The Prevalence and Disease Characteristics of Generalized Pustular Psoriasis
Generalized pustular psoriasis (GPP) is a rare disease that has only recently benefited from a consistent definition and clinical coding standard. A lack of disease awareness combined with clinical similarities to other types of psoriasis have historically complicated the diagnosis of GPP. It is now...
Ausführliche Beschreibung
Gespeichert in:
| Autor*in: |
Zheng, Min [verfasserIn] |
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| Format: |
E-Artikel |
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| Sprache: |
Englisch |
| Erschienen: |
2022 |
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| Anmerkung: |
© The Author(s) 2022 |
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| Übergeordnetes Werk: |
Enthalten in: American journal of clinical dermatology - Berlin [u.a.] : Springer, 2000, 23(2022), Suppl 1 vom: Jan., Seite 5-12 |
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| Übergeordnetes Werk: |
volume:23 ; year:2022 ; number:Suppl 1 ; month:01 ; pages:5-12 |
| Links: |
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| DOI / URN: |
10.1007/s40257-021-00664-x |
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| Katalog-ID: |
SPR046102604 |
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| 520 | |a Generalized pustular psoriasis (GPP) is a rare disease that has only recently benefited from a consistent definition and clinical coding standard. A lack of disease awareness combined with clinical similarities to other types of psoriasis have historically complicated the diagnosis of GPP. It is now clear that GPP requires a differential diagnosis from psoriasis vulgaris (plaque psoriasis), and better understanding of the genetic characteristics underlying GPP may improve the accuracy of diagnoses in the future. GPP can present at any age but is most common in the fifth decade of life. There appears to be a female preponderance in GPP, although there is notable variability in prevalence by geographical region and between ethnicities. GPP is potentially life-threatening, associated with several serious complications, and may require emergency treatment, particularly for complications arising from systemic inflammation. As with many rare diseases, there are inherent challenges to understanding the epidemiology of GPP. In addition to small patient numbers, estimating the prevalence of rare diseases is further complicated by studies that use non-standardized methodologies and that are conducted in different populations. These complications in data gathering have led to marked variability in GPP case estimates by geographical region and between ethnicities. There is ongoing research into disease characteristics, and insights into regional measures of prevalence are essential to increasing our understanding of GPP. Graphical abstract | ||
| 700 | 1 | |a Jullien, Denis |4 aut | |
| 700 | 1 | |a Eyerich, Kilian |4 aut | |
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10.1007/s40257-021-00664-x doi (DE-627)SPR046102604 (SPR)s40257-021-00664-x-e DE-627 ger DE-627 rakwb eng Zheng, Min verfasserin aut The Prevalence and Disease Characteristics of Generalized Pustular Psoriasis 2022 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier © The Author(s) 2022 Generalized pustular psoriasis (GPP) is a rare disease that has only recently benefited from a consistent definition and clinical coding standard. A lack of disease awareness combined with clinical similarities to other types of psoriasis have historically complicated the diagnosis of GPP. It is now clear that GPP requires a differential diagnosis from psoriasis vulgaris (plaque psoriasis), and better understanding of the genetic characteristics underlying GPP may improve the accuracy of diagnoses in the future. GPP can present at any age but is most common in the fifth decade of life. There appears to be a female preponderance in GPP, although there is notable variability in prevalence by geographical region and between ethnicities. GPP is potentially life-threatening, associated with several serious complications, and may require emergency treatment, particularly for complications arising from systemic inflammation. As with many rare diseases, there are inherent challenges to understanding the epidemiology of GPP. In addition to small patient numbers, estimating the prevalence of rare diseases is further complicated by studies that use non-standardized methodologies and that are conducted in different populations. These complications in data gathering have led to marked variability in GPP case estimates by geographical region and between ethnicities. There is ongoing research into disease characteristics, and insights into regional measures of prevalence are essential to increasing our understanding of GPP. Graphical abstract Jullien, Denis aut Eyerich, Kilian aut Enthalten in American journal of clinical dermatology Berlin [u.a.] : Springer, 2000 23(2022), Suppl 1 vom: Jan., Seite 5-12 (DE-627)327644176 (DE-600)2043675-0 1179-1888 nnns volume:23 year:2022 number:Suppl 1 month:01 pages:5-12 https://dx.doi.org/10.1007/s40257-021-00664-x kostenfrei Volltext GBV_USEFLAG_A SYSFLAG_A GBV_SPRINGER SSG-OLC-PHA GBV_ILN_11 GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_31 GBV_ILN_32 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_70 GBV_ILN_73 GBV_ILN_74 GBV_ILN_90 GBV_ILN_95 GBV_ILN_100 GBV_ILN_101 GBV_ILN_105 GBV_ILN_110 GBV_ILN_120 GBV_ILN_138 GBV_ILN_150 GBV_ILN_151 GBV_ILN_152 GBV_ILN_161 GBV_ILN_170 GBV_ILN_171 GBV_ILN_187 GBV_ILN_213 GBV_ILN_224 GBV_ILN_230 GBV_ILN_250 GBV_ILN_266 GBV_ILN_281 GBV_ILN_285 GBV_ILN_293 GBV_ILN_370 GBV_ILN_602 GBV_ILN_636 GBV_ILN_702 GBV_ILN_2001 GBV_ILN_2003 GBV_ILN_2004 GBV_ILN_2005 GBV_ILN_2006 GBV_ILN_2007 GBV_ILN_2008 GBV_ILN_2009 GBV_ILN_2010 GBV_ILN_2011 GBV_ILN_2014 GBV_ILN_2015 GBV_ILN_2020 GBV_ILN_2021 GBV_ILN_2025 GBV_ILN_2026 GBV_ILN_2027 GBV_ILN_2031 GBV_ILN_2034 GBV_ILN_2037 GBV_ILN_2038 GBV_ILN_2039 GBV_ILN_2044 GBV_ILN_2048 GBV_ILN_2049 GBV_ILN_2050 GBV_ILN_2055 GBV_ILN_2056 GBV_ILN_2057 GBV_ILN_2059 GBV_ILN_2061 GBV_ILN_2064 GBV_ILN_2065 GBV_ILN_2068 GBV_ILN_2088 GBV_ILN_2093 GBV_ILN_2106 GBV_ILN_2107 GBV_ILN_2108 GBV_ILN_2110 GBV_ILN_2111 GBV_ILN_2112 GBV_ILN_2113 GBV_ILN_2118 GBV_ILN_2122 GBV_ILN_2129 GBV_ILN_2143 GBV_ILN_2144 GBV_ILN_2147 GBV_ILN_2148 GBV_ILN_2152 GBV_ILN_2153 GBV_ILN_2188 GBV_ILN_2190 GBV_ILN_2232 GBV_ILN_2336 GBV_ILN_2446 GBV_ILN_2470 GBV_ILN_2472 GBV_ILN_2507 GBV_ILN_2522 GBV_ILN_2548 GBV_ILN_4035 GBV_ILN_4037 GBV_ILN_4046 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4242 GBV_ILN_4246 GBV_ILN_4249 GBV_ILN_4251 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4326 GBV_ILN_4328 GBV_ILN_4333 GBV_ILN_4334 GBV_ILN_4335 GBV_ILN_4336 GBV_ILN_4338 GBV_ILN_4393 GBV_ILN_4700 AR 23 2022 Suppl 1 01 5-12 |
| spelling |
10.1007/s40257-021-00664-x doi (DE-627)SPR046102604 (SPR)s40257-021-00664-x-e DE-627 ger DE-627 rakwb eng Zheng, Min verfasserin aut The Prevalence and Disease Characteristics of Generalized Pustular Psoriasis 2022 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier © The Author(s) 2022 Generalized pustular psoriasis (GPP) is a rare disease that has only recently benefited from a consistent definition and clinical coding standard. A lack of disease awareness combined with clinical similarities to other types of psoriasis have historically complicated the diagnosis of GPP. It is now clear that GPP requires a differential diagnosis from psoriasis vulgaris (plaque psoriasis), and better understanding of the genetic characteristics underlying GPP may improve the accuracy of diagnoses in the future. GPP can present at any age but is most common in the fifth decade of life. There appears to be a female preponderance in GPP, although there is notable variability in prevalence by geographical region and between ethnicities. GPP is potentially life-threatening, associated with several serious complications, and may require emergency treatment, particularly for complications arising from systemic inflammation. As with many rare diseases, there are inherent challenges to understanding the epidemiology of GPP. In addition to small patient numbers, estimating the prevalence of rare diseases is further complicated by studies that use non-standardized methodologies and that are conducted in different populations. These complications in data gathering have led to marked variability in GPP case estimates by geographical region and between ethnicities. There is ongoing research into disease characteristics, and insights into regional measures of prevalence are essential to increasing our understanding of GPP. Graphical abstract Jullien, Denis aut Eyerich, Kilian aut Enthalten in American journal of clinical dermatology Berlin [u.a.] : Springer, 2000 23(2022), Suppl 1 vom: Jan., Seite 5-12 (DE-627)327644176 (DE-600)2043675-0 1179-1888 nnns volume:23 year:2022 number:Suppl 1 month:01 pages:5-12 https://dx.doi.org/10.1007/s40257-021-00664-x kostenfrei Volltext GBV_USEFLAG_A SYSFLAG_A GBV_SPRINGER SSG-OLC-PHA GBV_ILN_11 GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_31 GBV_ILN_32 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_70 GBV_ILN_73 GBV_ILN_74 GBV_ILN_90 GBV_ILN_95 GBV_ILN_100 GBV_ILN_101 GBV_ILN_105 GBV_ILN_110 GBV_ILN_120 GBV_ILN_138 GBV_ILN_150 GBV_ILN_151 GBV_ILN_152 GBV_ILN_161 GBV_ILN_170 GBV_ILN_171 GBV_ILN_187 GBV_ILN_213 GBV_ILN_224 GBV_ILN_230 GBV_ILN_250 GBV_ILN_266 GBV_ILN_281 GBV_ILN_285 GBV_ILN_293 GBV_ILN_370 GBV_ILN_602 GBV_ILN_636 GBV_ILN_702 GBV_ILN_2001 GBV_ILN_2003 GBV_ILN_2004 GBV_ILN_2005 GBV_ILN_2006 GBV_ILN_2007 GBV_ILN_2008 GBV_ILN_2009 GBV_ILN_2010 GBV_ILN_2011 GBV_ILN_2014 GBV_ILN_2015 GBV_ILN_2020 GBV_ILN_2021 GBV_ILN_2025 GBV_ILN_2026 GBV_ILN_2027 GBV_ILN_2031 GBV_ILN_2034 GBV_ILN_2037 GBV_ILN_2038 GBV_ILN_2039 GBV_ILN_2044 GBV_ILN_2048 GBV_ILN_2049 GBV_ILN_2050 GBV_ILN_2055 GBV_ILN_2056 GBV_ILN_2057 GBV_ILN_2059 GBV_ILN_2061 GBV_ILN_2064 GBV_ILN_2065 GBV_ILN_2068 GBV_ILN_2088 GBV_ILN_2093 GBV_ILN_2106 GBV_ILN_2107 GBV_ILN_2108 GBV_ILN_2110 GBV_ILN_2111 GBV_ILN_2112 GBV_ILN_2113 GBV_ILN_2118 GBV_ILN_2122 GBV_ILN_2129 GBV_ILN_2143 GBV_ILN_2144 GBV_ILN_2147 GBV_ILN_2148 GBV_ILN_2152 GBV_ILN_2153 GBV_ILN_2188 GBV_ILN_2190 GBV_ILN_2232 GBV_ILN_2336 GBV_ILN_2446 GBV_ILN_2470 GBV_ILN_2472 GBV_ILN_2507 GBV_ILN_2522 GBV_ILN_2548 GBV_ILN_4035 GBV_ILN_4037 GBV_ILN_4046 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4242 GBV_ILN_4246 GBV_ILN_4249 GBV_ILN_4251 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4326 GBV_ILN_4328 GBV_ILN_4333 GBV_ILN_4334 GBV_ILN_4335 GBV_ILN_4336 GBV_ILN_4338 GBV_ILN_4393 GBV_ILN_4700 AR 23 2022 Suppl 1 01 5-12 |
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10.1007/s40257-021-00664-x doi (DE-627)SPR046102604 (SPR)s40257-021-00664-x-e DE-627 ger DE-627 rakwb eng Zheng, Min verfasserin aut The Prevalence and Disease Characteristics of Generalized Pustular Psoriasis 2022 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier © The Author(s) 2022 Generalized pustular psoriasis (GPP) is a rare disease that has only recently benefited from a consistent definition and clinical coding standard. A lack of disease awareness combined with clinical similarities to other types of psoriasis have historically complicated the diagnosis of GPP. It is now clear that GPP requires a differential diagnosis from psoriasis vulgaris (plaque psoriasis), and better understanding of the genetic characteristics underlying GPP may improve the accuracy of diagnoses in the future. GPP can present at any age but is most common in the fifth decade of life. There appears to be a female preponderance in GPP, although there is notable variability in prevalence by geographical region and between ethnicities. GPP is potentially life-threatening, associated with several serious complications, and may require emergency treatment, particularly for complications arising from systemic inflammation. As with many rare diseases, there are inherent challenges to understanding the epidemiology of GPP. In addition to small patient numbers, estimating the prevalence of rare diseases is further complicated by studies that use non-standardized methodologies and that are conducted in different populations. These complications in data gathering have led to marked variability in GPP case estimates by geographical region and between ethnicities. There is ongoing research into disease characteristics, and insights into regional measures of prevalence are essential to increasing our understanding of GPP. Graphical abstract Jullien, Denis aut Eyerich, Kilian aut Enthalten in American journal of clinical dermatology Berlin [u.a.] : Springer, 2000 23(2022), Suppl 1 vom: Jan., Seite 5-12 (DE-627)327644176 (DE-600)2043675-0 1179-1888 nnns volume:23 year:2022 number:Suppl 1 month:01 pages:5-12 https://dx.doi.org/10.1007/s40257-021-00664-x kostenfrei Volltext GBV_USEFLAG_A SYSFLAG_A GBV_SPRINGER SSG-OLC-PHA GBV_ILN_11 GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_31 GBV_ILN_32 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_70 GBV_ILN_73 GBV_ILN_74 GBV_ILN_90 GBV_ILN_95 GBV_ILN_100 GBV_ILN_101 GBV_ILN_105 GBV_ILN_110 GBV_ILN_120 GBV_ILN_138 GBV_ILN_150 GBV_ILN_151 GBV_ILN_152 GBV_ILN_161 GBV_ILN_170 GBV_ILN_171 GBV_ILN_187 GBV_ILN_213 GBV_ILN_224 GBV_ILN_230 GBV_ILN_250 GBV_ILN_266 GBV_ILN_281 GBV_ILN_285 GBV_ILN_293 GBV_ILN_370 GBV_ILN_602 GBV_ILN_636 GBV_ILN_702 GBV_ILN_2001 GBV_ILN_2003 GBV_ILN_2004 GBV_ILN_2005 GBV_ILN_2006 GBV_ILN_2007 GBV_ILN_2008 GBV_ILN_2009 GBV_ILN_2010 GBV_ILN_2011 GBV_ILN_2014 GBV_ILN_2015 GBV_ILN_2020 GBV_ILN_2021 GBV_ILN_2025 GBV_ILN_2026 GBV_ILN_2027 GBV_ILN_2031 GBV_ILN_2034 GBV_ILN_2037 GBV_ILN_2038 GBV_ILN_2039 GBV_ILN_2044 GBV_ILN_2048 GBV_ILN_2049 GBV_ILN_2050 GBV_ILN_2055 GBV_ILN_2056 GBV_ILN_2057 GBV_ILN_2059 GBV_ILN_2061 GBV_ILN_2064 GBV_ILN_2065 GBV_ILN_2068 GBV_ILN_2088 GBV_ILN_2093 GBV_ILN_2106 GBV_ILN_2107 GBV_ILN_2108 GBV_ILN_2110 GBV_ILN_2111 GBV_ILN_2112 GBV_ILN_2113 GBV_ILN_2118 GBV_ILN_2122 GBV_ILN_2129 GBV_ILN_2143 GBV_ILN_2144 GBV_ILN_2147 GBV_ILN_2148 GBV_ILN_2152 GBV_ILN_2153 GBV_ILN_2188 GBV_ILN_2190 GBV_ILN_2232 GBV_ILN_2336 GBV_ILN_2446 GBV_ILN_2470 GBV_ILN_2472 GBV_ILN_2507 GBV_ILN_2522 GBV_ILN_2548 GBV_ILN_4035 GBV_ILN_4037 GBV_ILN_4046 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4242 GBV_ILN_4246 GBV_ILN_4249 GBV_ILN_4251 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4326 GBV_ILN_4328 GBV_ILN_4333 GBV_ILN_4334 GBV_ILN_4335 GBV_ILN_4336 GBV_ILN_4338 GBV_ILN_4393 GBV_ILN_4700 AR 23 2022 Suppl 1 01 5-12 |
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10.1007/s40257-021-00664-x doi (DE-627)SPR046102604 (SPR)s40257-021-00664-x-e DE-627 ger DE-627 rakwb eng Zheng, Min verfasserin aut The Prevalence and Disease Characteristics of Generalized Pustular Psoriasis 2022 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier © The Author(s) 2022 Generalized pustular psoriasis (GPP) is a rare disease that has only recently benefited from a consistent definition and clinical coding standard. A lack of disease awareness combined with clinical similarities to other types of psoriasis have historically complicated the diagnosis of GPP. It is now clear that GPP requires a differential diagnosis from psoriasis vulgaris (plaque psoriasis), and better understanding of the genetic characteristics underlying GPP may improve the accuracy of diagnoses in the future. GPP can present at any age but is most common in the fifth decade of life. There appears to be a female preponderance in GPP, although there is notable variability in prevalence by geographical region and between ethnicities. GPP is potentially life-threatening, associated with several serious complications, and may require emergency treatment, particularly for complications arising from systemic inflammation. As with many rare diseases, there are inherent challenges to understanding the epidemiology of GPP. In addition to small patient numbers, estimating the prevalence of rare diseases is further complicated by studies that use non-standardized methodologies and that are conducted in different populations. These complications in data gathering have led to marked variability in GPP case estimates by geographical region and between ethnicities. There is ongoing research into disease characteristics, and insights into regional measures of prevalence are essential to increasing our understanding of GPP. Graphical abstract Jullien, Denis aut Eyerich, Kilian aut Enthalten in American journal of clinical dermatology Berlin [u.a.] : Springer, 2000 23(2022), Suppl 1 vom: Jan., Seite 5-12 (DE-627)327644176 (DE-600)2043675-0 1179-1888 nnns volume:23 year:2022 number:Suppl 1 month:01 pages:5-12 https://dx.doi.org/10.1007/s40257-021-00664-x kostenfrei Volltext GBV_USEFLAG_A SYSFLAG_A GBV_SPRINGER SSG-OLC-PHA GBV_ILN_11 GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_31 GBV_ILN_32 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_70 GBV_ILN_73 GBV_ILN_74 GBV_ILN_90 GBV_ILN_95 GBV_ILN_100 GBV_ILN_101 GBV_ILN_105 GBV_ILN_110 GBV_ILN_120 GBV_ILN_138 GBV_ILN_150 GBV_ILN_151 GBV_ILN_152 GBV_ILN_161 GBV_ILN_170 GBV_ILN_171 GBV_ILN_187 GBV_ILN_213 GBV_ILN_224 GBV_ILN_230 GBV_ILN_250 GBV_ILN_266 GBV_ILN_281 GBV_ILN_285 GBV_ILN_293 GBV_ILN_370 GBV_ILN_602 GBV_ILN_636 GBV_ILN_702 GBV_ILN_2001 GBV_ILN_2003 GBV_ILN_2004 GBV_ILN_2005 GBV_ILN_2006 GBV_ILN_2007 GBV_ILN_2008 GBV_ILN_2009 GBV_ILN_2010 GBV_ILN_2011 GBV_ILN_2014 GBV_ILN_2015 GBV_ILN_2020 GBV_ILN_2021 GBV_ILN_2025 GBV_ILN_2026 GBV_ILN_2027 GBV_ILN_2031 GBV_ILN_2034 GBV_ILN_2037 GBV_ILN_2038 GBV_ILN_2039 GBV_ILN_2044 GBV_ILN_2048 GBV_ILN_2049 GBV_ILN_2050 GBV_ILN_2055 GBV_ILN_2056 GBV_ILN_2057 GBV_ILN_2059 GBV_ILN_2061 GBV_ILN_2064 GBV_ILN_2065 GBV_ILN_2068 GBV_ILN_2088 GBV_ILN_2093 GBV_ILN_2106 GBV_ILN_2107 GBV_ILN_2108 GBV_ILN_2110 GBV_ILN_2111 GBV_ILN_2112 GBV_ILN_2113 GBV_ILN_2118 GBV_ILN_2122 GBV_ILN_2129 GBV_ILN_2143 GBV_ILN_2144 GBV_ILN_2147 GBV_ILN_2148 GBV_ILN_2152 GBV_ILN_2153 GBV_ILN_2188 GBV_ILN_2190 GBV_ILN_2232 GBV_ILN_2336 GBV_ILN_2446 GBV_ILN_2470 GBV_ILN_2472 GBV_ILN_2507 GBV_ILN_2522 GBV_ILN_2548 GBV_ILN_4035 GBV_ILN_4037 GBV_ILN_4046 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4242 GBV_ILN_4246 GBV_ILN_4249 GBV_ILN_4251 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4326 GBV_ILN_4328 GBV_ILN_4333 GBV_ILN_4334 GBV_ILN_4335 GBV_ILN_4336 GBV_ILN_4338 GBV_ILN_4393 GBV_ILN_4700 AR 23 2022 Suppl 1 01 5-12 |
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10.1007/s40257-021-00664-x doi (DE-627)SPR046102604 (SPR)s40257-021-00664-x-e DE-627 ger DE-627 rakwb eng Zheng, Min verfasserin aut The Prevalence and Disease Characteristics of Generalized Pustular Psoriasis 2022 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier © The Author(s) 2022 Generalized pustular psoriasis (GPP) is a rare disease that has only recently benefited from a consistent definition and clinical coding standard. A lack of disease awareness combined with clinical similarities to other types of psoriasis have historically complicated the diagnosis of GPP. It is now clear that GPP requires a differential diagnosis from psoriasis vulgaris (plaque psoriasis), and better understanding of the genetic characteristics underlying GPP may improve the accuracy of diagnoses in the future. GPP can present at any age but is most common in the fifth decade of life. There appears to be a female preponderance in GPP, although there is notable variability in prevalence by geographical region and between ethnicities. GPP is potentially life-threatening, associated with several serious complications, and may require emergency treatment, particularly for complications arising from systemic inflammation. As with many rare diseases, there are inherent challenges to understanding the epidemiology of GPP. In addition to small patient numbers, estimating the prevalence of rare diseases is further complicated by studies that use non-standardized methodologies and that are conducted in different populations. These complications in data gathering have led to marked variability in GPP case estimates by geographical region and between ethnicities. There is ongoing research into disease characteristics, and insights into regional measures of prevalence are essential to increasing our understanding of GPP. Graphical abstract Jullien, Denis aut Eyerich, Kilian aut Enthalten in American journal of clinical dermatology Berlin [u.a.] : Springer, 2000 23(2022), Suppl 1 vom: Jan., Seite 5-12 (DE-627)327644176 (DE-600)2043675-0 1179-1888 nnns volume:23 year:2022 number:Suppl 1 month:01 pages:5-12 https://dx.doi.org/10.1007/s40257-021-00664-x kostenfrei Volltext GBV_USEFLAG_A SYSFLAG_A GBV_SPRINGER SSG-OLC-PHA GBV_ILN_11 GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_31 GBV_ILN_32 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_70 GBV_ILN_73 GBV_ILN_74 GBV_ILN_90 GBV_ILN_95 GBV_ILN_100 GBV_ILN_101 GBV_ILN_105 GBV_ILN_110 GBV_ILN_120 GBV_ILN_138 GBV_ILN_150 GBV_ILN_151 GBV_ILN_152 GBV_ILN_161 GBV_ILN_170 GBV_ILN_171 GBV_ILN_187 GBV_ILN_213 GBV_ILN_224 GBV_ILN_230 GBV_ILN_250 GBV_ILN_266 GBV_ILN_281 GBV_ILN_285 GBV_ILN_293 GBV_ILN_370 GBV_ILN_602 GBV_ILN_636 GBV_ILN_702 GBV_ILN_2001 GBV_ILN_2003 GBV_ILN_2004 GBV_ILN_2005 GBV_ILN_2006 GBV_ILN_2007 GBV_ILN_2008 GBV_ILN_2009 GBV_ILN_2010 GBV_ILN_2011 GBV_ILN_2014 GBV_ILN_2015 GBV_ILN_2020 GBV_ILN_2021 GBV_ILN_2025 GBV_ILN_2026 GBV_ILN_2027 GBV_ILN_2031 GBV_ILN_2034 GBV_ILN_2037 GBV_ILN_2038 GBV_ILN_2039 GBV_ILN_2044 GBV_ILN_2048 GBV_ILN_2049 GBV_ILN_2050 GBV_ILN_2055 GBV_ILN_2056 GBV_ILN_2057 GBV_ILN_2059 GBV_ILN_2061 GBV_ILN_2064 GBV_ILN_2065 GBV_ILN_2068 GBV_ILN_2088 GBV_ILN_2093 GBV_ILN_2106 GBV_ILN_2107 GBV_ILN_2108 GBV_ILN_2110 GBV_ILN_2111 GBV_ILN_2112 GBV_ILN_2113 GBV_ILN_2118 GBV_ILN_2122 GBV_ILN_2129 GBV_ILN_2143 GBV_ILN_2144 GBV_ILN_2147 GBV_ILN_2148 GBV_ILN_2152 GBV_ILN_2153 GBV_ILN_2188 GBV_ILN_2190 GBV_ILN_2232 GBV_ILN_2336 GBV_ILN_2446 GBV_ILN_2470 GBV_ILN_2472 GBV_ILN_2507 GBV_ILN_2522 GBV_ILN_2548 GBV_ILN_4035 GBV_ILN_4037 GBV_ILN_4046 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4242 GBV_ILN_4246 GBV_ILN_4249 GBV_ILN_4251 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4326 GBV_ILN_4328 GBV_ILN_4333 GBV_ILN_4334 GBV_ILN_4335 GBV_ILN_4336 GBV_ILN_4338 GBV_ILN_4393 GBV_ILN_4700 AR 23 2022 Suppl 1 01 5-12 |
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prevalence and disease characteristics of generalized pustular psoriasis |
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The Prevalence and Disease Characteristics of Generalized Pustular Psoriasis |
| abstract |
Generalized pustular psoriasis (GPP) is a rare disease that has only recently benefited from a consistent definition and clinical coding standard. A lack of disease awareness combined with clinical similarities to other types of psoriasis have historically complicated the diagnosis of GPP. It is now clear that GPP requires a differential diagnosis from psoriasis vulgaris (plaque psoriasis), and better understanding of the genetic characteristics underlying GPP may improve the accuracy of diagnoses in the future. GPP can present at any age but is most common in the fifth decade of life. There appears to be a female preponderance in GPP, although there is notable variability in prevalence by geographical region and between ethnicities. GPP is potentially life-threatening, associated with several serious complications, and may require emergency treatment, particularly for complications arising from systemic inflammation. As with many rare diseases, there are inherent challenges to understanding the epidemiology of GPP. In addition to small patient numbers, estimating the prevalence of rare diseases is further complicated by studies that use non-standardized methodologies and that are conducted in different populations. These complications in data gathering have led to marked variability in GPP case estimates by geographical region and between ethnicities. There is ongoing research into disease characteristics, and insights into regional measures of prevalence are essential to increasing our understanding of GPP. Graphical abstract © The Author(s) 2022 |
| abstractGer |
Generalized pustular psoriasis (GPP) is a rare disease that has only recently benefited from a consistent definition and clinical coding standard. A lack of disease awareness combined with clinical similarities to other types of psoriasis have historically complicated the diagnosis of GPP. It is now clear that GPP requires a differential diagnosis from psoriasis vulgaris (plaque psoriasis), and better understanding of the genetic characteristics underlying GPP may improve the accuracy of diagnoses in the future. GPP can present at any age but is most common in the fifth decade of life. There appears to be a female preponderance in GPP, although there is notable variability in prevalence by geographical region and between ethnicities. GPP is potentially life-threatening, associated with several serious complications, and may require emergency treatment, particularly for complications arising from systemic inflammation. As with many rare diseases, there are inherent challenges to understanding the epidemiology of GPP. In addition to small patient numbers, estimating the prevalence of rare diseases is further complicated by studies that use non-standardized methodologies and that are conducted in different populations. These complications in data gathering have led to marked variability in GPP case estimates by geographical region and between ethnicities. There is ongoing research into disease characteristics, and insights into regional measures of prevalence are essential to increasing our understanding of GPP. Graphical abstract © The Author(s) 2022 |
| abstract_unstemmed |
Generalized pustular psoriasis (GPP) is a rare disease that has only recently benefited from a consistent definition and clinical coding standard. A lack of disease awareness combined with clinical similarities to other types of psoriasis have historically complicated the diagnosis of GPP. It is now clear that GPP requires a differential diagnosis from psoriasis vulgaris (plaque psoriasis), and better understanding of the genetic characteristics underlying GPP may improve the accuracy of diagnoses in the future. GPP can present at any age but is most common in the fifth decade of life. There appears to be a female preponderance in GPP, although there is notable variability in prevalence by geographical region and between ethnicities. GPP is potentially life-threatening, associated with several serious complications, and may require emergency treatment, particularly for complications arising from systemic inflammation. As with many rare diseases, there are inherent challenges to understanding the epidemiology of GPP. In addition to small patient numbers, estimating the prevalence of rare diseases is further complicated by studies that use non-standardized methodologies and that are conducted in different populations. These complications in data gathering have led to marked variability in GPP case estimates by geographical region and between ethnicities. There is ongoing research into disease characteristics, and insights into regional measures of prevalence are essential to increasing our understanding of GPP. Graphical abstract © The Author(s) 2022 |
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The Prevalence and Disease Characteristics of Generalized Pustular Psoriasis |
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2024-07-03T20:22:17.552Z |
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