keyword
https://read.qxmd.com/read/38505726/diseases-categorized-as-autoinflammatory-keratinization-diseases-aikds-and-their-pathologies-and-treatments
#1
REVIEW
Masashi Akiyama
Whole-exome and whole-genome sequencing have become widespread in approximately the last 15 years, and the predisposing factors and pathomechanisms of inflammatory keratinization diseases, which have been unknown for a long time, have gradually been revealed. Hence, various inflammatory keratinization diseases are recognized to cause innate immunity hyperactivation. Therefore, we have been advocating for the clinical entity, "autoinflammatory keratinization diseases (AiKDs)" since 2017. AiKDs are inflammatory keratinization diseases caused by autoinflammatory-related pathomechanisms in the skin...
February 2024: Nagoya Journal of Medical Science
https://read.qxmd.com/read/38401879/-not-available
#2
JOURNAL ARTICLE
Julia Montero Menárguez, Victoria Amat Samaranch, L Puig Sanz, R Ruiz Villaverde, S Arias Santiago, M Larrea García, D Ruiz Genao, Marta Ferrán, L Schneller Pavelescu, C Romero Ferreiro, R Rivera Díaz
INTRODUCTION: PRP is a rare entity of unknown etiopathogenesis. Lack of clinical practice guidelines makes management challenging for clinicians. OBJECTIVE: To add our experience to the corpus evidence on PRP. METHODS: This was a retrospective, descriptive, and multicentric study of 65 patients with PRP, the largest European case series of patients with PRP ever reported. RESULTS: PRP was more prevalent in men with a mean age of 51 years, yet erythrodermic forms presented in older patients (mean age, 61 years)...
February 22, 2024: Actas Dermo-sifiliográficas
https://read.qxmd.com/read/38375322/case-report-successful-treatment-with-biologics-in-a-pediatric-patient-with-a-severe-inflammatory-skin-disease-and-novel-card14-mutation
#3
Michał Niedźwiedź, Joanna Narbutt, Aleksandra Siekierko, Małgorzata Skibińska, Bartłomiej Kwiek, Dorota Sobolewska-Sztychny, Magdalena Ciążyńska, Katarzyna Poznańska-Kurowska, Antoni Gostyński, Aleksandra Lesiak
CARD14 (caspase activation and recruitment domain) mutations have been associated with psoriasis vulgaris, psoriatic arthritis, generalized and palmoplantar pustular psoriasis, pityriasis rubra pilaris, and atopic dermatitis. We present a pediatric patient with a novel CARD14 : c.394A > T/- (Ile123Phe) mutation, diagnosed with CARD14 -associated papulosquamous eruption (CAPE), who was successfully treated with biological treatment.
2024: Frontiers in Medicine
https://read.qxmd.com/read/38357556/sezary-syndrome-initially-presenting-as-pityriasis-rubra-pilaris-clinicopathologic-study-of-3-cases
#4
Mouaz Alsawas, Eyas Alzayadneh, Eric Mou, Vincent Liu
No abstract text is available yet for this article.
February 2024: JAAD Case Reports
https://read.qxmd.com/read/38344497/delayed-presentation-of-pityriasis-rubra-pilaris-in-a-patient-on-treatment-with-ponatinib
#5
Marissa M Yaldo, Hailey Olds, Meredith Hengy, Meena Moossavi
Pityriasis rubra pilaris (PRP) is a rare dermatologic condition whose etiology is largely unknown. However, some medications, including ponatinib, have been implicated. Our case features an 80-year-old patient who developed PRP after two-and-a-half years of ponatinib use. We present this case due to the rare presentation of ponatinib-induced PRP as well as its significantly delayed presentation.
January 2024: Curēus
https://read.qxmd.com/read/38319642/resolution-of-pityriasis-rubra-pilaris-induced-ectropion-with-oral-dexamethasone-a-case-report
#6
JOURNAL ARTICLE
Ourania Fydanaki, Haytham Rezq, Anastasios E Sepetis, Varajini Joganathan
An 82-year-old male presented with generalised Pityriasis rubra pilaris (PRP) managed initially by dermatology team. The patient did not respond to first- and second-line treatment, including oral acitretin, steroid creams, and methotrexate, and developed bilateral cicatricial ectropion, for which he was referred to oculoplastic team for surgical management. A head injury resulting in subacute subdural haematoma, managed with a week course of low dose oral dexamethasone, resulted in the improvement of his skin condition and complete resolution of the cicatricial ectropion within a few weeks...
February 6, 2024: Orbit
https://read.qxmd.com/read/38203406/key-factors-in-the-complex-and-coordinated-network-of-skin-keratinization-their-significance-and-involvement-in-common-skin-conditions
#7
REVIEW
Nives Pondeljak, Liborija Lugović-Mihić, Lucija Tomić, Ena Parać, Lovre Pedić, Elvira Lazić-Mosler
The epidermis serves many vital roles, including protecting the body from external influences and healing eventual injuries. It is maintained by an incredibly complex and perfectly coordinated keratinization process. In this process, desquamation is essential for the differentiation of epidermal basal progenitor cells into enucleated corneocytes, which subsequently desquamate through programmed death. Numerous factors control keratinocyte differentiation: epidermal growth factor, transforming growth factor-α, keratinocyte growth factor, interleukins IL-1-β and IL-6, elevated vitamin A levels, and changes in Ca2+ concentration...
December 23, 2023: International Journal of Molecular Sciences
https://read.qxmd.com/read/38174859/updates-on-pityriasis-rubra-pilaris-a-scoping-review
#8
REVIEW
Ted Zhou, Abdullah Al Muqrin, Mohannad Abu-Hilal
Pityriasis rubra pilaris (PRP) is a rare, inflammatory papulosquamous skin disease with unknown exact etiology. Historically, PRP has been challenging to diagnose, especially during the acute phase, and to treat, due to its unclear pathogenesis. To better inform clinical practice, a literature review was conducted employing a broad search strategy to capture PRP-related published studies between January 1, 2012 to October 31, 2022. Two hundred twenty-one studies were identified, which were categorized into 9 themes: (1) potential causes and triggering factors, (2) comorbidities, (3) diagnostic difficulties, (4) genetics, (5) clinical manifestations and laboratory values, (6) treatment, (7) treatment-related adverse events, (8) quality of life, and (9) other...
January 4, 2024: Journal of Cutaneous Medicine and Surgery
https://read.qxmd.com/read/38159213/pityriasis-rubra-pilaris-an-updated-review-of-clinical-presentation-etiopathogenesis-and-treatment-options
#9
REVIEW
Tejas P Joshi, Madeleine Duvic
Pityriasis rubra pilaris (PRP) is a rare papulosquamous reaction pattern with a significant impact on quality of life. Type I PRP is the most common PRP variant, presenting as erythematous papules emerging in a follicular distribution and later coalescing into plaques with characteristic islands of sparing; histologically, an alternating pattern of orthokeratosis and parakeratosis is considered the hallmark of PRP (checkerboard hyperkeratosis). Other PRP variants (types II-V) differ in their age of onset and clinical presentation...
March 2024: American Journal of Clinical Dermatology
https://read.qxmd.com/read/38126177/biologics-for-inherited-disorders-of-keratinisation-a%C3%A2-systematic-review
#10
JOURNAL ARTICLE
Michelle K Y Chen, Alice L Flanagan, Deshan F Sebaratnam, Yaron Gu
BACKGROUND/OBJECTIVES: Recent literature highlights the potential of biologics in the management of inherited disorders of keratinisation. In this study, we conducted a systematic review of existing literature on treatment outcomes of inherited keratinisation disorders treated with biologics. METHODS: Eligible records were retrieved through searches of the electronic databases MEDLINE, Embase, PubMed and Scopus. Databases were searched from inception to July 2023 for eligible records...
December 21, 2023: Australasian Journal of Dermatology
https://read.qxmd.com/read/38103162/autoinflammatory-keratinization-diseases-the-concept-pathophysiology-and-clinical-implications
#11
REVIEW
Leszek Blicharz, Joanna Czuwara, Lidia Rudnicka, Antonio Torrelo
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...
December 16, 2023: Clinical Reviews in Allergy & Immunology
https://read.qxmd.com/read/38099031/ponatinib-induced-pityriasis-rubra-pilaris-case-report-and-review-of-literature
#12
JOURNAL ARTICLE
Sejal Jain, Adhyatm Bhandari, Keshavamurthy Vinay, Debajyoti Chatterjee
No abstract text is available yet for this article.
2023: Indian Dermatology Online Journal
https://read.qxmd.com/read/38099018/utility-of-dermoscopy-in-the-diagnosis-of-erythroderma-a-cross-sectional-study
#13
JOURNAL ARTICLE
Jayati Batra, Samridhi Gulati, Rishu Sarangal, Dimple Chopra, Sulabh Puri, Ravneet Kaur
BACKGROUND: It is difficult to diagnose the underlying cause of erythroderma on mere clinical presentation. The role of dermoscopy in diagnosing erythroderma secondary to various etiologies is evolving. AIM AND OBJECTIVES: This study aimed to observe the dermoscopic features of erythroderma secondary to different cutaneous disorders and compare them with clinical features and histopathology. MATERIALS AND METHODS: Twenty-nine consecutive patients of erythroderma were enrolled in the study...
2023: Indian Dermatology Online Journal
https://read.qxmd.com/read/38098840/case-report-area-of-focus-of-management-of-severe-pityriasis-rubra-pilaris-by-dose-optimization-of-adalimumab-biosimilar-in-combination-with-acitretin-and-montelukast
#14
Kristine Heidemeyer, S Morteza Seyed Jafari, Lena Farnina, Simon Bossart, Laurence Feldmeyer, Nikhil Yawalkar
Pityriasis rubra pilaris (PRP) is a rare inflammatory skin disorder characterized by hyperkeratotic follicular papules, orange-red scaling plaques with islands of sparing and palmoplantar keratoderma. While spontaneous resolution occurs in some cases, treatment can be challenging for others. The use of biologics in PRP management has gained attention in recent studies, although their high costs and potential side effects present limitations. We present a case of a 71-year-old patient with treatment-resistant PRP who showed significant improvement through optimized adalimumab treatment...
2023: Frontiers in Medicine
https://read.qxmd.com/read/37992813/methotrexate-and-acitretin-in-pityriasis-rubra-pilaris-a-retrospective-cohort-study
#15
JOURNAL ARTICLE
Jonathan D Greenzaid, Matthew L Hrin, Steven R Feldman, Lindsay C Strowd
No abstract text is available yet for this article.
November 20, 2023: Journal of the American Academy of Dermatology
https://read.qxmd.com/read/37961078/erythrodermic-pityriasis-rubra-pilaris-following-sars-cov-2-vaccination-treated-with-bimekizumab
#16
Mysa Saad, Reetesh Bose
No abstract text is available yet for this article.
December 2023: JAAD Case Reports
https://read.qxmd.com/read/37883982/eosinophils-in-traditionally-noneosinophil-rich-dermatoses
#17
JOURNAL ARTICLE
Isabella Sanchez, Marina K Ibraheim, Bonnie A Lee, Christina N Kraus, Ashley Elsensohn
The presence or absence of tissue eosinophilia has previously aided in the diagnosis of inflammatory skin conditions. However, recent studies have elucidated the presence of eosinophils in traditionally eosinophil-poor inflammatory skin diseases, such as dermatomyositis (DM), psoriasis, and lichen sclerosus (LS). This systematic review of the literature explores previous studies of tissue eosinophilia in skin biopsies of dermatoses that are believed to be classically poor in eosinophil. We identified 26 studies, the majority of which were retrospective reviews...
October 26, 2023: American Journal of Dermatopathology
https://read.qxmd.com/read/37847660/rapid-response-of-erythrodermic-pityriasis-rubra-pilaris-to-tildrakizumab
#18
JOURNAL ARTICLE
Jose M Villa-Gonzalez, Maria R Gonzalez-Hermosa, Xabier Atxutegi-Ayestaran, Juan Antonio Ratón Nieto, Jesús Gardeazabal García, Olatz Lasa Elgezua
No abstract text is available yet for this article.
October 17, 2023: Clinical and Experimental Dermatology
https://read.qxmd.com/read/37842160/wong-type-dermatomyositis-with-interstitial-lung-disease-and-anti-srp-and-pm-scl-antibodies-treated-with-intravenous-immunoglobulin
#19
Eric H Kowalski, H Mark Kenney, Christopher T Richardson
No abstract text is available yet for this article.
November 2023: JAAD Case Reports
https://read.qxmd.com/read/37767651/corrigendum-to-successful-treatment-of-a-child-s-pityriasis-rubra-pilaris-prp-with-ustekinumab-and-acitretin-1
#20
(no author information available yet)
No abstract text is available yet for this article.
2023: Pediatric Dermatology
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