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Pityriasis rubra pilaris

J Krygier, G Leemans, R Forsyth, A de Becker, J Gutermuth, M Grosber
BACKGROUND: Pityriasis rubra pilaris (PRP) is a cutaneous syndrome of unknown origin. Most cases are sporadic and acquired. Herein we report a fifth case of PRP-like eruption associated with ponatinib, a tyrosine kinase inhibitor (TKI). PATIENTS AND METHODS: A 60-year-old woman presented at the dermatology department with an erythemato-squamous eruption present for 2weeks. The patient was also being treated in haematology for recurrence of acute lymphoblastic leukaemia...
June 11, 2018: Annales de Dermatologie et de Vénéréologie
Lauren Bonomo, Jacob O Levitt
Refractory pityriasis rubra pilaris (PRP) often is treated off-label with the same biologic therapies that are approved for the treatment of psoriasis, most commonly tumor necrosis factor (TNF) α antagonists and ustekinumab; however, the IL-17A antagonist secukinumab also has shown efficacy in the treatment of PRP. We report 2 new cases of severe refractory PRP that responded rapidly to treatment with secukinumab.
May 2018: Cutis; Cutaneous Medicine for the Practitioner
Lauren Bonomo, Aishwarya Raja, Kathryn Tan, Emma Guttman-Yassky
No abstract text is available yet for this article.
March 2018: JAAD Case Reports
Kathrin Hanfstingl, Agnes Pekar-Lukacs, Reinhard Motz, Emmanuella Guenova, Wolfram Hoetzenecker
Pityriasis rubra pilaris is an inflammatory dermatologic disorder of unknown cause and often confounded with psoriasis. It is characterised by hyperkeratotic follicular papules, scaly erythematous plaques, palmoplantar keratoderma, and a progression to generalised erythroderma. Here, we report the case of a 68-year-old man with pityriasis rubra pilaris, who was successfully treated with ixekizumab, an interleukin-17A inhibitor.
May 2018: Case Reports in Dermatology
A K Dewan, L Sowerby, S Jadeja, C Lian, P Wen, J R Brown, D C Fisher, N R LeBoeuf
BACKGROUND: Phosphoinositide 3-kinase (PI3K) inhibitors are a class of small-molecule inhibitors approved for the treatment of certain leukaemias and lymphomas. Their dermatological adverse event profile is poorly described. AIM: To characterize a rare cutaneous adverse event from PI3K inhibitors in order to help dermatologists and oncologists identify and effectively manage such eruptions. METHODS: This was a retrospective analysis of patients receiving PI3K inhibitors referred to the Skin Toxicities Program in The Center for Cutaneous Oncology...
May 30, 2018: Clinical and Experimental Dermatology
N Ismail, J Callander, M Williams, A V Anstey
No abstract text is available yet for this article.
May 20, 2018: Clinical and Experimental Dermatology
Megan E MacGillivray, Loretta Fiorillo
Pityriasis rubra pilaris (PRP) is an uncommon papulosquamous dermatosis characterized by follicular, erythematous, hyperkeratotic papules coalescing to salmon-coloured plaques with islands of sparing. The disease tends to be self-limited and resolves spontaneously after a few years. In some cases, the disease is persistent. However, recurrence of this disease has rarely been described. An 8-year-old male was diagnosed with type III (classic juvenile) PRP. He was treated with acitretin, and his skin was clear after 6 months...
May 1, 2018: Journal of Cutaneous Medicine and Surgery
I Spoerri, S Herms, O Eytan, O Sarig, K Heinimann, E Sprecher, P H Itin, B Burger
The familial form of pityriasis rubra pilaris (PRP) is caused by dominant mutations in the coiled-coiled domain of CARD14 1 . Different variants in CARD14 have been associated with psoriasis 2 . As some PRP-causing and psoriasis-associated CARD14 variants showed increased NF-κB activation 2,3 , inappropriate NF-κB-regulation is thought to be involved in the development of both diseases. This article is protected by copyright. All rights reserved.
April 28, 2018: Journal of the European Academy of Dermatology and Venereology: JEADV
Monica Napolitano, Damiano Abeni, Biagio Didona
Pityriasis rubra pilaris (PRP) is a rare inflammatory papulosquamous skin disease, often refractory to conventional therapies. The off-label use of biologics, such as anti-TNF, -IL-12/IL-23, -IL-17 agents, has been proven successful, in the last two decades, in PRP treatment. Our aim was to analyse the literature for the use of biologics in PRP treatment. We conducted a review by "Pubmed" and "" searches. 68 articles met our selection criteria and were herein discussed. Out of 86 PRP patients, the vast majority of which treated with anti-TNF, -IL-12/IL-23, and -IL-17 biologics, either alone or in combination therapy, a marked-to-complete response (50-78%), a partial response (11-25%) or no/poor response (11-25%) was observed...
March 30, 2018: Journal of the American Academy of Dermatology
Emily Yiping Gan, See Ket Ng, Chee Leok Goh, Siong See Joyce Lee
BACKGROUND: There are patients with recalcitrant psoriasiform plaques that do not fit into conventional categories of facial dermatoses. Our study aims to describe the clinicopathological characteristics of several patients with a unique presentation of persistent psoriasiform facial rashes. METHODS: This retrospective cross-sectional study analyzed clinical and histological data of known cases of recalcitrant psoriasiform dermatosis of the face diagnosed at National Skin Centre, Singapore, over 10 years...
March 31, 2018: Journal of Cutaneous Pathology
Nolan J Maloney, Lisa D Hisaw, Scott Worswick
No abstract text is available yet for this article.
March 5, 2018: Journal of the American Academy of Dermatology
Brittany G Craiglow, Lynn M Boyden, Ronghua Hu, Marie Virtanen, John Su, Gabriela Rodriguez, Catherine McCarthy, Paula Luna, Margarita Larralde, Stephen Humphrey, Kristen E Holland, Marcia Hogeling, Benjamin Hidalgo-Matlock, Bruno Ferrari, Esteban Fernandez-Faith, Beth Drolet, Kelly M Cordoro, Anne M Bowcock, Richard J Antaya, Kurt Ashack, Richard J Ashack, Richard P Lifton, Leonard M Milstone, Amy S Paller, Keith A Choate
BACKGROUND: Heterozygous mutations in caspase recruitment domain family member 14 gene (CARD14) have been shown to be associated with psoriasis and familial pityriasis rubra pilaris (PRP). Many subjects with CARD14 mutations display features of both disorders, which can result in diagnostic uncertainty. In addition, these eruptions are often recalcitrant to conventional psoriasis therapies such as methotrexate, oral retinoids, and tumor necrosis factor-α inhibitors. OBJECTIVE: We sought to describe the clinical characteristics, family history, and response to therapy in subjects with papulosquamous eruptions due to mutations in CARD14...
March 1, 2018: Journal of the American Academy of Dermatology
Aine Kelly, Aoife Lally
No abstract text is available yet for this article.
February 11, 2018: BMJ Case Reports
Masashi Akiyama, Takuya Takeichi, John A McGrath, Kazumitsu Sugiura
Classifying inflammatory skin diseases is challenging, especially for the expanding group of disorders triggered by genetic factors resulting in hyperactivated innate immunity that result in overlapping patterns of dermal and epidermal inflammation with hyperkeratosis. For such conditions, the umbrella term "autoinflammatory keratinization diseases" (AIKD) has been proposed. AIKD encompasses diseases with mixed pathomechanisms of autoinflammation and autoimmunity, and includes IL-36 receptor antagonist (IL-36Ra)-related pustulosis, CARD14-mediated pustular psoriasis, pityriasis rubra pilaris (PRP) type V, and familial keratosis lichenoides chronica (KLC)...
May 2018: Journal of Dermatological Science
Laura C Gironi, Rossana Tiberio, Lorenzo Dagna, Gianluca Landucci, Angela Giacalone, Renzo L Boldorini, Enrico Colombo, Paola Savoia
No abstract text is available yet for this article.
February 7, 2018: Giornale Italiano di Dermatologia e Venereologia: Organo Ufficiale, Società Italiana di Dermatologia e Sifilografia
Lucie Pellonnet, Fanny Beltzung, Frédéric Franck, Jacques Rouanet, Michel D'incan
No abstract text is available yet for this article.
February 1, 2018: European Journal of Dermatology: EJD
Lukas Koch, Clemens Schöffl, Werner Aberer, Cesare Massone
Treatment recommendations for pityriasis rubra pilaris (PRP) are based solely on case reports and small case series, as to-date no randomized controlled trials are available. We present here a case series of 3 patients and a literature review of 28 studies treating a total of 116 patients, with the aim of providing data regarding efficacy and safety of methotrexate in the treatment of PRP. Methotrexate was effective in our patients; the review showed an overall response rate of 65.5% with complete clearing in 23...
April 27, 2018: Acta Dermato-venereologica
Dingyuan Wang, Vanessa Cui-Lian Chong, Wei-Sheng Chong, Hazel H Oon
Pityriasis rubra pilaris (PRP) is an idiopathic, papulosquamous inflammatory dermatosis. It is characterized by hyperkeratotic follicular papules coalescing into orange-red scaly plaques, islands of sparing, and palmoplantar keratoderma. PRP can be subdivided into six clinical subtypes according to Griffiths' classification, based on age of onset, disease extent, prognosis, and other associated features. The sixth subtype of PRP occurs in individuals affected by HIV infection, and retroviral screening in all de novo cases of PRP is advised...
June 2018: American Journal of Clinical Dermatology
S Quenan, E Laffitte
Pityriasis rubra pilaris is a rare heterogeneous dermatosis associating three clinical signs to different degrees: follicular corneal papules, reddish-orange palmoplantar keratoderma and erythematosquamous lesions that may in some cases be very extensive, interspersed with patches of healthy skin. The aetiology is unclear, and in most cases, the trigger factors consist of trauma or infection, probably in subjects with an existing predisposition. In other cases, the condition is associated with immunological disorders or, in familial cases, genetic keratinisation abnormalities similar to ichthyosis...
January 2018: Annales de Dermatologie et de Vénéréologie
S Roenneberg, T Biedermann
Pityriasis rubra pilaris (PRP) is a rare inflammatory skin disease that affects men and women of all ages and also children. The clinical appearance of PRP is highly variable, as is the individual prognosis. Therefore, stratifying PRP into six disease subtypes represents a first step to personalized medicine for this rare inflammatory skin disease. The next step should be to associate specific therapeutic strategies with these subtypes of PRP. However, no randomized, controlled trials on the treatment of PRP have been performed...
December 15, 2017: Journal of the European Academy of Dermatology and Venereology: JEADV
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