Androgenetic Alopecia in Children and Adolescents: From Trichoscopy to Therapy.

Introduction: Pediatric androgenetic alopecia is a product of hormonal and genetic factors. The diagnosis depends on recognizing the hair loss pattern in the context of a positive family history and a typical trichoscopy. Methods: A multicenter retrospective study assessing medical data from January 2008 to January 2023 of two reference centers - one public and one private in west Mexico. Patients under 18 years old were included. The clinical features, trichoscopic findings, associated conditions, and treatment received were documented and analyzed. Results: We found 145 patients, with a mean age of 16.08 ± 1.30 years, predominantly comprising males (72%). Trichoscopy was performed on 33 patients. The main trichoscopic findings were hair shaft thickness variability in 100% of the cases, vellus hair in 85%, and single-hair units in 79%. Vitamin D deficiency was found in 84% of the cases with laboratory determination, insulin resistance in 33%, and hyperandrogenemia in 12.5%. Topical minoxidil emerged as the main treatment modality in 24% of cases, demonstrating both efficacy and tolerability. Conclusion: Pediatric androgenetic alopecia could be more prevalent than commonly perceived, potentially explained by the lower level of suspicion among medical practitioners. Distinctive trichoscopic findings offer valuable guidance for therapeutic strategies and ongoing management.

Trichoscopy of Androgenetic Alopecia: A Systematic Review.

Background: Androgenetic alopecia, the most common cause of non-scarring hair loss, is a consequence of the gradual miniaturization of the hair follicles. In the majority of male androgenetic alopecia cases, a patient's history and clinical evaluation may be sufficient to establish the diagnosis, while for women, they should be supplemented with trichoscopy. Methods: The PubMed and Scopus databases were used to collate published studies and to analyze the most typical trichoscopic findings in patients diagnosed with androgenetic alopecia. A total of 34 articles were retrieved after exclusion. Results: The most common features identified using trichoscopy included hair diameter variability (94.07% of patients), vellus hairs (66.45%) and the peripilar sign (43.27%). Others, such as the honeycomb pattern, yellow and white dots, were less relevant. Conclusions: We concluded that hair diameter variability, vellus hairs and the peripilar sign represented valuable indicators for the diagnosis of androgenetic alopecia.

Efficacy and Safety of Rilzabrutinib in Pemphigus: PEGASUS Phase 3 Randomized Study.

TRIAL DESIGN: Pemphigus is a rare, but life-threatening autoimmune disease requiring long-term treatment that minimizes corticosteroid (CS) exposure while providing consistent disease control. The phase 2 pemphigus study of oral, reversible, covalent Bruton tyrosine kinase (BTK) inhibitor rilzabrutinib demonstrated rapid and sustained efficacy with well-tolerated safety. METHODS: Adults (18-80 y) were randomized 1:1 to rilzabrutinib 400 mg (n=65) or placebo (n=66) twice daily (with CS ≤0.5 mg/kg/d) for 37 weeks in the phase 3 PEGASUS study in moderate-to-severe pemphigus vulgaris/foliaceus (PV/PF). RESULTS: The primary endpoint of complete remission (CR) from week 29-37 with the amended endpoint CS dose ≤10 mg/d was not significant for 13/54 (24%) rilzabrutinib vs. 10/55 (18%) placebo patients with PV (P=0.45). Secondary endpoints showed numerical, but non-significant, improvements with rilzabrutinib (vs. placebo) in reduced CS use, prolonged CR duration, and faster time to first CR. CONCLUSIONS: Overall, rilzabrutinib was well-tolerated with similar adverse events reported in both groups. Using minimal CS dose ≤10 mg/d and excluding remote observations, the primary efficacy endpoint was not met. However, results from a prespecified sensitivity analysis using CS dose ≤5 mg/d, considering all observations, and including all patients support BTK inhibition as a viable therapeutic approach for pemphigus.

Consensus statement on the diagnosis and treatment of sclerosing diseases of the skin, Part 2: Scleromyxoedema and scleroedema.

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 2 of this consensus provides clinicians with an overview of the diagnosis and treatment of scleromyxoedema and scleroedema (of Buschke).

Integrated Safety Analysis of Ritlecitinib, an Oral JAK3/TEC Family Kinase Inhibitor, for the Treatment of Alopecia Areata from the ALLEGRO Clinical Trial Program.

BACKGROUND: The ALLEGRO phase 2a and 2b/3 studies demonstrated that ritlecitinib, an oral JAK3/TEC family kinase inhibitor, is efficacious at doses of ≥ 30 mg in patients aged ≥ 12 years with alopecia areata (AA). OBJECTIVE: The objective of this study was to evaluate the safety of ritlecitinib in an integrated analysis of four studies in AA. METHODS: Two cohorts were analyzed: a placebo-controlled and an all-exposure cohort. Proportions and study size-adjusted incidence rates (IRs) of adverse events (AEs) of interest and laboratory abnormalities are reported. RESULTS: In the placebo-controlled cohort (n = 881; median exposure: 169 days), the proportion of ritlecitinib-treated patients with AEs was 70.2-75.4% across doses versus 69.5% in the placebo group; serious AEs occurred in 0-3.2% versus 1.9% for the placebo. A total of 19 patients permanently discontinued due to AEs (5 while receiving the placebo). In the all-exposure cohort (n = 1294), median ritlecitinib exposure was 624 days [2091.7 total patient-years (PY)]. AEs were reported in 1094 patients (84.5%) and serious AEs in 57 (4.4%); 78 (6.0%) permanently discontinued due to AEs. The most common AEs were headache (17.7%; 11.9/100 PY), severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) positive test (15.5%; 9.8/100 PY), and nasopharyngitis (12.4%; 8.2/100 PY). There were two deaths (breast cancer and acute respiratory failure/cardiorespiratory arrest). Proportions (IRs) were < 0.1% (0.05/100 PY) for opportunistic infections, 1.5% (0.9/100 PY) for herpes zoster, 0.5% (0.3/100 PY) for malignancies (excluding nonmelanoma skin cancer), and 0.2% (0.1/100 PY) for major adverse cardiovascular events. CONCLUSIONS: Ritlecitinib is well tolerated with an acceptable safety profile up to 24 months in patients aged ≥ 12 years with AA (video abstract and graphical plain language summary available). TRIAL REGISTRIES: ClinicalTrials.gov: NCT02974868 (date of registration: 11/29/2016), NCT04517864 (08/18/2020), NCT03732807 (11/07/2018), and NCT04006457 (07/05/2019).

Trichoscopy - a valuable tool for identifying conditions mimicking androgenetic alopecia.

Androgenetic alopecia (AGA) is the most prevalent type of hair loss in women and men. Recently, a European consensus group published guidelines for the diagnostic evaluation of AGA in men, women, and adolescents. This S1 guideline presents expert opinion-based recommendations for gender-dependent steps in the diagnostic procedure, which can easily be implemented in the daily clinical routine. For diagnosing AGA, detailed anamnesis and objective learning are not enough because there are several conditions mimicking this disease. Trichoscopy can be considered an important, non-invasive tool for diagnosing hair and scalp disorders that may have similar clinical signs to AGA.

Autoinflammatory Keratinization Diseases-The Concept, Pathophysiology, and Clinical Implications.

Recent advances in medical genetics elucidated the background of diseases characterized by superficial dermal and epidermal inflammation with resultant aberrant keratosis. This led to introducing the term autoinflammatory keratinization diseases encompassing entities in which monogenic mutations cause spontaneous activation of the innate immunity and subsequent disruption of the keratinization process. Originally, autoinflammatory keratinization diseases were attributed to pathogenic variants of CARD14 (generalized pustular psoriasis with concomitant psoriasis vulgaris, palmoplantar pustulosis, type V pityriasis rubra pilaris), IL36RN (generalized pustular psoriasis without concomitant psoriasis vulgaris, impetigo herpetiformis, acrodermatitis continua of Hallopeau), NLRP1 (familial forms of keratosis lichenoides chronica), and genes of the mevalonate pathway, i.e., MVK, PMVK, MVD, and FDPS (porokeratosis). Since then, endotypes underlying novel entities matching the concept of autoinflammatory keratinization diseases have been discovered (mutations of JAK1, POMP, and EGFR). This review describes the concept and pathophysiology of autoinflammatory keratinization diseases and outlines the characteristic clinical features of the associated entities. Furthermore, a novel term for NLRP1-associated autoinflammatory disease with epithelial dyskeratosis (NADED) describing the spectrum of autoinflammatory keratinization diseases secondary to NLRP1 mutations is proposed.

Dermoscopic Characteristics of Cutaneous Larva Migrans in the Dark Skin: A Study from Banjul, The Gambia.

Cutaneous larva migrans (CLM), results from hookworm larvae infestation, mainly A. braziliense or A. caninum. It is common in Sub-Saharan Africa, often acquired through soil contact, especially in sandy beaches, manifesting as serpiginous, erythematous, and intensely pruritic tracts within the epidermis and presenting with diverse clinical appearances. Diagnosis is mostly clinical, however, dermoscopy can enhance diagnostic accuracy and distinction from mimics. The current body of literature is deficient in its representation of dermoscopic data for CLM in blacks. This study explores dermoscopy in 9 dark-skinned patients with 16 CLM lesions. Distinctive serpiginous structureless areas displaying a range of colors, peripheral scales surrounding brown areas and brown dots were predominant features, whereas vascular characteristics were less evident. This article highlights the presence of distinct reaction patterns, including brown dots, scales, and accentuated, often disrupted brown reticular lines in addition to the characteristic winding tracts in the darker skin.

Differential diagnosis of red scalp. The importance of trichoscopy.

Red scalp is a common complaint which may constitute a diagnostic and therapeutic challenge in daily clinical practice. Among the numerous diseases which cause diffuse scalp erythema are psoriasis, seborrheic dermatitis, contact dermatitis, diffuse lichen planopilaris, dermatomyositis and scalp rosacea. Accurate diagnosis is crucial for optimal treatment outcomes. Histology most frequently discriminates the underlying condition, but it requires scalp biopsy. In many cases the combination of clinical examination and trichoscopy is sufficient for establishing the correct diagnosis. The main trichoscopic features of psoriasis are silver-white scaling, regular distributed dotted (glomerular) vessels or twisted red loops and punctate hemorrhages. Yellowish-white scaling and thin arborizing vessels are typical features of seborrheic dermatitis. Contact dermatitis is characterized by the presence of yellow exudate and polymorphic vessels, while perifollicular scaling and erythema with the lack of follicular openings are typical findings in lichen planopilaris. In scalp dermatomyositis, tortuous and arborizing vessels with interfollicular and perifollicular pigmentation may be detected. The most characteristic features of scalp rosacea are perifollicular scaling and arborizing vessels. This review also summarizes histologic features and therapeutic options for these conditions.

Dermoscopy as a Noninvasive Diagnostic Tool for Hailey-Hailey Disease and Darier Disease.

INTRODUCTION: Hailey-Hailey disease (HHD) and Darier disease (DD) are rare genetic disorders for which differential diagnosis, especially in less obvious cases, can be difficult. The diagnosis is based on the clinical picture and family history, and is confirmed by histopathologic examination. Dermoscopy is a noninvasive technique that is primarily used at the present time to diagnose skin cancers. However, in the past few years this technique has also been increasingly used as a noninvasive diagnostic tool of inflammatory skin diseases. The aim of the study was to evaluate whether dermoscopy is a useful noninvasive diagnostic tool for HHD and DD. METHODS: We performed an observational retrospective case series study involving 13 patients with HHD (n = 8) and DD (n = 5). The presence or absence of standardized dermoscopic features of inflammatory diseases (according to International Dermoscopy Society [IDS] guidelines) was assessed in these patients. RESULTS: The most distinctive feature of HHD was white clouds separated by pink furrows, visible in all cases (8/8; 100.0%). Another distinctive clue of HHD was the crumbled fabric pattern seen in six patients with HHD (6/8; 75.0%). These dermoscopic findings were not present in patients with DD. The most typical features of DD in the dermoscopic examination was star-like or oval-shaped yellow areas surrounded by whitish halo, visible in all patients (5/5; 100.0%). Another distinctive dermoscopic clue of DD was pinkish homogeneous structureless background, which was present in all patients (5/5, 100.0%). These latter two features were not observed in patients with HHD. CONCLUSION: Dermoscopy reveals distinctive features of HHD and DD, respectively. Therefore, we conclude that dermoscopy can be an excellent complementary noninvasive tool in the diagnostic process of patients with HHD and DD.

Hailey-Hailey disease and Darier disease are rare genetic disorders, which are diagnosed based on the clinical picture and confirmed with skin biopsy. Dermoscopy is noninvasive diagnostic tool, which enables skin visualization at a 10-fold magnification. Currently, dermoscopy is mainly used to diagnose skin cancers. In the recent years, dermoscopy has been also increasingly used as a noninvasive diagnostic tool of inflammatory skin diseases. The aim of the study was to assess whether demoscopy may be a useful tool in diagnosing Hailey-Hailey disease and Darier disease. The study included thirteen patients: eight with Hailey-Hailey disease and five with Darier disease. The most typical dermoscopic feature of Hailey-Hailey disease was white clouds separated by pink furrows, which were visible in all cases. Another distinctive clue was crumbled fabric pattern seen in 75.0% of patients with Hailey-Hailey disease. These dermoscopic findings were not present in patients with Darier disease. In dermoscopic examination the most typical feature of Darier disease was star-like or oval-shaped yellow areas surrounded by whitish halo, visible in all patients. Also, pinkish homogeneous structureless background was present in all patients with Darier disease. These features were not observed in patients with Hailey-Hailey disease. Dermoscopy reveals characteristic features of Hailey-Hailey disease and Darier disease. Therefore, it can be an excellent complementary tool in the diagnostic process of patients with those diseases.

Topical proactive therapy in dermatology. A scoping review.

The term 'proactive therapy' refers to a long-term management of clinically intact skin in previously disease-affected areas. This method was initially implemented in atopic dermatitis to maintain the remission and decrease the risk of exacerbations. Proactive therapy aims to limit the need for reactive treatment and improve the patients' quality of life. A proactive approach is likely to be adopted for other relapsing and inflammatory skin conditions in the future. This scoping review aims to identify dermatological conditions to be treated with the proactive approach, evaluate the available evidence for its efficacy and safety, as well as highlight the research gaps.

The profile of adipokines associated with fibrosis and impaired microcirculation in systemic sclerosis.

PURPOSE: Adipokines belong to a group of molecules mostly produced by adipose tissue. Abnormalities in the secretion of several adipokines have already implicated to play a pathogenic role in systemic sclerosis (SSc). However, the possible role of numerous molecules still needs to be clarified. The aim of the study was to determine whether the altered level of selected circulating adipokines might correlate with the intensity of fibrosis and vasculopathy in the course of SSc. MATERIALS AND METHODS: Serum concentrations of chemerin, adipsin, retinol-binding protein 4, apelin, visfatin, omentin-1, and vaspin were determined with ELISA in the sera of patients with SSc (n â€‹= â€‹55) and healthy controls (n â€‹= â€‹25). RESULTS: The serum concentration of adipsin (p â€‹= â€‹0.03) and visfatin (p â€‹= â€‹0.04) was significantly increased and the level of retinol-binding protein 4 (p â€‹= â€‹0.03) was decreased in diffuse compared to limited cutaneous SSc. Moreover, serum adipsin level correlated positively with the intensity of skin fibrosis measured with the modified Rodnan skin score (r â€‹= â€‹0.31, p â€‹= â€‹0.02) and was significantly higher in patients with pulmonary arterial hypertension than in those without the condition (p â€‹= â€‹0.03). The concentrations of adipsin (p â€‹= â€‹0.01) and visfatin (p â€‹= â€‹0.04) were significantly increased and the level of apelin (p â€‹= â€‹0.02) was decreased in patients with active digital ulcerations compared to individuals without this complication. CONCLUSION: Adipsin may be considered a pivotal protein in the development of both fibrosis and impaired microcirculation. Its abnormal concentration reflects the intensity of skin thickening and the presence of pulmonary arterial hypertension. Adipsin, visfatin, and apelin are adipose tissue-derived molecules associated with digital vasculopathy.

Ocular involvement in autoimmune bullous diseases.

Autoimmune bullous diseases represent a heterogenous group of disorders caused by autoantibodies against adhesion molecules; the location of the target protein determines the level of cleft formation. The spectrum of ocular lesions in autoimmune bullous diseases can range from mild symptoms to severe involvement with sight impairment and even, in some cases, blindness. In pemphigus vulgaris, the prevalence of ocular involvement has been reported to be between 7% and 26%. The most common clinical sign of ocular pemphigus vulgaris is bilateral conjunctivitis with hyperemia. Ocular involvement also occurs in 41% to 70% of patients with paraneoplastic pemphigus. The main ocular manifestations are bilateral cicatrizing conjunctivitis with symblepharon formation, and shortening of the fornices. In mucous membrane pemphigoid, ocular involvement is seen in 61% to 70% of patients; the most frequent ocular finding is cicatricial conjunctivitis. Patients with autoimmune bullous diseases having common ocular involvement should be assessed by an ophthalmologist to avoid serious complications. Diagnostic procedures and treatment require multidisciplinary care based on the close cooperation between dermatologists and ophthalmologists.

Multilocus-sequence typing reveals clonality of Staphylococcus aureus in atopic dermatitis.

BACKGROUND: Atopic dermatitis (AD) is exacerbated by Staphylococcus aureus, which is capable of displacing not only the physiological microbiota, but also other strains of its own species. Analyses of the molecular characteristics and relationships of S. aureus strains present in different microniches are lacking. OBJECTIVES: To determine, using multilocus sequence typing (MLST), the relationship of S. aureus isolates from the lesional and nonlesional skin and anterior nares of patients with AD, and to review the characteristics of the dominant clones. METHODS: Sixty-three individuals with active AD were enrolled. Ten patients with moderate-to-severe AD (SCoring of Atopic Dermatitis score ≥ 25) colonized by S. aureus in all analysed locations were included in the MLST analysis. RESULTS: The most prevalent sequence types were 7 (10/30 strains; 33.3%), 15 and 97 (both 5/30 strains; 16.7%) all of which were associated with the expression of adhesins and toxins promoting chronic microbial dysbiosis, skin barrier damage and inflammation. Six patients (60%) were carriers of clonal S. aureus strains at all analysed locations, three (30%) carriers in lesional and nonlesional skin, and one (10%) was a carrier in nonlesional skin and the anterior nares. CONCLUSIONS: The results imply that the identified S. aureus lineages are better adapted to dominate the microbiota in AD. Decontaminating the identified reservoirs of S. aureus (i.e. anterior nares and nonlesional skin) could reduce the severity of AD.