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Review ARTICLE Provisionally accepted The full-text will be published soon. Notify me

Front. Immunol. | doi: 10.3389/fimmu.2019.02448


  • 1University of Barcelona, Spain
  • 2Hospital Universitario de Bellvitge, Spain
  • 3Hospital Clínic de Barcelona, Spain

Autoinflammatory diseases include disorders with a monogenic cause and also complex conditions associated to polygenic or multifactorial factors. An increased number of both monogenic and polygenic autoinflammatory conditions have been identified during the last years. Although skin manifestations are often predominant in monogenic autoinflammatory diseases, clinical and histopathological information regarding their dermatological involvement is still scarce. Monogenic autoinflammatory diseases with cutaneous expression can be classified based on the predominant lesion: 1) maculopapular rashes or inflammatory plaques; 2) urticarial rashes; 3) pustular, pyogenic or neutrophilic dermatosis-like rashes; 4) panniculitis or subcutaneous nodules; 5) vasculitis or vasculopathy; 6) hyperkeratotic lesions; 7) hyperpigmented lesions; 8) bullous lesions; and 9) aphthous lesions. By using this classification, this review intends to provide clinical and histopathological knowledge about cutaneous involvement in monogenic autoinflammatory diseases.

Keywords: Monogenic autoinflammatory diseases, Autoinflammatory diseases, Clinical dermatology, Dermatopathology, Autoinflammation

Received: 24 Jul 2019; Accepted: 01 Oct 2019.

Copyright: © 2019 Figueras-Nart, Mascaró-Gali, Solanich and Hernández-Rodríguez. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.

* Correspondence: MD. Ignasi Figueras-Nart, University of Barcelona, Barcelona, Spain,