Clinical, Histopathological, and Therapeutic Features in Lupus Erythematosus Tumidus: A Retrospective Study
Lupus erythematosus tumidus (LET) is a rare form of cutaneous lupus erythematosus (CLE) with a controversial connection to systemic lupus erythematosus (SLE). We conducted a retrospective study with 34 LET patients in a Spanish tertiary referral center from 2007 to 2019. Most were women [52.9% (18/3...
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Elsevier
2025-06-01
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| Series: | Actas Dermo-Sifiliográficas |
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| Online Access: | http://www.sciencedirect.com/science/article/pii/S0001731025000729 |
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| author | E. La Rotta-Higuera D. Morgado-Carrasco M. Mansilla-Polo P. Giavedoni A. García-Herrera J.M. Mascaró, Jr |
| author_facet | E. La Rotta-Higuera D. Morgado-Carrasco M. Mansilla-Polo P. Giavedoni A. García-Herrera J.M. Mascaró, Jr |
| author_sort | E. La Rotta-Higuera |
| collection | DOAJ |
| description | Lupus erythematosus tumidus (LET) is a rare form of cutaneous lupus erythematosus (CLE) with a controversial connection to systemic lupus erythematosus (SLE). We conducted a retrospective study with 34 LET patients in a Spanish tertiary referral center from 2007 to 2019. Most were women [52.9% (18/34)], with a median age of 53.5 years. Autoimmune or rheumatologic disorders were reported in 52.9% (18/34) of cases, and other CLE variants in 26.5% (9/34). SLE occurred in 8.82% (3/34), while 64.7% (22/34) had autoantibodies. Immunohistochemical CD123 testing tested positive in 75.9% (22/34), while direct immunofluorescence (DIF) showed positivity in 31.8% (7/22) of cases. Treatment included topical agents (100%), antimalarials (73.5%), oral corticosteroids (23.5%), and immunosuppressants (14.7%). All achieved clinical remission, but a delayed response (>3 months) was linked to SLE (p = 0.002) and anti-DNA antibodies (p = 0.003).LET usually associates with autoimmune disorders and autoantibodies. CD123 and DIF aid diagnosis, and systemic treatment may be needed, especially with SLE and anti-DNA antibodies. Resumen: El lupus eritematoso túmido (LET) es una forma rara de lupus eritematoso cutáneo (LEC) con vínculos inciertos con el lupus eritematoso sistémico (LES). Estudiamos retrospectivamente a 34 pacientes con LET entre 2007 y 2019. La mayoría eran mujeres [52,9% 18/34)] (mediana de edad de 53,5 años). El 52,9% (18/34) presentaba trastornos autoinmunitarios o reumatológicos, y el 26,5% (9/34), otras variantes de LEC. Tres pacientes (8,2%) desarrollaron LES. El 64,7% (22/34) presentaba autoanticuerpos. La inmunohistoquímica de CD123 fue positiva en el 75,9% (22/34), y la inmunofluorescencia directa (IFD) en el 31,8% (7/22). El tratamiento incluyó agentes tópicos (100%), antipalúdicos (73,5%), corticosteroides orales (23,5%) y otros inmunosupresores (14,7%). Todos alcanzaron la remisión clínica. Una respuesta tardía (> 3 meses) se asoció a la presencia de LES (p = 0,002) y de anticuerpos anti-ADN (p = 0,003).El LET suele asociarse con trastornos autoinmunitarios y autoanticuerpos. CD123 e IFD podrían ser útiles para el diagnóstico. Puede requerirse tratamiento sistémico, especialmente en casos de LES y presencia de anticuerpos anti-ADN. |
| format | Article |
| id | doaj-art-46384fd4cdd64a60b6d4ffca93297162 |
| institution | OA Journals |
| issn | 0001-7310 |
| language | English |
| publishDate | 2025-06-01 |
| publisher | Elsevier |
| record_format | Article |
| series | Actas Dermo-Sifiliográficas |
| spelling | doaj-art-46384fd4cdd64a60b6d4ffca932971622025-08-20T02:32:04ZengElsevierActas Dermo-Sifiliográficas0001-73102025-06-01116662863210.1016/j.ad.2024.02.038Clinical, Histopathological, and Therapeutic Features in Lupus Erythematosus Tumidus: A Retrospective StudyE. La Rotta-Higuera0D. Morgado-Carrasco1M. Mansilla-Polo2P. Giavedoni3A. García-Herrera4J.M. Mascaró, Jr5Department of Dermatology, Hospital Clínic de Barcelona, Barcelona, SpainDepartment of Dermatology, Hospital Clínic de Barcelona, Barcelona, SpainDepartment of Dermatology, Hospital Universitario y Politécnico La Fe, Valencia, Spain; Instituto de Investigación Sanitaria (IIS) La Fe, Valencia, SpainDepartment of Dermatology, Hospital Clínic de Barcelona, Barcelona, SpainDepartment of Pathology, Hospital Clínic de Barcelona, Barcelona, SpainDepartment of Dermatology, Hospital Clínic de Barcelona, Barcelona, Spain; Corresponding author.Lupus erythematosus tumidus (LET) is a rare form of cutaneous lupus erythematosus (CLE) with a controversial connection to systemic lupus erythematosus (SLE). We conducted a retrospective study with 34 LET patients in a Spanish tertiary referral center from 2007 to 2019. Most were women [52.9% (18/34)], with a median age of 53.5 years. Autoimmune or rheumatologic disorders were reported in 52.9% (18/34) of cases, and other CLE variants in 26.5% (9/34). SLE occurred in 8.82% (3/34), while 64.7% (22/34) had autoantibodies. Immunohistochemical CD123 testing tested positive in 75.9% (22/34), while direct immunofluorescence (DIF) showed positivity in 31.8% (7/22) of cases. Treatment included topical agents (100%), antimalarials (73.5%), oral corticosteroids (23.5%), and immunosuppressants (14.7%). All achieved clinical remission, but a delayed response (>3 months) was linked to SLE (p = 0.002) and anti-DNA antibodies (p = 0.003).LET usually associates with autoimmune disorders and autoantibodies. CD123 and DIF aid diagnosis, and systemic treatment may be needed, especially with SLE and anti-DNA antibodies. Resumen: El lupus eritematoso túmido (LET) es una forma rara de lupus eritematoso cutáneo (LEC) con vínculos inciertos con el lupus eritematoso sistémico (LES). Estudiamos retrospectivamente a 34 pacientes con LET entre 2007 y 2019. La mayoría eran mujeres [52,9% 18/34)] (mediana de edad de 53,5 años). El 52,9% (18/34) presentaba trastornos autoinmunitarios o reumatológicos, y el 26,5% (9/34), otras variantes de LEC. Tres pacientes (8,2%) desarrollaron LES. El 64,7% (22/34) presentaba autoanticuerpos. La inmunohistoquímica de CD123 fue positiva en el 75,9% (22/34), y la inmunofluorescencia directa (IFD) en el 31,8% (7/22). El tratamiento incluyó agentes tópicos (100%), antipalúdicos (73,5%), corticosteroides orales (23,5%) y otros inmunosupresores (14,7%). Todos alcanzaron la remisión clínica. Una respuesta tardía (> 3 meses) se asoció a la presencia de LES (p = 0,002) y de anticuerpos anti-ADN (p = 0,003).El LET suele asociarse con trastornos autoinmunitarios y autoanticuerpos. CD123 e IFD podrían ser útiles para el diagnóstico. Puede requerirse tratamiento sistémico, especialmente en casos de LES y presencia de anticuerpos anti-ADN.http://www.sciencedirect.com/science/article/pii/S0001731025000729Lupus eritematoso túmidoLupus cutáneo eritematosoLupus eritematoso sistémicoCD123Inmunofluorescencia directaTrastornos autoinmunes |
| spellingShingle | E. La Rotta-Higuera D. Morgado-Carrasco M. Mansilla-Polo P. Giavedoni A. García-Herrera J.M. Mascaró, Jr Clinical, Histopathological, and Therapeutic Features in Lupus Erythematosus Tumidus: A Retrospective Study Actas Dermo-Sifiliográficas Lupus eritematoso túmido Lupus cutáneo eritematoso Lupus eritematoso sistémico CD123 Inmunofluorescencia directa Trastornos autoinmunes |
| title | Clinical, Histopathological, and Therapeutic Features in Lupus Erythematosus Tumidus: A Retrospective Study |
| title_full | Clinical, Histopathological, and Therapeutic Features in Lupus Erythematosus Tumidus: A Retrospective Study |
| title_fullStr | Clinical, Histopathological, and Therapeutic Features in Lupus Erythematosus Tumidus: A Retrospective Study |
| title_full_unstemmed | Clinical, Histopathological, and Therapeutic Features in Lupus Erythematosus Tumidus: A Retrospective Study |
| title_short | Clinical, Histopathological, and Therapeutic Features in Lupus Erythematosus Tumidus: A Retrospective Study |
| title_sort | clinical histopathological and therapeutic features in lupus erythematosus tumidus a retrospective study |
| topic | Lupus eritematoso túmido Lupus cutáneo eritematoso Lupus eritematoso sistémico CD123 Inmunofluorescencia directa Trastornos autoinmunes |
| url | http://www.sciencedirect.com/science/article/pii/S0001731025000729 |
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