Dermatological features of auto-inflammatory recurrent fevers
Auto-inflammatory diseases are characterized by unexplained and recurrent attacks of systemic inflammation often involving the skin, joints, or serosal membranes. They are due to a dysfunction or dysregulation of the innate immunity, which is the first line of defense against pathogens. Early recogn...
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Veröffentlicht in: | Archives de pédiatrie : organe officiel de la Société française de pédiatrie 2018-02, Vol.25 (2), p.150-162 |
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creator | Escudier, A Mauvais, F-X Bastard, P Boussard, C Jaoui, A Koskas, V Lecoq, E Michel, A Orcel, M-C Truelle, P-E Wohrer, D Piram, M |
description | Auto-inflammatory diseases are characterized by unexplained and recurrent attacks of systemic inflammation often involving the skin, joints, or serosal membranes. They are due to a dysfunction or dysregulation of the innate immunity, which is the first line of defense against pathogens. Early recognition of these diseases by the clinician, especially by pediatricians encountering such pathologies in pediatric patients, is primordial to avoid complications. Skin manifestations, common in most auto-inflammatory diseases, are helpful for prompt diagnosis. After a brief physiopathological review, we will describe auto-inflammatory recurrent fevers by their main dermatological presentations: urticarial lesions, neutrophilic dermatoses, panniculitis, other maculopapular eruptions, dyskeratosis, skin vasculitis, and oral aphthous. We finally suggest a decision tree to help clinicians better target genetic exams in patients with recurrent fevers and dermatological manifestations. |
doi_str_mv | 10.1016/j.arcped.2017.12.001 |
format | Article |
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They are due to a dysfunction or dysregulation of the innate immunity, which is the first line of defense against pathogens. Early recognition of these diseases by the clinician, especially by pediatricians encountering such pathologies in pediatric patients, is primordial to avoid complications. Skin manifestations, common in most auto-inflammatory diseases, are helpful for prompt diagnosis. After a brief physiopathological review, we will describe auto-inflammatory recurrent fevers by their main dermatological presentations: urticarial lesions, neutrophilic dermatoses, panniculitis, other maculopapular eruptions, dyskeratosis, skin vasculitis, and oral aphthous. 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They are due to a dysfunction or dysregulation of the innate immunity, which is the first line of defense against pathogens. Early recognition of these diseases by the clinician, especially by pediatricians encountering such pathologies in pediatric patients, is primordial to avoid complications. Skin manifestations, common in most auto-inflammatory diseases, are helpful for prompt diagnosis. After a brief physiopathological review, we will describe auto-inflammatory recurrent fevers by their main dermatological presentations: urticarial lesions, neutrophilic dermatoses, panniculitis, other maculopapular eruptions, dyskeratosis, skin vasculitis, and oral aphthous. We finally suggest a decision tree to help clinicians better target genetic exams in patients with recurrent fevers and dermatological manifestations.</description><subject>Autoimmune Diseases - complications</subject><subject>Child</subject><subject>Decision Trees</subject><subject>Fever - complications</subject><subject>Humans</subject><subject>Inflammation - complications</subject><subject>Recurrence</subject><subject>Skin Diseases - etiology</subject><subject>Skin Diseases - immunology</subject><issn>1769-664X</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2018</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNo1z81KxDAUhuEgiDOO3oFIl25aT_6ThQsZf2HAjYK7kklOpEM7rUkrePdWHFff5uGDl5ALChUFqq53lUt-wFAxoLqirAKgR2RJtbKlUuJ9QU5z3gGAAcNPyIJZbqUxcklu7jB1buzb_qPxri0iunFKmIs-Fm4a-7LZx9Z1vyR9Fwn9lBLux9l9Ycpn5Di6NuP5YVfk7eH-df1Ubl4en9e3m3JglI6lC0qAsNtAHVKvhQnBCCMViiBtRIM6cOs1MA3ApeeKR-ZlFEJoIxkPfEWu_n6H1H9OmMe6a7LHtnV77KdcUzsH2VmzmV4e6LTtMNRDajqXvuv_ZP4D5H5YiQ</recordid><startdate>201802</startdate><enddate>201802</enddate><creator>Escudier, A</creator><creator>Mauvais, F-X</creator><creator>Bastard, P</creator><creator>Boussard, C</creator><creator>Jaoui, A</creator><creator>Koskas, V</creator><creator>Lecoq, E</creator><creator>Michel, A</creator><creator>Orcel, M-C</creator><creator>Truelle, P-E</creator><creator>Wohrer, D</creator><creator>Piram, M</creator><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>7X8</scope></search><sort><creationdate>201802</creationdate><title>Dermatological features of auto-inflammatory recurrent fevers</title><author>Escudier, A ; Mauvais, F-X ; Bastard, P ; Boussard, C ; Jaoui, A ; Koskas, V ; Lecoq, E ; Michel, A ; Orcel, M-C ; Truelle, P-E ; Wohrer, D ; Piram, M</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-p211t-ad64049bd1ae1c748dd84856e4d59fe8e7d39c70270035c363f2c5f44478523d3</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>fre</language><creationdate>2018</creationdate><topic>Autoimmune Diseases - complications</topic><topic>Child</topic><topic>Decision Trees</topic><topic>Fever - complications</topic><topic>Humans</topic><topic>Inflammation - complications</topic><topic>Recurrence</topic><topic>Skin Diseases - etiology</topic><topic>Skin Diseases - immunology</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Escudier, A</creatorcontrib><creatorcontrib>Mauvais, F-X</creatorcontrib><creatorcontrib>Bastard, P</creatorcontrib><creatorcontrib>Boussard, C</creatorcontrib><creatorcontrib>Jaoui, A</creatorcontrib><creatorcontrib>Koskas, V</creatorcontrib><creatorcontrib>Lecoq, E</creatorcontrib><creatorcontrib>Michel, A</creatorcontrib><creatorcontrib>Orcel, M-C</creatorcontrib><creatorcontrib>Truelle, P-E</creatorcontrib><creatorcontrib>Wohrer, D</creatorcontrib><creatorcontrib>Piram, M</creatorcontrib><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>MEDLINE - Academic</collection><jtitle>Archives de pédiatrie : organe officiel de la Société française de pédiatrie</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Escudier, A</au><au>Mauvais, F-X</au><au>Bastard, P</au><au>Boussard, C</au><au>Jaoui, A</au><au>Koskas, V</au><au>Lecoq, E</au><au>Michel, A</au><au>Orcel, M-C</au><au>Truelle, P-E</au><au>Wohrer, D</au><au>Piram, M</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Dermatological features of auto-inflammatory recurrent fevers</atitle><jtitle>Archives de pédiatrie : organe officiel de la Société française de pédiatrie</jtitle><addtitle>Arch Pediatr</addtitle><date>2018-02</date><risdate>2018</risdate><volume>25</volume><issue>2</issue><spage>150</spage><epage>162</epage><pages>150-162</pages><eissn>1769-664X</eissn><abstract>Auto-inflammatory diseases are characterized by unexplained and recurrent attacks of systemic inflammation often involving the skin, joints, or serosal membranes. They are due to a dysfunction or dysregulation of the innate immunity, which is the first line of defense against pathogens. Early recognition of these diseases by the clinician, especially by pediatricians encountering such pathologies in pediatric patients, is primordial to avoid complications. Skin manifestations, common in most auto-inflammatory diseases, are helpful for prompt diagnosis. After a brief physiopathological review, we will describe auto-inflammatory recurrent fevers by their main dermatological presentations: urticarial lesions, neutrophilic dermatoses, panniculitis, other maculopapular eruptions, dyskeratosis, skin vasculitis, and oral aphthous. We finally suggest a decision tree to help clinicians better target genetic exams in patients with recurrent fevers and dermatological manifestations.</abstract><cop>France</cop><pmid>29395885</pmid><doi>10.1016/j.arcped.2017.12.001</doi><tpages>13</tpages></addata></record> |
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source | MEDLINE; Elsevier ScienceDirect Journals |
subjects | Autoimmune Diseases - complications Child Decision Trees Fever - complications Humans Inflammation - complications Recurrence Skin Diseases - etiology Skin Diseases - immunology |
title | Dermatological features of auto-inflammatory recurrent fevers |
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