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Bullous lupus as the first manifestation of systemic lupus erythematosus in the pediatric population: A diagnostic challenge in daily practice
Introduction In the GLADEL cohort, the bullous lupus (BSLE) prevalence was 0.41%. However, literature on pediatric BSLE is scarce. This study described the clinical, histological, and immunological characteristics and the treatment response in a series of children with BSLE as the first clinical man...
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| Published in: | Lupus 2020-12, Vol.29 (14), p.1937-1942 |
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| container_end_page | 1942 |
| container_issue | 14 |
| container_start_page | 1937 |
| container_title | Lupus |
| container_volume | 29 |
| creator | Torres Saavedra, Fabio Andrés Campo, Lyna Ramirez Mendez, Monica Velasquez Barreneche, Natalia Mejia Suaza, Gloria Andrea Vargas Restrepo, Juan David Ruiz Martinez-Gomez, Manuel |
| description | Introduction
In the GLADEL cohort, the bullous lupus (BSLE) prevalence was 0.41%. However, literature on pediatric BSLE is scarce. This study described the clinical, histological, and immunological characteristics and the treatment response in a series of children with BSLE as the first clinical manifestation of pediatric SLE.
Methods
The clinical, histological, and immunological characteristics of a series of 5 cases of BSLE between 2010-2019 from two reference centers in Colombia were analyzed.
Results
All cases had bullous lesions that resolved with residual hypopigmentation. One had a focal seizure, and another arthritis with thrombocytopenia. Two had transient proteinuria with normal urinalysis. Anti-nuclear antibody titers ranged from 1:160 to 1:2560, and four were anti-dsDNA (+). Five patients had anti-RNP antibodies, and four anti-Sm antibodies. All had low C3, and 80% low C4 counts; 80% had erythrocyte sedimentation rate (ESR) ≥20 mm/hour and 60% had C-reactive protein (CRP) ≥0.5 mg/dL. All patients responded to glucocorticoids and dapsone. Histology reports and direct immunofluorescence (DIF) test showed subepidermal blisters with neutrophils in the papillary dermis and linear deposits of Igs/complement proteins in 80% of the skin biopsies. IgG/IgM was present in 5 samples. IgA was positive in 60% and C3 in 80%.
Conclusions
In this pediatric series, BSLE tends to have a monophasic behavior associated with neuropsychiatric, skeletal, and hematological involvement in 40% of the patients, and with good prognosis. |
| doi_str_mv | 10.1177/0961203320950814 |
| format | article |
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In the GLADEL cohort, the bullous lupus (BSLE) prevalence was 0.41%. However, literature on pediatric BSLE is scarce. This study described the clinical, histological, and immunological characteristics and the treatment response in a series of children with BSLE as the first clinical manifestation of pediatric SLE.
Methods
The clinical, histological, and immunological characteristics of a series of 5 cases of BSLE between 2010-2019 from two reference centers in Colombia were analyzed.
Results
All cases had bullous lesions that resolved with residual hypopigmentation. One had a focal seizure, and another arthritis with thrombocytopenia. Two had transient proteinuria with normal urinalysis. Anti-nuclear antibody titers ranged from 1:160 to 1:2560, and four were anti-dsDNA (+). Five patients had anti-RNP antibodies, and four anti-Sm antibodies. All had low C3, and 80% low C4 counts; 80% had erythrocyte sedimentation rate (ESR) ≥20 mm/hour and 60% had C-reactive protein (CRP) ≥0.5 mg/dL. All patients responded to glucocorticoids and dapsone. Histology reports and direct immunofluorescence (DIF) test showed subepidermal blisters with neutrophils in the papillary dermis and linear deposits of Igs/complement proteins in 80% of the skin biopsies. IgG/IgM was present in 5 samples. IgA was positive in 60% and C3 in 80%.
Conclusions
In this pediatric series, BSLE tends to have a monophasic behavior associated with neuropsychiatric, skeletal, and hematological involvement in 40% of the patients, and with good prognosis.</description><identifier>ISSN: 0961-2033</identifier><identifier>EISSN: 1477-0962</identifier><identifier>DOI: 10.1177/0961203320950814</identifier><language>eng</language><publisher>London, England: SAGE Publications</publisher><subject>Anti-DNA antibodies ; Arthritis ; C-reactive protein ; Complement component C3 ; Complement component C4 ; Dapsone ; Dermis ; Erythrocyte sedimentation rate ; Glucocorticoids ; Immunofluorescence ; Immunoglobulin A ; Immunoglobulin G ; Immunoglobulin M ; Immunology ; Leukocytes (neutrophilic) ; Lupus ; Patients ; Pediatrics ; Pigmentation ; Proteinuria ; Systemic lupus erythematosus ; Thrombocytopenia ; Urinalysis</subject><ispartof>Lupus, 2020-12, Vol.29 (14), p.1937-1942</ispartof><rights>The Author(s) 2020</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c342t-ce46d0940aaf92cc83f4cc0b41e7e0b5a5594d16310c6d77df65c90ff5b285f03</citedby><cites>FETCH-LOGICAL-c342t-ce46d0940aaf92cc83f4cc0b41e7e0b5a5594d16310c6d77df65c90ff5b285f03</cites><orcidid>0000-0003-0385-8160 ; 0000-0001-6593-4275 ; 0000-0003-3638-3771 ; 0000-0002-2370-2916</orcidid></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><link.rule.ids>315,786,790,27957,27958</link.rule.ids></links><search><creatorcontrib>Torres Saavedra, Fabio Andrés</creatorcontrib><creatorcontrib>Campo, Lyna Ramirez</creatorcontrib><creatorcontrib>Mendez, Monica Velasquez</creatorcontrib><creatorcontrib>Barreneche, Natalia Mejia</creatorcontrib><creatorcontrib>Suaza, Gloria Andrea Vargas</creatorcontrib><creatorcontrib>Restrepo, Juan David Ruiz</creatorcontrib><creatorcontrib>Martinez-Gomez, Manuel</creatorcontrib><title>Bullous lupus as the first manifestation of systemic lupus erythematosus in the pediatric population: A diagnostic challenge in daily practice</title><title>Lupus</title><description>Introduction
In the GLADEL cohort, the bullous lupus (BSLE) prevalence was 0.41%. However, literature on pediatric BSLE is scarce. This study described the clinical, histological, and immunological characteristics and the treatment response in a series of children with BSLE as the first clinical manifestation of pediatric SLE.
Methods
The clinical, histological, and immunological characteristics of a series of 5 cases of BSLE between 2010-2019 from two reference centers in Colombia were analyzed.
Results
All cases had bullous lesions that resolved with residual hypopigmentation. One had a focal seizure, and another arthritis with thrombocytopenia. Two had transient proteinuria with normal urinalysis. Anti-nuclear antibody titers ranged from 1:160 to 1:2560, and four were anti-dsDNA (+). Five patients had anti-RNP antibodies, and four anti-Sm antibodies. All had low C3, and 80% low C4 counts; 80% had erythrocyte sedimentation rate (ESR) ≥20 mm/hour and 60% had C-reactive protein (CRP) ≥0.5 mg/dL. All patients responded to glucocorticoids and dapsone. Histology reports and direct immunofluorescence (DIF) test showed subepidermal blisters with neutrophils in the papillary dermis and linear deposits of Igs/complement proteins in 80% of the skin biopsies. IgG/IgM was present in 5 samples. IgA was positive in 60% and C3 in 80%.
Conclusions
In this pediatric series, BSLE tends to have a monophasic behavior associated with neuropsychiatric, skeletal, and hematological involvement in 40% of the patients, and with good prognosis.</description><subject>Anti-DNA antibodies</subject><subject>Arthritis</subject><subject>C-reactive protein</subject><subject>Complement component C3</subject><subject>Complement component C4</subject><subject>Dapsone</subject><subject>Dermis</subject><subject>Erythrocyte sedimentation rate</subject><subject>Glucocorticoids</subject><subject>Immunofluorescence</subject><subject>Immunoglobulin A</subject><subject>Immunoglobulin G</subject><subject>Immunoglobulin M</subject><subject>Immunology</subject><subject>Leukocytes (neutrophilic)</subject><subject>Lupus</subject><subject>Patients</subject><subject>Pediatrics</subject><subject>Pigmentation</subject><subject>Proteinuria</subject><subject>Systemic lupus erythematosus</subject><subject>Thrombocytopenia</subject><subject>Urinalysis</subject><issn>0961-2033</issn><issn>1477-0962</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2020</creationdate><recordtype>article</recordtype><recordid>eNp1kctKAzEUhoMoWKt7lwE3bkZPLjPpuKvFGwhudD2kmaRNyUzGJLPoS_jMplYQCm4Scv7vP5cchC4J3BAixC3UFaHAGIW6hBnhR2hCuBBFjtNjNNnJxU4_RWcxbgCAkbqaoK_70Tk_RuzGIZ8y4rTW2NgQE-5kb42OSSbre-wNjtuYdGfVL6zDNsOdTD7ml-1_rINurUwhQ4MfRvfjvcNznKOr3seUBbWWzul-pXeeVlq3xUOQKkv6HJ0Y6aK--L2n6OPx4X3xXLy-Pb0s5q-FYpymQmletVBzkNLUVKkZM1wpWHKihYZlKcuy5i2pGAFVtUK0pipVDcaUSzorDbAput7nHYL_HPOMTWej0s7JXuffaChnggMlJcvo1QG68WPoc3eZqmiVqzCRKdhTKvgYgzbNEGwnw7Yh0OwW1BwuKFuKvSXKlf5L-i__DQ6Akm0</recordid><startdate>202012</startdate><enddate>202012</enddate><creator>Torres Saavedra, Fabio Andrés</creator><creator>Campo, Lyna Ramirez</creator><creator>Mendez, Monica Velasquez</creator><creator>Barreneche, Natalia Mejia</creator><creator>Suaza, Gloria Andrea Vargas</creator><creator>Restrepo, Juan David Ruiz</creator><creator>Martinez-Gomez, Manuel</creator><general>SAGE Publications</general><general>Sage Publications Ltd</general><scope>AAYXX</scope><scope>CITATION</scope><scope>7T5</scope><scope>H94</scope><scope>K9.</scope><scope>7X8</scope><orcidid>https://orcid.org/0000-0003-0385-8160</orcidid><orcidid>https://orcid.org/0000-0001-6593-4275</orcidid><orcidid>https://orcid.org/0000-0003-3638-3771</orcidid><orcidid>https://orcid.org/0000-0002-2370-2916</orcidid></search><sort><creationdate>202012</creationdate><title>Bullous lupus as the first manifestation of systemic lupus erythematosus in the pediatric population: A diagnostic challenge in daily practice</title><author>Torres Saavedra, Fabio Andrés ; Campo, Lyna Ramirez ; Mendez, Monica Velasquez ; Barreneche, Natalia Mejia ; Suaza, Gloria Andrea Vargas ; Restrepo, Juan David Ruiz ; Martinez-Gomez, Manuel</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c342t-ce46d0940aaf92cc83f4cc0b41e7e0b5a5594d16310c6d77df65c90ff5b285f03</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2020</creationdate><topic>Anti-DNA antibodies</topic><topic>Arthritis</topic><topic>C-reactive protein</topic><topic>Complement component C3</topic><topic>Complement component C4</topic><topic>Dapsone</topic><topic>Dermis</topic><topic>Erythrocyte sedimentation rate</topic><topic>Glucocorticoids</topic><topic>Immunofluorescence</topic><topic>Immunoglobulin A</topic><topic>Immunoglobulin G</topic><topic>Immunoglobulin M</topic><topic>Immunology</topic><topic>Leukocytes (neutrophilic)</topic><topic>Lupus</topic><topic>Patients</topic><topic>Pediatrics</topic><topic>Pigmentation</topic><topic>Proteinuria</topic><topic>Systemic lupus erythematosus</topic><topic>Thrombocytopenia</topic><topic>Urinalysis</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Torres Saavedra, Fabio Andrés</creatorcontrib><creatorcontrib>Campo, Lyna Ramirez</creatorcontrib><creatorcontrib>Mendez, Monica Velasquez</creatorcontrib><creatorcontrib>Barreneche, Natalia Mejia</creatorcontrib><creatorcontrib>Suaza, Gloria Andrea Vargas</creatorcontrib><creatorcontrib>Restrepo, Juan David Ruiz</creatorcontrib><creatorcontrib>Martinez-Gomez, Manuel</creatorcontrib><collection>CrossRef</collection><collection>Immunology Abstracts</collection><collection>AIDS and Cancer Research Abstracts</collection><collection>ProQuest Health & Medical Complete (Alumni)</collection><collection>MEDLINE - Academic</collection><jtitle>Lupus</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Torres Saavedra, Fabio Andrés</au><au>Campo, Lyna Ramirez</au><au>Mendez, Monica Velasquez</au><au>Barreneche, Natalia Mejia</au><au>Suaza, Gloria Andrea Vargas</au><au>Restrepo, Juan David Ruiz</au><au>Martinez-Gomez, Manuel</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Bullous lupus as the first manifestation of systemic lupus erythematosus in the pediatric population: A diagnostic challenge in daily practice</atitle><jtitle>Lupus</jtitle><date>2020-12</date><risdate>2020</risdate><volume>29</volume><issue>14</issue><spage>1937</spage><epage>1942</epage><pages>1937-1942</pages><issn>0961-2033</issn><eissn>1477-0962</eissn><notes>ObjectType-Case Study-2</notes><notes>SourceType-Scholarly Journals-1</notes><notes>ObjectType-Feature-4</notes><notes>content type line 23</notes><notes>ObjectType-Report-1</notes><notes>ObjectType-Article-3</notes><abstract>Introduction
In the GLADEL cohort, the bullous lupus (BSLE) prevalence was 0.41%. However, literature on pediatric BSLE is scarce. This study described the clinical, histological, and immunological characteristics and the treatment response in a series of children with BSLE as the first clinical manifestation of pediatric SLE.
Methods
The clinical, histological, and immunological characteristics of a series of 5 cases of BSLE between 2010-2019 from two reference centers in Colombia were analyzed.
Results
All cases had bullous lesions that resolved with residual hypopigmentation. One had a focal seizure, and another arthritis with thrombocytopenia. Two had transient proteinuria with normal urinalysis. Anti-nuclear antibody titers ranged from 1:160 to 1:2560, and four were anti-dsDNA (+). Five patients had anti-RNP antibodies, and four anti-Sm antibodies. All had low C3, and 80% low C4 counts; 80% had erythrocyte sedimentation rate (ESR) ≥20 mm/hour and 60% had C-reactive protein (CRP) ≥0.5 mg/dL. All patients responded to glucocorticoids and dapsone. Histology reports and direct immunofluorescence (DIF) test showed subepidermal blisters with neutrophils in the papillary dermis and linear deposits of Igs/complement proteins in 80% of the skin biopsies. IgG/IgM was present in 5 samples. IgA was positive in 60% and C3 in 80%.
Conclusions
In this pediatric series, BSLE tends to have a monophasic behavior associated with neuropsychiatric, skeletal, and hematological involvement in 40% of the patients, and with good prognosis.</abstract><cop>London, England</cop><pub>SAGE Publications</pub><doi>10.1177/0961203320950814</doi><tpages>6</tpages><orcidid>https://orcid.org/0000-0003-0385-8160</orcidid><orcidid>https://orcid.org/0000-0001-6593-4275</orcidid><orcidid>https://orcid.org/0000-0003-3638-3771</orcidid><orcidid>https://orcid.org/0000-0002-2370-2916</orcidid></addata></record> |
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| ispartof | Lupus, 2020-12, Vol.29 (14), p.1937-1942 |
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| source | SAGE |
| subjects | Anti-DNA antibodies Arthritis C-reactive protein Complement component C3 Complement component C4 Dapsone Dermis Erythrocyte sedimentation rate Glucocorticoids Immunofluorescence Immunoglobulin A Immunoglobulin G Immunoglobulin M Immunology Leukocytes (neutrophilic) Lupus Patients Pediatrics Pigmentation Proteinuria Systemic lupus erythematosus Thrombocytopenia Urinalysis |
| title | Bullous lupus as the first manifestation of systemic lupus erythematosus in the pediatric population: A diagnostic challenge in daily practice |
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