Prevalence and age distribution of pemphigus and pemphigoid diseases among paediatric patients in Germany
Background Autoimmune bullous diseases are rare and mostly occur in adults. Several cases and small case series have been described in children, but no systematic study about the prevalence of autoimmune bullous diseases (AIBD) in children is available. Patients and methods We analysed data of 1.7 m...
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| Veröffentlicht in: | Journal of the European Academy of Dermatology and Venereology 2020-11, Vol.34 (11), p.2600-2605 |
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| creator | Hübner, F. König, I.R. Holtsche, M.M. Zillikens, D. Linder, R. Schmidt, E. |
| description | Background
Autoimmune bullous diseases are rare and mostly occur in adults. Several cases and small case series have been described in children, but no systematic study about the prevalence of autoimmune bullous diseases (AIBD) in children is available.
Patients and methods
We analysed data of 1.7 million children insured in the largest German health insurance company based on the ICD‐10‐GM classification for the year 2015. Data were adjusted to the general German population based on the data of the Federal Statistical Office for the year 2015.
Results
The prevalence of AIBD was calculated to 101.1/million children in 2015, resulting in about 1351 patients below the age of 18 years in Germany. The highest prevalence of all AIBD was seen for pemphigus vulgaris (30.5/million children) followed by linear IgA disease (24.5/million children) and bullous pemphigoid (4.9/million children).
Conclusion
Autoimmune bullous diseases in minors are scarce but should be taken into consideration in patients with pruritus and/or blisters and erosions on the skin and/or mucous membranes. Treatment is challenging, and due to the rarity of AIBD in minors, the management of these disorders in this patient population is best performed in specialized centres in a multidisciplinary approach, including paediatric dermatologists or dermatologists and paediatricians. |
| doi_str_mv | 10.1111/jdv.16467 |
| format | Article |
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Autoimmune bullous diseases are rare and mostly occur in adults. Several cases and small case series have been described in children, but no systematic study about the prevalence of autoimmune bullous diseases (AIBD) in children is available.
Patients and methods
We analysed data of 1.7 million children insured in the largest German health insurance company based on the ICD‐10‐GM classification for the year 2015. Data were adjusted to the general German population based on the data of the Federal Statistical Office for the year 2015.
Results
The prevalence of AIBD was calculated to 101.1/million children in 2015, resulting in about 1351 patients below the age of 18 years in Germany. The highest prevalence of all AIBD was seen for pemphigus vulgaris (30.5/million children) followed by linear IgA disease (24.5/million children) and bullous pemphigoid (4.9/million children).
Conclusion
Autoimmune bullous diseases in minors are scarce but should be taken into consideration in patients with pruritus and/or blisters and erosions on the skin and/or mucous membranes. Treatment is challenging, and due to the rarity of AIBD in minors, the management of these disorders in this patient population is best performed in specialized centres in a multidisciplinary approach, including paediatric dermatologists or dermatologists and paediatricians.</description><identifier>ISSN: 0926-9959</identifier><identifier>EISSN: 1468-3083</identifier><identifier>DOI: 10.1111/jdv.16467</identifier><identifier>PMID: 32289873</identifier><language>eng</language><publisher>England</publisher><subject>Adolescent ; Adult ; Age Distribution ; Autoimmune Diseases ; Child ; Germany - epidemiology ; Humans ; Pemphigoid, Bullous - epidemiology ; Pemphigus - epidemiology ; Prevalence</subject><ispartof>Journal of the European Academy of Dermatology and Venereology, 2020-11, Vol.34 (11), p.2600-2605</ispartof><rights>2020 The Authors. Journal of the European Academy of Dermatology and Venereology published by John Wiley & Sons Ltd on behalf of European Academy of Dermatology and Venereology</rights><rights>2020 The Authors. Journal of the European Academy of Dermatology and Venereology published by John Wiley & Sons Ltd on behalf of European Academy of Dermatology and Venereology.</rights><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c3607-65b831ca193204f7b00cf53b5b8f91539e7752790cc94d7ef52092a768f4caba3</citedby><cites>FETCH-LOGICAL-c3607-65b831ca193204f7b00cf53b5b8f91539e7752790cc94d7ef52092a768f4caba3</cites><orcidid>0000-0003-2886-9499</orcidid></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://onlinelibrary.wiley.com/doi/pdf/10.1111%2Fjdv.16467$$EPDF$$P50$$Gwiley$$Hfree_for_read</linktopdf><linktohtml>$$Uhttps://onlinelibrary.wiley.com/doi/full/10.1111%2Fjdv.16467$$EHTML$$P50$$Gwiley$$Hfree_for_read</linktohtml><link.rule.ids>314,776,780,1411,27903,27904,45553,45554</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/32289873$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Hübner, F.</creatorcontrib><creatorcontrib>König, I.R.</creatorcontrib><creatorcontrib>Holtsche, M.M.</creatorcontrib><creatorcontrib>Zillikens, D.</creatorcontrib><creatorcontrib>Linder, R.</creatorcontrib><creatorcontrib>Schmidt, E.</creatorcontrib><title>Prevalence and age distribution of pemphigus and pemphigoid diseases among paediatric patients in Germany</title><title>Journal of the European Academy of Dermatology and Venereology</title><addtitle>J Eur Acad Dermatol Venereol</addtitle><description>Background
Autoimmune bullous diseases are rare and mostly occur in adults. Several cases and small case series have been described in children, but no systematic study about the prevalence of autoimmune bullous diseases (AIBD) in children is available.
Patients and methods
We analysed data of 1.7 million children insured in the largest German health insurance company based on the ICD‐10‐GM classification for the year 2015. Data were adjusted to the general German population based on the data of the Federal Statistical Office for the year 2015.
Results
The prevalence of AIBD was calculated to 101.1/million children in 2015, resulting in about 1351 patients below the age of 18 years in Germany. The highest prevalence of all AIBD was seen for pemphigus vulgaris (30.5/million children) followed by linear IgA disease (24.5/million children) and bullous pemphigoid (4.9/million children).
Conclusion
Autoimmune bullous diseases in minors are scarce but should be taken into consideration in patients with pruritus and/or blisters and erosions on the skin and/or mucous membranes. Treatment is challenging, and due to the rarity of AIBD in minors, the management of these disorders in this patient population is best performed in specialized centres in a multidisciplinary approach, including paediatric dermatologists or dermatologists and paediatricians.</description><subject>Adolescent</subject><subject>Adult</subject><subject>Age Distribution</subject><subject>Autoimmune Diseases</subject><subject>Child</subject><subject>Germany - epidemiology</subject><subject>Humans</subject><subject>Pemphigoid, Bullous - epidemiology</subject><subject>Pemphigus - epidemiology</subject><subject>Prevalence</subject><issn>0926-9959</issn><issn>1468-3083</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2020</creationdate><recordtype>article</recordtype><sourceid>24P</sourceid><sourceid>WIN</sourceid><sourceid>EIF</sourceid><recordid>eNp1kD9PwzAQxS0EoqUw8AVQRhjS2nESxyMqUECVYABWy3HOxVX-YSdF_fa4TWHjlrt7-t2T7iF0SfCU-Jqti82UpHHKjtCYxGkWUpzRYzTGPEpDzhM-QmfOrTHGhCTZKRrRKMp4xugYmVcLG1lCrSCQdRHIFQSFcZ01ed-Zpg4aHbRQtZ9m1bs9cdgaU-xAkA68XjX1KmglFEb6U-XHzkDducDUwQJsJevtOTrRsnRwcegT9P5w_zZ_DJcvi6f57TJUNMUsTJM8o0RJwmmEY81yjJVOaO5lzUlCOTCWRIxjpXhcMNBJ5N-ULM10rGQu6QRdD76tbb56cJ2ojFNQlrKGpnciohyTmKWcevRmQJVtnLOgRWtNJe1WECx2yQqfrNgn69mrg22fV1D8kb9RemA2AN-mhO3_TuL57mOw_AF9RYOf</recordid><startdate>202011</startdate><enddate>202011</enddate><creator>Hübner, F.</creator><creator>König, I.R.</creator><creator>Holtsche, M.M.</creator><creator>Zillikens, D.</creator><creator>Linder, R.</creator><creator>Schmidt, E.</creator><scope>24P</scope><scope>WIN</scope><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>7X8</scope><orcidid>https://orcid.org/0000-0003-2886-9499</orcidid></search><sort><creationdate>202011</creationdate><title>Prevalence and age distribution of pemphigus and pemphigoid diseases among paediatric patients in Germany</title><author>Hübner, F. ; König, I.R. ; Holtsche, M.M. ; Zillikens, D. ; Linder, R. ; Schmidt, E.</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c3607-65b831ca193204f7b00cf53b5b8f91539e7752790cc94d7ef52092a768f4caba3</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2020</creationdate><topic>Adolescent</topic><topic>Adult</topic><topic>Age Distribution</topic><topic>Autoimmune Diseases</topic><topic>Child</topic><topic>Germany - epidemiology</topic><topic>Humans</topic><topic>Pemphigoid, Bullous - epidemiology</topic><topic>Pemphigus - epidemiology</topic><topic>Prevalence</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Hübner, F.</creatorcontrib><creatorcontrib>König, I.R.</creatorcontrib><creatorcontrib>Holtsche, M.M.</creatorcontrib><creatorcontrib>Zillikens, D.</creatorcontrib><creatorcontrib>Linder, R.</creatorcontrib><creatorcontrib>Schmidt, E.</creatorcontrib><collection>Wiley Online Library Open Access</collection><collection>Wiley Online Library Free Content</collection><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>MEDLINE - Academic</collection><jtitle>Journal of the European Academy of Dermatology and Venereology</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Hübner, F.</au><au>König, I.R.</au><au>Holtsche, M.M.</au><au>Zillikens, D.</au><au>Linder, R.</au><au>Schmidt, E.</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Prevalence and age distribution of pemphigus and pemphigoid diseases among paediatric patients in Germany</atitle><jtitle>Journal of the European Academy of Dermatology and Venereology</jtitle><addtitle>J Eur Acad Dermatol Venereol</addtitle><date>2020-11</date><risdate>2020</risdate><volume>34</volume><issue>11</issue><spage>2600</spage><epage>2605</epage><pages>2600-2605</pages><issn>0926-9959</issn><eissn>1468-3083</eissn><abstract>Background
Autoimmune bullous diseases are rare and mostly occur in adults. Several cases and small case series have been described in children, but no systematic study about the prevalence of autoimmune bullous diseases (AIBD) in children is available.
Patients and methods
We analysed data of 1.7 million children insured in the largest German health insurance company based on the ICD‐10‐GM classification for the year 2015. Data were adjusted to the general German population based on the data of the Federal Statistical Office for the year 2015.
Results
The prevalence of AIBD was calculated to 101.1/million children in 2015, resulting in about 1351 patients below the age of 18 years in Germany. The highest prevalence of all AIBD was seen for pemphigus vulgaris (30.5/million children) followed by linear IgA disease (24.5/million children) and bullous pemphigoid (4.9/million children).
Conclusion
Autoimmune bullous diseases in minors are scarce but should be taken into consideration in patients with pruritus and/or blisters and erosions on the skin and/or mucous membranes. Treatment is challenging, and due to the rarity of AIBD in minors, the management of these disorders in this patient population is best performed in specialized centres in a multidisciplinary approach, including paediatric dermatologists or dermatologists and paediatricians.</abstract><cop>England</cop><pmid>32289873</pmid><doi>10.1111/jdv.16467</doi><tpages>6</tpages><orcidid>https://orcid.org/0000-0003-2886-9499</orcidid><oa>free_for_read</oa></addata></record> |
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| source | MEDLINE; Wiley Online Library Journals Frontfile Complete |
| subjects | Adolescent Adult Age Distribution Autoimmune Diseases Child Germany - epidemiology Humans Pemphigoid, Bullous - epidemiology Pemphigus - epidemiology Prevalence |
| title | Prevalence and age distribution of pemphigus and pemphigoid diseases among paediatric patients in Germany |
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