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
Hauptverfasser: 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
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container_title Archives de pédiatrie : organe officiel de la Société française de pédiatrie
container_volume 25
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
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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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