Louis Armstrong's Lip Problems: Satchmo's Syndrome Reviewed.

Rupture of the orbicularis muscle of the lips in wind musicians is known as Satchmo's syndrome because it is assumed that Louis Armstrong (nicknamed Satchmo) suffered this condition. But whether Louis Armstrong really had this problem or not has never been documented. AIMS: This study is a biographical review and an analysis of the existing photographs of Armstrong's lip with the aim of better understanding the origin and progression of his disorder. METHODS: A review was made of the most important biographies, newspapers, and magazines referring to Louis Armstrong and medical reports about Satchmo's syndrome. Major archives related to Louis Armstrong were searched for documents, photographs, articles, videos, letters, and other items, using the terms "lip," "embouchure," "mouth," and "problem." Information was sought on Armstrong's playing technique, playing schedule, lip changes, lip problems, and other related health issues. RESULTS: It was found that Louis Armstrong suffered dermatological lip problems due to his intense playing schedule and the way he played from the very beginning of his career. This first created swelling and superficial erosions that later became cracks and fissures. These lesions finally produced ulcers which evolved into scar tissue after healing. This made the mucosa thicker and stiffer, making it more difficult for the lip to vibrate and forcing him to increase the tension and pressure in the area in order to play the trumpet. This created a vicious cycle with the scar tissue leading to further problems. These injuries even bled several times while he was playing and, on some occasions, left his mouth and clothes bloodied in the middle of concerts. His biography does not make any reference to difficulties or symptoms that could indicate that he had orbicularis oris muscle rupture, except for the fact that he had trouble playing, a symptom common to any injury to the lips in a wind instrument player. CONCLUSION: Analysis of the existing data indicates that Louis Armstrong's lip problems were most likely due to mucosa fibromatous hyperplasia resulting from chronic microtrauma rather than a rupture of the orbicularis oris muscle. Therefore, we propose that this disorder should no longer be referred to as Satchmo's syndrome.

Mini pulse corticosteroid therapy with oral dexamethasone for moderate to severe alopecia areata: A multicentric study.

Extensive subtypes of alopecia areata (AA) (totalis, universalis, or multifocal) still have no approved and effective treatments in Europe, although Janus kinase inhibitors, such as baricitinib, are promising treatments that have been recently approved by the FDA. Nowadays, the higher costs and the lower experience with Janus kinase inhibitors, provide more difficulties in its accessibility. On the other hand, different corticosteroids regimens have been evaluated with conflicting results from decades. In 2016, a new regimen of mini pulse corticosteroid therapy with oral dexamethasone (MPCT-OD) 0.1mg/kg/day twice per week for adult patients with alopecia areata totalis or universalis, was reported to be effective with a lower rate of adverse effects. We performed a retrospective and multicentric study to collect data from patients with extensive forms of alopecia areata who had received MPCTOD (0.1 mg/kg/day twice weekly of dexamethasone) for at least 24 weeks. We included adult patients (≥18 years) with extensive forms of AA (SALT index ≥ 10) that did not respond to previous treatments. Variables including epidemiological and clinical data were recorded. Therapeutic response was assessed through the % change in SALT score (from 0 to 100%) and the changes in eyebrow and eyelash alopecia index (EBA, ELA) from baseline to 24 weeks after the beginning of the treatment. Dexamethasone dosage, duration of the treatment, time until response, time to relapse, adverse effects, and discontinuation were also recorded.

The Alopecia Areata Consensus of Experts (ACE) study part II: Results of an international expert opinion on diagnosis and laboratory evaluation for alopecia areata.

BACKGROUND: We previously reported the Alopecia Areata Consensus of Experts study, which presented results of an international expert opinion on treatments for alopecia areata. OBJECTIVE: To report the results of the Alopecia Areata Consensus of Experts international expert opinion on diagnosis and laboratory evaluation for alopecia areata. METHODS: Fifty hair experts from 5 continents were invited to participate in a 3-round Delphi process. Consensus threshold was set at greater than or equal to 66%. RESULTS: Of 148 questions, expert consensus was achieved in 82 (55%). Round 1 consensus was achieved in 10 of 148 questions (7%). Round 2 achieved consensus in 47 of 77 questions (61%). The final face-to-face achieved consensus in 25 of 32 questions (78%). Consensus was greatest for laboratory evaluation (12 of 14 questions [86%]), followed by diagnosis (11 of 14 questions [79%]) of alopecia areata. Overall, etiopathogenesis achieved the least category consensus (31 of 68 questions [46%]). LIMITATIONS: The study had low representation from Africa, South America, and Asia. CONCLUSION: There is expert consensus on aspects of epidemiology, etiopathogenesis, clinical features, diagnosis, laboratory evaluation, and prognostic indicators of alopecia areata. The study also highlights areas where future clinical research could be directed to address unresolved hypotheses in alopecia areata patient care.

The Alopecia Areata Consensus of Experts (ACE) study: Results of an international expert opinion on treatments for alopecia areata.

BACKGROUND: A systematic review failed to identify any systemic therapy used in alopecia areata (AA) where use is supported by robust evidence from high-quality randomized controlled trials. OBJECTIVE: To produce an international consensus statement on the use and utility of various treatments for AA. METHODS: Fifty hair experts from 5 continents were invited to participate in a 3-round Delphi process. Agreement of 66% or greater was considered consensus. RESULTS: In the first round, consensus was achieved in 22 of 423 (5%) questions. After a face-to-face meeting in round 3, overall, consensus was achieved for only 130 (33%) treatment-specific questions. There was greater consensus for intralesional treatment of AA (19 [68%]) followed by topical treatment (25 [43%]). Consensus was achieved in 45 (36%) questions pertaining to systemic therapies in AA. The categories with the least consensus were phototherapy and nonprescription therapies. LIMITATIONS: The study included a comprehensive list of systemic treatments for AA but not all treatments used. CONCLUSION: Despite divergent opinions among experts, consensus was achieved on a number of pertinent questions. The concluding statement also highlights areas where expert consensus is lacking and where an international patient registry could enable further research.

Ozenoxacin, a New Effective and Safe Topical Treatment for Impetigo in Children and Adolescents.

BACKGROUND: Ozenoxacin is a topical antibiotic approved in Europe to treat non-bullous impetigo in adults and children aged ≥6 months. This analysis evaluated the efficacy and safety of ozenoxacin in paediatric patients by age group. METHODS: Pooled data for patients aged 6 months to <18 years who had participated in a phase I or in two phase III clinical trials of ozenoxacin 1% cream were analysed by age group: 0.5-<2, 2-<6, 6-<12, and 12-<18 years. RESULTS: The combined population comprised 529 patients with non-bullous impetigo treated with ozenoxacin (n = 239), vehicle (n = 201), or retapamulin as internal validation control (n = 89). Studies were well matched for extent and severity of impetigo and therapeutic schedule (twice daily application for 5 days). The clinical success rate after 5 days' treatment (day 6-7, end of therapy), and microbiological success rates after 3-4 days' treatment and at the end of therapy, were significantly higher with ozenoxacin than vehicle (p < 0.0001 for all comparisons). Clinical and bacterial eradication rates were higher with ozenoxacin than vehicle in each age group. No safety concerns were identified with ozenoxacin. One (0.3%) of 327 plasma samples exceeded the lower limit of quantification for ozenoxacin, but the low concentration indicated negligible systemic absorption. CONCLUSION: This combined analysis supports the efficacy and safety of ozenoxacin administered twice daily for 5 days. Ozenoxacin 1% cream is a new option to consider for treatment of non-bullous impetigo in children aged 6 months to <18 years.

Mutations in Three Genes Encoding Proteins Involved in Hair Shaft Formation Cause Uncombable Hair Syndrome.

Uncombable hair syndrome (UHS), also known as "spun glass hair syndrome," "pili trianguli et canaliculi," or "cheveux incoiffables" is a rare anomaly of the hair shaft that occurs in children and improves with age. UHS is characterized by dry, frizzy, spangly, and often fair hair that is resistant to being combed flat. Until now, both simplex and familial UHS-affected case subjects with autosomal-dominant as well as -recessive inheritance have been reported. However, none of these case subjects were linked to a molecular genetic cause. Here, we report the identification of UHS-causative mutations located in the three genes PADI3 (peptidylarginine deiminase 3), TGM3 (transglutaminase 3), and TCHH (trichohyalin) in a total of 11 children. All of these individuals carry homozygous or compound heterozygous mutations in one of these three genes, indicating an autosomal-recessive inheritance pattern in the majority of UHS case subjects. The two enzymes PADI3 and TGM3, responsible for posttranslational protein modifications, and their target structural protein TCHH are all involved in hair shaft formation. Elucidation of the molecular outcomes of the disease-causing mutations by cell culture experiments and tridimensional protein models demonstrated clear differences in the structural organization and activity of mutant and wild-type proteins. Scanning electron microscopy observations revealed morphological alterations in hair coat of Padi3 knockout mice. All together, these findings elucidate the molecular genetic causes of UHS and shed light on its pathophysiology and hair physiology in general.

Folliculitis decalvans: Effectiveness of therapies and prognostic factors in a multicenter series of 60 patients with long-term follow-up.

BACKGROUND: Folliculitis decalvans (FD) is a rare neutrophilic cicatricial alopecia that poses a therapeutic challenge. OBJECTIVES: To describe the therapeutic response in a large number of cases of FD with long-term follow-up and analyze potential prognostic factors associated with severity of form and with a better therapeutic response. METHODS: This multicenter prospective study included patients with FD who had a minimum of 5 years of follow-up. Severity was assessed by the maximum diameter of the cicatricial area. Therapeutic response was evaluated according to stabilization of the size of the cicatricial areas and the improvement in clinical symptoms. RESULTS: A total of 60 patients (37 men [61.7%] and 23 women [38.3%]) with a mean age of 40 years were included. Earlier age of onset (P = .01) was statistically associated with severity of form. Treatment with rifampicin and clindamycin, tetracyclines, and intralesional steroids was the most effective. No statistically significant prognostic factors predicting a better therapeutic response were found. LIMITATIONS: Because FD is a rare disease, the main limitation was the sample size. CONCLUSIONS: An earlier age of onset was associated with the severe form of the disease. The proposed specific therapeutic protocol can be a very useful tool in clinical dermatologic practice.

Topical Antibacterial Agent for Treatment of Adult and Pediatric Patients With Impetigo: Pooled Analysis of Phase 3 Clinical Trials.

Ozenoxacin is a novel topical antibacterial agent with potent bactericidal activity against Gram-positive bacteria that has been developed as a 1% cream for treatment of impetigo. This article presents pooled results of pivotal clinical trials of ozenoxacin with the objective of evaluating the efficacy, safety, and tolerability of ozenoxacin 1% cream after twice-daily topical treatment for 5 days in patients with impetigo. A pooled analysis was performed of individual patient data from two multicenter, randomized, double-blind, vehicle-controlled phase 3 registration studies conducted in patients with impetigo. Both clinical trials followed a similar methodology. Patients were randomized 1:1 to ozenoxacin or vehicle. One trial included retapamulin as an internal control. Efficacy was measured using the Skin Infection Rating Scale and microbiological culture. Safety and tolerability were evaluated. Ozenoxacin demonstrated superior clinical success versus vehicle after 5 days of therapy, superior microbiological success versus vehicle after 2 days of therapy, and was safe and well-tolerated. Ozenoxacin showed superior clinical and microbiological response versus vehicle in children as young as 2 months of age, and adults, with impetigo. Clinical Trial Registration: ClinicalTrials.gov identifier: NCT01397461 and NCT02090764; European Clinical Trials Database Number: 2011-003032-31 and 2014-000228-52. J Drugs Dermatol. 2018;17(10):1051-1057.

Randomized Placebo-Controlled, Double-Blind, Half-Head Study to Assess the Efficacy of Platelet-Rich Plasma on the Treatment of Androgenetic Alopecia.

BACKGROUND: Platelet-rich plasma (PRP) was identified as having a beneficial effect in alopecia and has been postulated as a new therapy for androgenetic alopecia (AGA). OBJECTIVE: To assess the efficacy of PRP for the treatment of AGA. MATERIALS AND METHODS: This was a randomized, placebo-controlled, double-blind study in 25 patients with AGA. Platelet-rich plasma was injected in half-head and the other half-head with placebo. Each patient received a total of 3 treatments of PRP, 1 month apart. RESULTS: Six months after the first treatment with PRP, significant differences were seen in mean anagen hairs (67.6 ± 13.1), telogen hairs (32.4 ± 13.1), hair density (179.9 ± 62.7), and terminal hair density (165.8 ± 56.8) when compared with baseline (p < .05). Platelet-rich plasma was also found to increase hair density when comparing with the control side (p < .05). For the first time, the authors found a correlation between anagen hairs and patients >40 years and beginning of AGA ≥25 years old (p < .05) and hair density and male sex, age ≤40 years, positive family history of AGA and >10 years of duration of the disease (p < 0.05). CONCLUSION: Application of PRP showed a positive effect on AGA and could be regarded as an adjuvant therapy for AGA.

EAACI position paper for practical patch testing in allergic contact dermatitis in children.

INTRODUCTION: Allergic contact dermatitis (ACD) in children appears to be on the increase, and contact sensitization may already begin in infancy. The diagnosis of contact dermatitis requires a careful evaluation of a patient's clinical history, physical examination, and skin testing. Patch testing is the gold standard diagnostic test. METHODS: Based on consensus, the EAACI Task Force on Allergic Contact Dermatitis in Children produced this document to provide details on clinical aspects, the standardization of patch test methodology, and suggestions for future research in the field. RESULTS: We provide a baseline list of test allergens to be tested in children with suspected ACD. Additional tests should be performed only on specific indications.

Frontal fibrosing alopecia: a multicenter review of 355 patients.

BACKGROUND: To our knowledge, there are no large multicenter studies concerning frontal fibrosing alopecia (FFA) that could give clues about its pathogenesis and best treatment. OBJECTIVE: We sought to describe the epidemiology, comorbidities, clinical presentation, diagnostic findings, and therapeutic choices in a large series of patients with FFA. METHODS: This retrospective multicenter study included patients given the diagnosis of FFA. Clinical severity was classified based on the recession of the frontotemporal hairline. RESULTS: In all, 355 patients (343 women [49 premenopausal] and 12 men) with a mean age of 61 years (range 23-86) were included. Early menopause was detected in 49 patients (14%), whereas 46 (13%) had undergone hysterectomy. Severe FFA was observed in 131 patients (37%). Independent factors associated with severe FFA after multivariate analysis were: eyelash loss, facial papules, and body hair involvement. Eyebrow loss as the initial clinical presentation was associated with mild forms. Antiandrogens such as finasteride and dutasteride were used in 111 patients (31%), with improvement in 52 (47%) and stabilization in 59 (53%). LIMITATIONS: The retrospective design is a limitation. CONCLUSIONS: Eyelash loss, facial papules, and body hair involvement were associated with severe FFA. Antiandrogens were the most useful treatment.

Alopecia and hypotrichosis as characteristic findings in Woodhouse-Sakati syndrome: report of a family with mutation in the C2orf37 gene.

Woodhouse-Sakati syndrome (WSS) is a rare autosomal recessive disorder characterized by alopecia, hypogonadism, diabetes mellitus, intellectual disability, sensorineural deafness, extrapyramidal signs, and low insulinlike growth factor 1 levels. Inter- and intrafamilial phenotypic variability have been reported. Mutations in the C2orf37 gene cause WSS. The present report describes the clinical signs and symptoms of three affected siblings from a consanguineous Bedouin family from Kuwait. Direct sequencing of the C2orf37 gene revealed that the c.436delC (p.Ala147Hisfs*9) mutation was present in a homozygous state in all affected siblings and in a heterozygous state in the parents and a healthy sister. Nine C2orf37 mutations causing WSS have been identified. This family shared the mutation reported earlier in Saudi families and families of Bedouin tribes from Qatar and Israel. No phenotypic or genotypic correlation has been observed. Despite the great phenotypic variability of WSS, hypotrichosis has been observed in all individuals with WSS reported. This condition has not been reported in the dermatologic literature. WSS should be included in the differential diagnosis of syndromic congenital hypotrichosis.

The Alopecia Areata Severity and Morbidity Index (ASAMI) Study: Results From a Global Expert Consensus Exercise on Determinants of Alopecia Areata Severity.

Importance: Current measures of alopecia areata (AA) severity, such as the Severity of Alopecia Tool score, do not adequately capture overall disease impact. Objective: To explore factors associated with AA severity beyond scalp hair loss, and to support the development of the Alopecia Areata Severity and Morbidity Index (ASAMI). Evidence Review: A total of 74 hair and scalp disorder specialists from multiple continents were invited to participate in an eDelphi project consisting of 3 survey rounds. The first 2 sessions took place via a text-based web application following the Delphi study design. The final round took place virtually among participants via video conferencing software on April 30, 2022. Findings: Of all invited experts, 64 completed the first survey round (global representation: Africa [4.7%], Asia [9.4%], Australia [14.1%], Europe [43.8%], North America [23.4%], and South America [4.7%]; health care setting: public [20.3%], private [28.1%], and both [51.6%]). A total of 58 specialists completed the second round, and 42 participated in the final video conference meeting. Overall, consensus was achieved in 96 of 107 questions. Several factors, independent of the Severity of Alopecia Tool score, were identified as potentially worsening AA severity outcomes. These factors included a disease duration of 12 months or more, 3 or more relapses, inadequate response to topical or systemic treatments, rapid disease progression, difficulty in cosmetically concealing hair loss, facial hair involvement (eyebrows, eyelashes, and/or beard), nail involvement, impaired quality of life, and a history of anxiety, depression, or suicidal ideation due to or exacerbated by AA. Consensus was reached that the Alopecia Areata Investigator Global Assessment scale adequately classified the severity of scalp hair loss. Conclusions and Relevance: This eDelphi survey study, with consensus among global experts, identified various determinants of AA severity, encompassing not only scalp hair loss but also other outcomes. These findings are expected to facilitate the development of a multicomponent severity tool that endeavors to competently measure disease impact. The findings are also anticipated to aid in identifying candidates for current and emerging systemic treatments. Future research must incorporate the perspectives of patients and the public to assign weight to the domains recognized in this project as associated with AA severity.

Allergic contact dermatitis in children: which factors are relevant? (review of the literature).

Allergic contact dermatitis (ACD) in children is increasing. Sensitization to contact allergens can start in early infancy. The epidermal barrier is crucial for the development of sensitization and elicitation of ACD. Factors that may influence the onset of sensitization in children are atopic dermatitis, skin barrier defects and intense or repetitive contact with allergens. Topical treatment of ACD is associated with cutaneous sensitization, although the prevalence is not high. ACD because of haptens in shoes or shin guards should be considered in cases of persistent foot eruptions or sharply defined dermatitis on the lower legs. Clinical polymorphism of contact dermatitis to clothing may cause difficulties in diagnosing textile dermatitis. Toys are another potentially source of hapten exposure in children, especially from toy-cosmetic products such as perfumes, lipstick and eye shadow. The most frequent contact allergens in children are metals, fragrances, preservatives, neomycin, rubber chemicals and more recently also colourings. It is very important to remember that ACD in young children is not rare, and should always be considered when children with recalcitrant eczema are encountered. Children should be patch-tested with a selection of allergens having the highest proportion of positive, relevant patch test reactions. The allergen exposure pattern differs between age groups and adolescents may also be exposed to occupational allergens. The purpose of this review is to alert the paediatrician and dermatologist of the frequency of ACD in young children and of the importance of performing patch tests in every case of chronic recurrent or therapy-resistant eczema in children.