43.3 ng/mL (42.3-44.2), P < 0.001]. Patients who achieved ΔPASI90 response after 16 weeks of treatment showed tendency to decrease in circulating claudin-3 plasma concentration. Positive correlations between claudin-3 concentration and the PASI score (r = 0.828; P < 0.001) as well as claudin-3 and neutrophil-to-lymphocyte ratio (r = 0.847; P < 0.001) were found. A multivariable linear regression analysis confirmed association of claudin-3 with the PASI score (P < 0.001), neutrophil-to-lymphocyte ratio (P < 0.01) and active smoking (P < 0.05). CONCLUSION: Claudin-3, a biomarker for gut permeability, is increased in psoriasis and correlates with disease severity and smoking. Further investigations are needed to determine whether reinforcing intestinal barrier may be a new therapeutic target in psoriasis.

The value of dermoscopy in diagnosing eyebrow loss in patients with alopecia areata and frontal fibrosing alopecia.

INTRODUCTION: Alopecia areata and frontal fibrosing alopecia are common causes of eyebrow loss (madarosis). OBJECTIVE: Assessment of trichoscopic markers of eyebrow loss in alopecia areata and frontal fibrosing alopecia. MATERIALS AND METHODS: The analysis included 50 patients with scalp alopecia areata with madarosis, 50 patients with scalp frontal fibrosing alopecia with madarosis and 50 healthy controls. In every case, trichoscopy of the eyebrow area was performed. RESULTS: Empty follicular and eccrine duct openings were observed in all patients and presented predominantly as yellow dots. Exclamation mark hairs were only detected in patients with alopecia areata (30%). Tapered hairs, broken hair, black dots and Pohl-Pinkus constrictions were observed in 14%, 36%, 26% and 4% of patients with alopecia areata, respectively, 4%, 16%, 2% and 0% of patients with frontal fibrosing alopecia, respectively, and they were not present in healthy controls. Dystrophic hairs and whitish areas were observed only in patients with frontal fibrosing alopecia (28% and 32%, respectively). Eyebrow regrowth in distinct directions was present in 32% of patients with frontal fibrosing alopecia, 8% of patients with alopecia areata and 4% of healthy controls. Diffuse erythema was detected in 60% of patients with alopecia areata and frontal fibrosing alopecia and 56% of healthy controls. Vellus hairs and upright regrowing hairs were observed in patients with alopecia areata (62% and 58%, respectively), frontal fibrosing alopecia (60% and 84%, respectively) and healthy controls (100% and 100%, respectively). CONCLUSION: Trichoscopy of the eyebrow area is useful in diagnosing patients with isolated eyebrow loss. The most characteristic trichoscopic features of eyebrow loss in alopecia areata include exclamation mark hairs, tapered hairs, broken hairs and black dots. Frontal fibrosing alopecia of the eyebrows is characterized by the presence of dystrophic hairs, white areas and eyebrow regrowth in distinct directions.

Dermoscopy of basal cell carcinoma.

Dermoscopy is widely used in dermatological practice. The method increases the accuracy of basal cell carcinoma (BCC) detection. Pigmented and nonpigmented variants of basal cell carcinoma present different dermoscopic features. Specific dermoscopy criteria have been recognized in different subtypes of BCC. Differentiation of superficial BCC from other subtypes is the most important issue, as it may determine further management decisions.

Efficacy of perilesional and intralesional triamcinolone acetonide injections in pemphigus vulgaris lesions of the scalp: an effective therapeutic option.

The scalp is a common location for pemphigus vulgaris (PV), and scalp lesions may be resistant to standard treatment. Perilesional/intralesional triamcinolone acetonide (TA) injections have been used successfully to treat oropharyngeal and ocular involvement in PV. Data on the efficacy of perilesional and intralesional triamcinolone acetonide injections in scalp lesions in PV are lacking. We report two patients with immunopathologically and histopathologically confirmed PV and residual scalp lesions resistant to standard treatment, who were treated with perilesional and intralesional injections of TA 10 mg/mL. Clearance of scalp lesions was achieved after one after, respectively, one and two perilesional and intralesional injections. Perilesional and intralesional TA injections may serve as an effective and safe treatment for recalcitrant scalp lesions in pemphigus.

What do we know about palmoplantar pustulosis?

Palmoplantar pustulosis is characterized by a chronic eruption of sterile pustules on palms and soles. The disease affects mainly women in the sixth and seventh decade of life. Some authors consider palmoplantar pustulosis a separate entity, whereas others consider it a condition in the spectrum of psoriasis. Aim of this study was to summarize the most recent data about PPP which aimed at establishing the nosological position of palmoplantar pustulosis. A systematic search of published literature was carried out. General characteristics of patients with PPP in different populations were present. We reviewed histological, immunological and genetic studies, as well as treatment options for PPP. PPP presents with clinical features, which are not present in psoriasis; however, the common coexistence of psoriasis vulgaris and/or positive family history for psoriasis indicates at least a close relationship between PPP and psoriasis. At present, there are not sufficient data to exclude PPP from psoriasis group.

European dermatology forum S1-guideline on the diagnosis and treatment of sclerosing diseases of the skin, Part 2: Scleromyxedema, scleredema and nephrogenic systemic fibrosis.

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 guideline focuses on characteristic clinical and histopathological features, diagnostic scores and the serum autoantibodies most useful for differential diagnosis. In addition, current strategies in the first- and advanced-line therapy of sclerosing skin diseases are addressed in detail. Part 2 of this guideline provides clinicians with an overview of the diagnosis and treatment of scleromyxedema, scleredema (of Buschke) and nephrogenic systemic sclerosis (nephrogenic fibrosing dermopathy).

European Dermatology Forum S1-guideline 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 guideline focuses on characteristic clinical and histopathological features, diagnostic scores and the serum autoantibodies most useful for differential diagnosis. In addition, current strategies in the first- and advanced-line therapy of sclerosing skin diseases are addressed in detail. Part 1 of this guideline provides clinicians with an overview of the diagnosis and treatment of localized scleroderma (morphea), and systemic sclerosis including overlap syndromes of systemic sclerosis with diseases of the rheumatological spectrum.