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Linked Article: Greenblatt et al. Br J Dermatol 2022; 186:620–632. [ABSTRACT FROM AUTHOR]

The Krafla geothermal system is located in Iceland's northeastern neovolcanic zone, within the Krafla central volcanic complex. Geothermal fluids are superheated steam closest to the magma heat source, two-phase at higher depths, and sub-boiling at the shallowest depths. Hydrogen isotope ratios of geothermal fluids range from −87‰, equivalent to local meteoric water, to −94‰. These fluids are enriched in 18O relative to the global meteoric line by +0.5-3.2‰. Calculated vapor fractions of the fluids are 0.0-0.5 wt% (~0-16% by volume) in the northwestern portion of the geothermal system and increase towards the southeast, up to 5.4 wt% (~57% by volume). Hydrothermal epidote sampled from 900 to 2500 m depth has δD values from −127 to −108‰, and δ18O from −13.0 to −9.6‰. Fluids in equilibrium with epidote have isotope compositions similar to those calculated for the vapor phase of two-phase aquifer fluids. We interpret the large range in δ DEPIDOTE and δ18 OEPIDOTE across the system and within individual wells (up to 7‰ and 3.3‰, respectively) to result from variable mixing of shallow sub-boiling groundwater with condensates of vapor rising from a deeper two-phase reservoir. The data suggest that meteoric waters derived from a single source in the northwest are separated into the shallow sub-boiling reservoir, and deeper two-phase reservoir. Interaction between these reservoirs occurs by channelized vertical flow of vapor along fractures, and input of magmatic volatiles further alters fluid chemistry in some wells. Isotopic compositions of hydrothermal epidote reflect local equilibrium with fluids formed by mixtures of shallow water, deep vapor condensates, and magmatic volatiles, whose ionic strength is subsequently derived from dissolution of basalt host rock. This study illustrates the benefits of combining phase segregation effects in two-phase systems during analysis of wellhead fluid data with stable isotope values of hydrous alteration minerals when evaluating the complex hydrogeology of volcano-hosted geothermal systems. [ABSTRACT FROM AUTHOR]

Given its improved safety profile compared with systemic corticosteroids, propranolol has become the mainstay treatment of infantile haemangioma ( IH) worldwide. There is evidence, mainly from adult volunteer studies, that propranolol use is associated with central nervous system ( CNS) effects. Impairment to short- and long-term memory, psychomotor function, sleep quality and mood with relatively low doses and durations of treatment have been reported. The exact magnitude of CNS effects resulting from propranolol use, especially in the early developmental stages and for prolonged periods of use, is not currently known. These effects may not be readily recognizable and require specialized assessment of cognitive function not routinely performed. Furthermore, there may be a delay between exposure and cognitive defects. The evidence to date provides a strong rationale to proceed with caution when prescribing propranolol for IH: treatment should be used only when indicated (in the presence of ulceration, impairment of a vital function or risk of permanent disfigurement) and for a limited duration, and the benefits of treatment should be weighed carefully against potential adverse events before treatment is initiated. This narrative review describes the evidence for an effect of propranolol use on CNS function from volunteer and patient studies, including IH. [ABSTRACT FROM AUTHOR]

Objective Previous research has demonstrated a significant relationship between posttraumatic stress disorder ( PTSD) and pain. While several models attempt to explain this relationship, significant questions remain regarding factors that may play a role in this interaction. The purpose of this study was to determine whether posttraumatic cognitions mediate the relationship between PTSD and pain. Design The sample comprised 136 veterans who presented to the VA Ann Arbor Health Care System seeking evaluation and treatment in the PTSD clinic. Participants completed the Clinician-Administered PTSD Scale, the Posttraumatic Cognitions Inventory, and Brief Pain Inventory-Short Form, along with other assessments as part of their evaluation. Results This study showed that the majority of patients (86.8%) reported some problems with pain. Further, the findings indicate that there is a significant relationship between PTSD severity and pain severity. Posttraumatic cognitions were not related to the level of pain experienced, but they were related to pain interference in this population. Conclusions In particular, negative cognitions regarding the self were associated with the level of pain-related interference, and partially mediated the relationship between PTSD and pain. The clinical implications of these findings are discussed. [ABSTRACT FROM AUTHOR]

Summary Background Both clobetasol propionate 0·05% (CP 0·05%) and tacrolimus 0·1% (T 0·1%) ointments have been shown to be efficacious and safe in treating vitiligo in the paediatric population. Objectives To assess efficacy and safety of these two therapies compared with each other and with placebo. Methods In this prospective study, children aged 2-16 years with vitiligo, stratified into 'facial' ( n = 55) and 'nonfacial' ( n = 45) groups, were randomized into three arms: CP 0·05% ointment ( n = 30), T 0·1% ointment ( n = 31) and placebo ( n = 29) for 6 months. Successful repigmentation, defined as > 50% improvement, was evaluated by comparing photographs taken at baseline and at 2, 4 and 6 months. Results In the facial group, 58% of the CP 0·05% group responded successfully compared with 58% of the T 0·1% group, and in the nonfacial group, 39% of the CP 0·05% group responded compared with 23% of the T 0·1% group ( P > 0·05). There was a significant difference in response between the CP 0·05% group vs. placebo ( P < 0·0001) and the T 0·1% group vs. placebo ( P = 0·0004). Spontaneous repigmentation was evaluated as 2·4%. No significant clinical adverse events were noted in any group. Conclusions Both CP 0·05% and T 0·1% ointments offer similar benefit in paediatric vitiligo, both facial and nonfacial. The facial lesions responded faster than the nonfacial ones. [ABSTRACT FROM AUTHOR]

A letter to the editor is presented regarding the systematic review of cases of cutaneous T-cell lymphoma transformation (CTCL) in pityriasis lichenoides (PL) and small plaque parapsoriasis (SPP).

A letter to the editor is presented on a study conducted on the comparison between nadolol and propranolol for the treatment of patients with infantile haemangiomas (IHs).

Linked Article:Has et al. Br J Dermatol 2020; 183: 614–627. [ABSTRACT FROM AUTHOR]

Eleven dogs with naturally occurring oral squamous cell carcinomas were treated with photodynamic therapy (PDT) using Photochlor (HPPH) as the photosensitizer. The largest length of the tumours measured in a two-dimensional plane ranged from 0.9 to 6.8 cm. Seven of the tumours invaded underlying bone as determined by radiograph appearance. Photochlor was injected intravenously at a dose of 0.3 mg kg[SUP-1]. Forty-eight hours later the tumours were treated. Tumours with a surface to base depth of greater than 1 cm were surgically reduced to less than 1 cm. Irradiation with 665 nm light with an energy density of 100 J cm[SUP-2] was administered. Eight dogs were considered cured with no tumour recurrence for at least 17 months after treatment. Local treatment of oral squamous cell carcinomas with PDT appears to give results similar to those obtained with surgical removal of large portions of the mandible or maxilla. The cosmetic results with PDT are superior to those of radical surgical removal. The new sensitizer, Photochlor, appears effective for oral squamous carcinomas with results similar to those reported for other sensitizers. [ABSTRACT FROM AUTHOR]

OBJECTIVES: Oral systemic corticosteroids are the mainstay of treatment for problematic hemangiomas; however, current information is based on anecdotal experience and retrospective studies. We aimed to determine whether systemic steroids are efficacious in proliferating hemangioma and to compare the efficacy and safety of 2 corticosteroid treatment modalities. PATIENTS AND METHODS: Twenty patients with problematic hemangiomas of infancy were randomly assigned to either daily oral prednisolone or monthly intravenous pulses of methylprednisolone. Their clinical outcomes (improvement using a visual analog score) and adverse events were compared at 3 months from baseline and 1 year of age. Data on possible surrogate markers of angiogenesis were available for the first 3 months. RESULTS: At 3 months, orally treated patients had a median visual analog score of 70 compared with 12 in the intravenous group. This response pattern was similar at the patients' first birthday: 50.0 vs -1.5. Additional treatment beyond 3 months was needed for 65% of the patients (7 in the intravenous and 6 in the oral group). Six of 8 patients with impaired vision at enrollment had an improved function at 1 year (4 patients in the intravenous group and 3 patients in the oral group). Of the 4 surrogate markers of angiogenesis measured (plasma basic fibroblast growth factor, vascular endothelial growth factor, vascular cellular adhesion molecule 1, endoglin, and urine basic fibroblast growth factor), the only 2 that decreased over time were vascular cellular adhesion molecule 1 and endoglin. Patients in the oral group had a higher rate of adverse effects, such as hypertension (18.6% vs 13.1%), abnormal cortisol (78% vs 60%), and growth retardation. CONCLUSIONS: Systemic corticosteroids are efficacious in stopping the proliferation of hemangiomas. The oral corticosteroids offered more clinical and biological benefit than the pulse steroids with higher risk of adverse effects. [ABSTRACT FROM AUTHOR]

A letter to the editor is presented regarding the association of clinical tools to identity localized scleroderma activity in paediatric patients.

The relationship between offense-specific forgiveness and post-traumatic stress disorder (PTSD) symptoms was examined in a cross-sectional survey of 178 college students reporting interpersonal trauma exposure, that is, a trauma exposure in which they identified a specific perpetrator. Higher levels of offense-specific forgiveness were significantly related to lower levels of PTSD symptoms. In path analyses, however, when gender and offense severity were allowed to directly predict both forgiveness and PTSD symptoms, the relationship was reduced to marginal significance. Exploratory analyses revealed that within the five most-commonly endorsed trauma subtypes, the relationship between forgiveness and PTSD symptoms may differ in strength and direction as a function of trauma type. Implications for research and treatment are discussed. [ABSTRACT FROM AUTHOR]

Recent observations have revealed the existence of enormously energetic active galactic nuclei outbursts in three relatively distant galaxy clusters. These outbursts have produced bubbles in the intracluster medium, apparently supported by pressure from relativistic particles and/or magnetic fields. Here, we argue that if ≥GeV particles are responsible then these particles are very likely protons and nuclei, rather than electrons, and that the γ-ray emission from these objects, arising from the interactions of these hadrons in the intracluster medium, may be marginally detectable with instruments such as the Gamma-ray Large Area Space Telescope (GLAST) and the High Energy Stereoscopic System (HESS). [ABSTRACT FROM AUTHOR]

To confirm the diagnosis, at least two of the following must be present: noncontributory medication history, erosive mucositis affecting two or more sites, or vesiculobullous lesions/atypical (often bullous) targets affecting < 10% BSA. Dear Editor, Severe cutaneous adverse reactions (SCARs) are challenging to diagnose and manage in children for the following two main reasons: (i) the literature on SCARs in children is sparse and extrapolated from adult data and (ii) many paediatric blistering SCAR cases are qualified as "atypical" or "incomplete" erythema multiforme (EM), Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) because certain clinical features prevent SCAR cases from fitting into this adult classification. Arch Dermatol 1993; 129: 92 - 6. 5 Canavan TN, Mathes EF, Frieden I, Shinkai K. Mycoplasma pneumoniae-induced rash and mucositis as a syndrome distinct from Stevens-Johnson syndrome and erythema multiforme: a systematic review. [Extracted from the article]

Summary: Background: Facial morphoea is a chronic inflammatory skin disorder, typically presenting in childhood and adolescence, which can be disfiguring, and which has been suggested to cause mild‐to‐moderate impairment in quality of life. Objectives: To explore the everyday experiences of children with facial morphoea by examining the psychosocial impact of living with facial morphoea and how children and their families manage its impact. Methods: We used a qualitative, social constructionist approach involving focus groups, in‐depth interviews and drawing activities with 10 children with facial morphoea aged 8–17 years and 13 parents. Interpretive thematic analysis was utilized to examine the data. Results: Children and parents reported on the stress of living with facial morphoea, which was related to the lack of knowledge about facial morphoea and the extent to which they perceived themselves as different from others. Self‐perceptions were based on the visibility of the lesion, different phases of life transitions and the reactions of others (e.g. intrusive questioning and bullying). Medication routines, and side‐effects such as weight gain, added to the stress experienced by the participants. To manage the impact of facial morphoea, children and their parents used strategies to normalize the experience by hiding physical signs of the illness, constructing explanations about what ‘it’ is, and by connecting with their peers. Conclusions: Understanding what it is like to live with facial morphoea from the perspectives of children and parents is important for devising ways to help children with the disorder achieve a better quality of life. Healthcare providers can help families access resources to manage anxiety, deal with bullying and construct adequate explanations of facial morphoea, in addition to providing opportunities for peer support. [ABSTRACT FROM AUTHOR]

Summary: Background: Epidermolysis bullosa (EB) is a group of rare and currently incurable genetic blistering disorders. As more pathogenic‐driven therapies are being developed, there is an important need for EB‐specific validated outcomes measures designed for use in clinical trials. Objectives: To test the reliability and construct validity of an instrument for scoring clinical outcomes of research for EB (iscorEB), a new combined clinician‐ and patient‐reported outcomes tool. Methods: We conducted an observational study consisting of independent 1‐day assessments (six assessors) at two academic hospitals. The assessments consisted of iscorEB clinician (iscorEB‐c), Birmingham Epidermolysis Bullosa Severity (BEBS) and global severity assessment for physicians; and iscorEB patient (iscorEB‐p), Quality of Life evaluation in Epidermolysis Bullosa and Children's Dermatology Life Quality Index for patients. Construct validity and intraclass correlation coefficients (ICCs) for interobserver, intraobserver and test–retest reliability were calculated. Results: Overall, 31 patients with a mean age of 19·5 years (1·8–45·2) were included. Disease severity was mild in 42% of cases, moderate in 29% and severe in 29%. The interobserver ICC was 0·96 for both the clinician‐reported section of iscorEB‐c and BEBS. The ICC for intraobserver reliability was 0·91 and 0·70 for the skin and mucosal domains of iscorEB‐c, respectively. Cronbach's alpha for iscorEB‐c was 0·89. The test–retest reliability of iscorEB‐p was 0·97 and Cronbach's alpha was 0·84. The clinical score differentiated between subjects with mild, moderate and severe disease, and both clinical and patient subscores discriminated between recessive dystrophic EB and other EB subtypes. Conclusions: iscorEB has robust reliability and construct validity, including strong ability to distinguish EB types and severities. Further studies are planned to test its responsiveness to change. [ABSTRACT FROM AUTHOR]

Juvenile edible crabs, Cancer pagurus L., were surveyed from Mumbles Head and Oxwich Bay in South Wales, UK, and the number of heterotrophic bacteria and vibrios in the hemolymph was determined. The percentage of crabs with hemolymph containing bacteria was variable over the survey with higher numbers of animals affected in summer than in winter. Post-moult crabs contained significantly higher numbers of heterotrophic bacteria in the hemolymph than pre- and intermoult animals. Crabs with cuticular damage to the gills also had significantly higher numbers of bacteria in the hemolymph. Crabs were found to have a high prevalence of infection by the dinoflagellate, Hematodinium. Such animals had significantly fewer bacteria in the blood in comparison with Hematodinium-free animals. Of the 463 crabs surveyed, only 3 individuals had hemolymph containing 2000 + CFU mL−1. Based on 16S r RNA gene sequences, two of these crabs contained a Vibrio pectenicida-like isolate, while the other had a mixed assemblage of vibrios. Although 59% of the crabs surveyed had culturable bacteria in the hemolymph, the majority only had small numbers (<2000 CFU mL−1), suggesting that such infections may be of limited importance to the sustainability of the crab fishery in this region. [ABSTRACT FROM AUTHOR]

Clinical evaluation of a young woman with subcutaneous fibrotic nodules, progressive distal joint contractures and marfanoid stature revealed a previously unrecognized fibrotic disorder characterized by several unique phenotypic features and some features overlapping with known disorders. Mutational analysis of the FBN1 and FBN2 genes excluded Marfan syndrome and congenital contractural arachnodactyly. MMP2 gene sequence analysis excluded multicentric osteolysis, nodulosis and arthropathy. The lack of mutations within the MAGP2 gene also excluded an MAGP2-associated disorder. In order to establish the mechanistic basis for the severe skin pathology noted in this patient, we performed transcriptional profiling of dermal fibroblasts, and candidate gene expression studies in conjunction with immunocytochemistry and cell-based and functional assays. Data from these experiments have further excluded any previously recognized fibrotic disorder and identified a unique pattern of gene expression in this patient consistent with progressive fibrosis. The pathogenic mechanisms included persistent proliferation of dermal fibroblasts in coexistence with a disarray of the microfibrillar network. Collagen accumulation, moreover, could be linked to extensive crosslinking resulting from increased activities of lysyl oxidases (LOX and LOXL), and lack of remodelling due to deficiencies in collagenolytic matrix metalloproteinases. The disorder may represent a novel syndrome in which transforming growth factor-β1-independent dermal fibrosis, unlike known microfibrillar disorders caused by single gene deficiencies, associates with a disarray of the microfibrillar network. [ABSTRACT FROM AUTHOR]

Two Rongcheng eclogite-bearing peridotite bodies (Chijiadian and Macaokuang) occur as lenses within the country rock gneiss of the northern Sulu terrane. The Chijiadian ultramafic body consists of garnet lherzolite, whereas the Macaokuang body is mainly meta-dunite. Both ultramafics are characterized by high MgO contents, low fertile element concentrations and total REE contents, which suggests that they were derived from depleted, residual mantle. High FeO contents, an LREE-enriched pattern and trace-element contents indicate that the bulk-rock compositions of these ultramafic rocks were modified by metasomatism. Oxygen-isotope compositions of analysed garnet, olivine, clinopyroxene and orthopyroxene from these two ultramafic bodies are between +5.2‰ and +6.2‰ ( δ18O), in the range of typical mantle values (+5.1 to +6.6‰). The eclogite enclosed within the Chijiadian lherzolite shows an LREE-enriched pattern and was formed by melts derived from variable degrees (0.005–0.05) of partial melting of peridotite. It has higher δ18O values (+7.6‰ for garnet and +7.7‰ for omphacite) than those of lherzolite. Small O-isotope fractionations (ΔCpx-Ol: 0.4‰, ΔCpx-Grt: 0.1‰, ΔGrt-Ol: 0.3–0.4‰) in both eclogite and ultramafic rocks suggest isotopic equilibrium at high temperature. The P–T estimates suggest that these rocks experienced subduction-zone ultrahigh-pressure (UHP) metamorphism at ∼700–800 °C, 5 GPa, with a low geothermal gradient. Zircon from the Macaokuang eclogite contains inclusions of garnet and diopside. The 225 ± 2 Ma U/Pb age obtained from these zircon may date either the prograde conditions just before peak metamorphism or the UHP metamorphic event, and therefore constrains the timing of subduction-related UHP metamorphism for the Rongcheng mafic–ultramafic bodies. [ABSTRACT FROM AUTHOR]

The article discusses a study which emphasizes that the number of satellites is the strongest independent predictor for neurocutaneous melanosis (NCM), exceeding other recognized factors, including size of the naevus and posterior axial location. Topics cited include a brief definition of NCM, the congenital melanocytic naevi features according to presence of NCM signs on magnetic resonance imaging (MRI) and strengths and limitations of the study.

The article discusses the diagnosis and treatment of patients with morphoea, also known as localized scleroderma. The disease is a rare disorder characterized by inflammation leading to thickening and sclerosis of the skin and subcutaneous tissue, potentially leading to permanent morbidity and functional and psychological disability. The retrospective cohort study has been used to discover the efficacy of methotrexate for treating morphoea.

The article presents a case series of patients with mosaic neurofibromatosis type 1 (NF1). It mentions NF1 is confined to a body segment and is thought to be caused by somatic mosaicism and analysis on blood- and patient-derived cultured Schwann cells or melanocytes from affected tissue. It also mentions MNF1 occurs when there is a postzygotic mutation in NF1 and somatic mosaicism can result from an NF1 mutation.

Summary: 面部硬皮病是一种罕见的慢性炎症性皮肤病,发生在面部并且可能导致毁容。它通常出现在童年和青春期,可能对自尊、心理社会转归和生活质量造成显著影响。调查硬皮病儿童患者生活质量的一些研究报告称,硬皮病会产生轻度至中度影响。然而,这些研究仅纳入少数面部硬皮病案例,所采用的调查问卷也无法描述患有这种可见皮肤病的复杂经历。本研究采用定性方法和社会建构主义途径来了解面部硬皮病对儿童及其父母日常生活的影响。该研究在加拿大开展,针对10名患有面部硬皮病的儿童(年龄在8‐17岁)和13名家长进行了单独或集体访谈,勾勒出患有面部硬皮病的经历。研究发现,患有面部硬皮病压力很大。患病压力与儿童认为自己是异类的观念有关,涉及到生活转变、他人的反应(例如侵入性质疑和欺凌)以及治疗影响(例如体重增加)。应对面部硬皮病影响的方法包括隐藏疾病体征、解释清楚疾病实质以及与病友交流。临床医生可以帮助面部硬皮病儿童患者学习如何应对侵入性质疑、提供获得病友支持的机会以及注意与疾病不确定性和医学治疗有关的焦虑,从而帮助他们改善生活质量。 [ABSTRACT FROM AUTHOR]

Summary: Facial morphea is a rare chronic inflammatory skin disorder that occurs on the face and can be disfiguring. It typically appears in childhood and adolescence and may have a significant impact on self‐esteem, psychosocial outcomes and quality of life. The few studies examining quality of life in children with morphea reported mild to moderate impact. However, these studies included small numbers of facial morphea cases and used questionnaires that cannot describe the complex experience of living with this visible skin disorder. This study used qualitative methods and a social constructionism approach to understand how facial morphea affects the everyday lives of children and their parents. The study was located in Canada and ten children with facial morphea, aged 8–17 years, and 13 parents took part in individual or group interviews and were asked to create a drawing about what it was like to live with facial morphea. Findings indicated that living with facial morphea was stressful. Stress was related to children's perceptions of being different, which was linked to life transitions, reactions of others (e.g. intrusive questioning and bullying) and treatment effects (e.g. weight gain). Managing the impact of facial morphea involved hiding its physical signs, constructing explanations about what “it” is and connecting with peers. Clinicians can support children with facial morphea achieve a better quality of life by helping them learn how to respond to intrusive queries, developing opportunities for peer support and attending to the anxiety associated with illness uncertainty and medical treatment. [ABSTRACT FROM AUTHOR]

Summary: 大疱性表皮松解(EB)是一种罕见的基因性发疱病,影响皮肤,甚至是内部器官。在研究中准确估算此类复杂疾病的严重性非常困难。我们研发了一项工具,被称为大疱性表皮松解研究流程成果评分工具(instrument for scoring clinical outcomes of research for Epidermolysis Bullosa, iscorEB),用于从医生和患者角度评估疾病严重性。在本文中,我们提到的数据来自于加拿大和美国的2家机构对iscorEB的测试。三十一名患者以及六名不同医学背景的医生参与了研究,以体现评分对于评估疾病严重性的一致性和有效性。我们发现,医生之间以及医生和患者之间有很好的一致性。我们还证明了iscorEB能够区分轻度、中度和重度疾病患者以及亚型EB。 [ABSTRACT FROM AUTHOR]

Summary: Epidermolysis bullosa (EB) is a group of rare genetic blistering disorders that affects the skin and occasionally internal organs. Accurate estimates of disease severity of such complex disease in the context of research is difficult. We developed a tool called instrument for scoring clinical outcomes of research for Epidermolysis Bullosa (iscorEB) with the goal to assess the disease severity from both physician's and patient's perspective. In the current paper, we report the data from testing the iscorEB in 2 institutions from Canada and the USA. Thirty‐one patients and six physicians from various medical specialties took part in a study to demonstrate how consistent and good the score was at assessing the disease severity. We found that there was good agreement between physicians and between physicians and patients. We also demonstrated that iscorEB is able to differentiate between patients with mild, moderate and severe disease and between subtypes of EB. [ABSTRACT FROM AUTHOR]