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Summary: Chronic prurigo (CPG) is a neuroinflammatory dermatosis characterized by prolonged pruritus lasting more than 6 weeks, pruriginous skin lesions, and repeated scratching. Patients with CPG suffer significantly from psychological distress and a marked impairment in their quality of life. The most common subtype of CPG is chronic nodular prurigo (CNPG, also called prurigo nodularis). In addition to the clinical features of CPG and the burden of disease, this CME article provides an overview of the significant advances in understanding the pathophysiology, including the associated therapeutic options for CPG. Dupilumab is the first approved therapy for moderate and severe CNPG to date from the European Medicines Agency (EMA) and the US Food & Drug Administration (FDA). It also highlights other agents currently being studied in Phase II and Phase III clinical, randomized, placebo‐controlled trials. These include biologics such as nemolizumab (anti‐IL‐31‐RA‐mAb), vixarelimab/KPL‐716 (anti‐Oncostatin‐M receptor β‐mAb), and barzolvolimab/CDX‐0159 (anti‐KIT‐mAb), as well as Janus kinase inhibitors such as povorcitinib/INCB054707 and abrocitinib, and opioid modulators such as nalbuphine. [ABSTRACT FROM AUTHOR]

Background Objectives Methods Results Conclusions Prurigo nodularis (PN) also known as chronic prurigo, is a chronic inflammatory skin disease characterized by intensely itchy nodules/lesions which occur due to intensive scratching. PN management is, in part, based on clinician evaluations of PN lesions, which can be supported by clinician‐reported outcomes (ClinRO) such as the Prurigo Activity and Severity (PAS) instrument. A 5‐item version of PAS was included in recent phase‐3 dupilumab PN trials (PRIME [NCT04183335]/PRIME2 [NCT04202679]). The PAS score was derived using the unweighted sum of 3‐items of the 5‐item PAS (range, 0–11; higher score indicates worse activity and severity): Item 2 (number of lesions), Item 5a (percentage of lesions with excoriations/crusts) and Item 5b (percentage of healed lesions) for use in clinical practice and for communication of treatment benefit to physicians.To evaluate the measurement properties of PAS score and derive within‐patient (responder definition) and between‐group improvement thresholds for interpreting changes in PAS score in patients with PN.The data source was the pooled treatment group, intention‐to‐treat (ITT) data from the phase‐3 PRIME (NCT04183335) and PRIME2 (NCT04202679) studies evaluating the efficacy of dupilumab in adult patients with PN with ≥20 nodules and severe itch uncontrolled with topical therapies. PAS score reliability, validity and sensitivity to change were evaluated, and anchor‐ and distribution‐based methods were applied to derive meaningful change thresholds.The pooled ITT population included 311 patients (mean age 49.5 years, 65.3% female). Adequate to good psychometric properties were demonstrated for PAS score. The within‐patient meaningful improvement threshold was estimated as 3.0 points (absolute change) and 37% (per cent change). A 1.7‐point (absolute change) and 20% (per cent change) improvement were estimated to reflect a between‐group meaningful change in PAS score.PAS score is a simple, clinically relevant indicator of PN lesion activity and severity supported by suitable psychometric performance. [ABSTRACT FROM AUTHOR]

Copyright of Journal der Deutschen Dermatologischen Gesellschaft is the property of Wiley-Blackwell and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Summary: Pruritus is a cross‐disciplinary leading symptom of numerous diseases and represents an interdisciplinary diagnostic and therapeutic challenge. In contrast to acute pruritus, chronic pruritus (CP) is a symptom of various diseases that is usually difficult to treat. Scratching and the development of scratch‐associated skin lesions can alter the original skin status. In the presence of an itch‐scratch‐cycle, even secondary diseases such as chronic prurigo can develop. Chronic pruritus leads to considerable subjective suffering of those affected, which can result in restrictions on the health‐related quality of life such as sleep disturbances, anxiety, depressiveness, experience of stigmatization and/or social withdrawal up to clinically relevant psychic comorbidities. Medical care of patients should therefore include (a) interdisciplinary diagnosis and therapy of the triggering underlying disease, (b) therapy of the secondary symptoms of pruritus (dermatological therapy, sleep promotion, in the case of an accompanying or underlying psychological or psychosomatic disease an appropriate psychological‐psychotherapeutic treatment) and (c) symptomatic antipruritic therapy. The aim of this interdisciplinary guideline is to define and standardize the therapeutic procedure as well as the interdisciplinary diagnosis of CP. This is the short version of the updated S2k‐guideline for chronic pruritus. The long version can be found at www.awmf.org. [ABSTRACT FROM AUTHOR]

Dear Editor, Brachioradial pruritus (BRP) is typically located at the dorsolateral part of the forearms, mostly at the dermatomes C5/C6 but it may also involve other dermatomes at the arm and neck. Prior to treatment with naloxone, pruritus intensity was severe in all patients (worst pruritus intensity on the numerical rating scale [WI-NRS]: 8-10/10). [Extracted from the article]

Copyright of Journal der Deutschen Dermatologischen Gesellschaft is the property of Wiley-Blackwell and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Associated with a host of different diseases, pruritus is a cardinal symptom that poses an interdisciplinary diagnostic and therapeutic challenge. Over time, that symptom may progress independently of the initial cause, thus losing its function as a warning sign and turning into a clinically relevant disease of its own. In Germany, approximately 13.5 % of the general population are affected by chronic pruritus, with an incidence of 7 %. All forms of chronic pruritus require targeted treatment consisting of (a) diagnosis and management of the underlying disease, (b) dermatological treatment of primary or secondary (for example, dry skin, scratch lesions) symptoms, (c) symptomatic antipruritic treatment, and (d) psychological/psychotherapeutic treatment in case of an underlying or associated psychological or psychosomatic condition. Medical care of patients with chronic pruritus should therefore include an interdisciplinary approach, in particular with respect to diagnosis and therapy of the underlying disease as well as in terms of the management of treatment and adverse events. The objective of the present interdisciplinary guidelines is to define and standardize diagnostic and therapeutic procedures in patients with chronic pruritus. This is a short version of the current S2 guidelines on chronic pruritus. The long version may be found at . [ABSTRACT FROM AUTHOR]

Zusammenfassung Die Versorgung von Patienten mit chronischem Pruritus als Symptom unterschiedlicher Erkrankungen stellt weiterhin eine diagnostische und therapeutische Herausforderung in der Dermatologie dar. Doch ist es bereits durch eine strukturierte Anamnese und körperliche Untersuchung möglich, den Kreis der Differenzialdiagnosen einzukreisen. In der Therapie sind verschiedene Aspekte zu beachten: Im Vordergrund steht neben der Reduktion der Pruritusintensität an erster Stelle der Patientenwunsch, die zugrundeliegende Ursache zu finden und entsprechend zu therapieren. Führt dies nicht zu einer Besserung des Pruritus, sind eine Reihe topischer und systemischer Therapien zur symptomatischen Therapie in der aktuellen Leitlinie beschrieben. Durch Manifestation des Symptoms an der Haut (z. B. Xerosis durch Nutzung irritierender Substanzen, sekundäre Kratzläsionen) werden Dermatologen oft von den betroffenen Patienten konsultiert, es erfordert jedoch in den meisten Fällen eine interdisziplinäre Versorgung, um die internistischen, neurologischen oder psychosomatischen Aspekte zu identifizieren und behandeln. Obwohl die Grundlagenforschung bereits große Fortschritte gemacht hat, das Symptom in der Entstehung zu charakterisieren, sind spezifische Therapien noch rar und eine genaue Kenntnis der Rahmenbedingungen zur Umsetzung der Leitlinie (z. B. Off-Label-Use) ist unerlässlich. Der vorliegende CME-Artikel gibt einen Überblick über die Ursachen, Versorgung und Therapieoptionen bei chronischem Pruritus und bespricht sowohl die Grundlagen als auch das in den letzten Jahren neu gewonnene klinische Wissen. [ABSTRACT FROM AUTHOR]

The care of patients with chronic pruritus as a symptom of a wide variety of underlying diseases continues to confront dermatologists with diagnostic and therapeutic challenges. However, a structured history and a physical examination may already substantially help in narrowing down the number of potential differential diagnoses. Apart form reducing the intensity of pruritus, identification and appropriate treatment of the underlying disease are important needs of patients. If these goals doesn't lead to improvement of itch, current guidelines provide a number of topical and systemic therapies for symptomatic treatment. Various skin lesions (for example, xerosis caused by irritant substances, secondary scratch lesions) prompt patients to consult a dermatologist, but most cases require an interdisciplinary therapeutic approach to identify potential internal medicine, neurologic, or psychosomatic aspects. Although great strides have been made in basic research, specific therapies are still rare, and a precise knowledge of the legal framework for the implementation of guidelines (for example, off-label use) is essential. This CME article gives an overview of the causes of and treatment options for chronic pruritus and discusses both advances in basic research as well as progress in clinical knowledge. [ABSTRACT FROM AUTHOR]

Background The acronym VATER/VACTERL association describes the combination of at least three of the following cardinal features: vertebral defects, anorectal malformations, cardiac defects, tracheoesophageal fistula with or without esophageal atresia, renal malformations, and limb defects. Although fibroblast growth factor-8 ( FGF8) mutations have mainly found in patients with Kallmann syndrome, mice with a hypomorphic Fgf8 allele or complete gene invalidation display, aside from gonadotropin-releasing hormone deficiency, parts or even the entire spectrum of human VATER/VACTERL association. Methods We performed FGF8 gene analysis in 49 patients with VATER/VACTERL association and 27 patients presenting with a VATER/VACTERL-like phenotype (two cardinal features). Results We identified two heterozygous FGF8 mutations in patients displaying either VATER/VACTERL association (p.Gly29_Arg34dup) or a VATER/VACTERL-like phenotype (p.Pro26Leu) without limb anomalies. Whereas the duplication mutation has not been reported before, p.Pro26Leu was once observed in a Kallmann syndrome patient. Both our patients had additional bilateral cryptorchidism, a key phenotypic feature in males with FGF8 associated Kallmann syndrome. Each mutation was paternally inherited. Besides delayed puberty in both and additional unilateral cryptorchidism in one of the fathers, they were otherwise healthy. Serum hormone levels downstream the gonadotropin-releasing hormone in both patients and their fathers were within normal range. Conclusion Our results suggest FGF8 mutations to contribute to the formation of the VATER/VACTERL association. Further studies are needed to support this observation. Birth Defects Research (Part A) 100:750-759, 2014. © 2014 Wiley Periodicals, Inc. [ABSTRACT FROM AUTHOR]

Objective of this open, noninterventional, noncontrolled study was to investigate the tolerability and performance of a woad extract containing cream in subjects with dry, pruritic skin after twice daily application over 2 weeks. Assessments included sensorial characteristics, skin condition, pruritus (numeric rating scale [NRS], dynamic pruritus score [DPS]), skin dryness (itch-controlled days [ItchCD], overall dry skin [ODS]), transepidermal water loss (TEWL), skin hydration and quality of life (5PLQ, DLQI). All sensorial characteristics were well perceived (>4), with tolerability (4.77 ± 0.47) and no negative skin feelings (4.73 ± 0.74) achieving the highest scores (0-5 scale). Dry skin and symptoms improved significantly (p < .001) for all variables: NRS (from 4.8 ± 1.74 to 1.83 ± 2.18), ItchCD (from 8.23 ± 4.40 to 4.81 ± 4.07), ODS (from 1.80 ± 0.85 to 0.65 ± 0.65), hydration (23.33 ± 1.33 to 40.70 ± 1.86), and quality of life (5PLQ: from 7.12 ± 4.25 to 4.24 ± 3.67; DLQI: from 5.29 ± 4.79 to 3.00 ± 3.98). The DPS revealed a moderate antipruritic effect. The TEWL decreased slightly (p = .511). No side effects were reported. The study suggests that, the cream is effective, and well tolerated in treating dry, irritated, and pruritic skin., (© 2019 Wiley Periodicals, Inc.)