1. Autoinflammatory Diseases in Pediatric Dermatology-Part 1: Urticaria-like Syndromes, Pustular Syndromes, and Mucocutaneous Ulceration Syndromes.
- Author
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Hernández-Ostiz S, Prieto-Torres L, Xirotagaros G, Noguera-Morel L, Hernández-Martín Á, and Torrelo A
- Subjects
- Autoantibodies immunology, Autoantigens immunology, Child, Enzymes genetics, Enzymes immunology, Humans, Receptors, Cytokine immunology, Skin Ulcer genetics, Skin Ulcer immunology, Urticaria classification, Urticaria genetics, Urticaria immunology, Hereditary Autoinflammatory Diseases classification, Hereditary Autoinflammatory Diseases immunology, Skin Diseases, Genetic classification, Skin Diseases, Genetic immunology
- Abstract
Monogenic autoinflammatory diseases are a heterogeneous emergent group of conditions that are currently under intensive study. We review the etiopathogenesis of these syndromes and their principal manifestations. Our aim is to propose a classification system based on the clinicopathologic features of typical skin lesions for routine clinical use in dermatology. Our focus is on diagnosis in pediatric practice given that this is the period when the signs and symptoms of these syndromes first appear. In Part 1 we discuss the course of urticaria-like syndromes, which include cryopyrin-associated periodic conditions and hereditary periodic fever syndromes. Pustular syndromes are also covered in this part. Finally, we review the range of therapies available as well as the genetic mutations associated with these autoinflammatory diseases., (Copyright © 2017 AEDV. Publicado por Elsevier España, S.L.U. All rights reserved.)
- Published
- 2017
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