Skin manifestations in autoinflammatory syndromes
Summary Autoinflammatory diseases encompass a group of inflammatory diseases that are non‐infectious, non‐allergic, non‐autoimmune and non‐immunodeficient. The term was initially coined for a small group of familial periodic fever syndromes of which familial Mediterranean fever (FMF) is the most com...
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| Published in | Journal der Deutschen Dermatologischen Gesellschaft Vol. 9; no. 3; pp. 232 - 245 |
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| Main Authors | , |
| Format | Journal Article |
| Language | English |
| Published |
Oxford, UK
Blackwell Publishing Ltd
01.03.2011
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| Abstract | Summary
Autoinflammatory diseases encompass a group of inflammatory diseases that are non‐infectious, non‐allergic, non‐autoimmune and non‐immunodeficient. The term was initially coined for a small group of familial periodic fever syndromes of which familial Mediterranean fever (FMF) is the most common and best known. Genetic and molecular analyses demonstrated for the majority of these diseases an impairment of inflammasomes to cause an increased activity of an interleukin‐1‐dependent inflammatory response. Over the last years an increasing number of either rare hereditary syndromes or acquired common diseases could be summarized under the designation of autoinflammatory disease, thus creating an emerging new rubric of inflammatory diseases. Many of them display cutaneous manifestations as both concomitant or more rarely main symptoms. To name some of them like erysipelas‐like erythema in FMF; urticaria‐like rashes in tumor necrosis factor receptor 1‐ or cryopyrin‐associated periodic syndromes (TRAPS, CAPS), hyperimmunoglobulin D syndrome (HIDS) or Schnitzler syndrome; pyoderma gangrenosum and acne in PAPA syndrome; or behçetoid aphthous ulcerations in HIDS and PFAPA syndrome. Based on the new insights into pathogenesis one increasingly realizes the good response of these diseases to IL‐1 antagonist therapies. |
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| AbstractList | Autoinflammatory diseases encompass a group of inflammatory diseases that are non-infectious, non-allergic, non-autoimmune and non-immunodeficient. The term was initially coined for a small group of familial periodic fever syndromes of which familial Mediterranean fever (FMF) is the most common and best known. Genetic and molecular analyses demonstrated for the majority of these diseases an impairment of inflammasomes to cause an increased activity of an interleukin-1-dependent inflammatory response. Over the last years an increasing number of either rare hereditary syndromes or acquired common diseases could be summarized under the designation of autoinflammatory disease, thus creating an emerging new rubric of inflammatory diseases. Many of them display cutaneous manifestations as both concomitant or more rarely main symptoms. To name some of them like erysipelas-like erythema in FMF; urticaria-like rashes in tumor necrosis factor receptor 1- or cryopyrin-associated periodic syndromes (TRAPS, CAPS), hyperimmunoglobulin D syndrome (HIDS) or Schnitzler syndrome; pyoderma gangrenosum and acne in PAPA syndrome; or behçetoid aphthous ulcerations in HIDS and PFAPA syndrome. Based on the new insights into pathogenesis one increasingly realizes the good response of these diseases to IL-1 antagonist therapies. Summary Autoinflammatory diseases encompass a group of inflammatory diseases that are non‐infectious, non‐allergic, non‐autoimmune and non‐immunodeficient. The term was initially coined for a small group of familial periodic fever syndromes of which familial Mediterranean fever (FMF) is the most common and best known. Genetic and molecular analyses demonstrated for the majority of these diseases an impairment of inflammasomes to cause an increased activity of an interleukin‐1‐dependent inflammatory response. Over the last years an increasing number of either rare hereditary syndromes or acquired common diseases could be summarized under the designation of autoinflammatory disease, thus creating an emerging new rubric of inflammatory diseases. Many of them display cutaneous manifestations as both concomitant or more rarely main symptoms. To name some of them like erysipelas‐like erythema in FMF; urticaria‐like rashes in tumor necrosis factor receptor 1‐ or cryopyrin‐associated periodic syndromes (TRAPS, CAPS), hyperimmunoglobulin D syndrome (HIDS) or Schnitzler syndrome; pyoderma gangrenosum and acne in PAPA syndrome; or behçetoid aphthous ulcerations in HIDS and PFAPA syndrome. Based on the new insights into pathogenesis one increasingly realizes the good response of these diseases to IL‐1 antagonist therapies. |
| Author | Braun-Falco, Markus Ruzicka, Thomas |
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| BackLink | https://www.ncbi.nlm.nih.gov/pubmed/21176105$$D View this record in MEDLINE/PubMed |
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| Cites_doi | 10.1016/j.jpeds.2010.02.040 10.1016/j.jaci.2009.11.016 10.1007/s00296-010-1474-6 10.1001/archderm.144.3.392 10.1016/j.jaci.2009.10.066 10.1016/j.jaci.2009.11.001 10.1111/j.1525-1470.2009.01060.x 10.1111/j.1365-2133.2009.09404.x 10.1007/s10067-010-1375-9 10.1002/art.25057 10.1111/j.1346-8138.2007.00342.x 10.1111/j.1651-2227.2009.01554.x 10.1016/j.cell.2010.03.002 10.1097/MD.0b013e318190cfb7 10.1016/j.cell.2010.01.040 10.1007/s10067-010-1516-1 10.1002/art.23475 10.1067/mjd.2000.103048 10.1056/NEJMoa0807865 10.1001/archderm.136.12.1487 10.1097/00005792-200101000-00004 |
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Autoinflammatory diseases encompass a group of inflammatory diseases that are non‐infectious, non‐allergic, non‐autoimmune and non‐immunodeficient. The... Autoinflammatory diseases encompass a group of inflammatory diseases that are non-infectious, non-allergic, non-autoimmune and non-immunodeficient. The term... |
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| SubjectTerms | CAPS Hereditary Autoinflammatory Diseases - complications Hereditary Autoinflammatory Diseases - diagnosis Hereditary Autoinflammatory Diseases - therapy Humans inflammasome interleukin-1 Mediterranean fever PAPA syndrome periodic fever syndromes Schnitzler syndrome Skin Diseases - diagnosis Skin Diseases - etiology Skin Diseases - therapy Syndrome |
| Title | Skin manifestations in autoinflammatory syndromes |
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