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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Bibliographic Details
Published in:Lupus 2020-12, Vol.29 (14), p.1937-1942
Main Authors: Torres Saavedra, Fabio Andrés, Campo, Lyna Ramirez, Mendez, Monica Velasquez, Barreneche, Natalia Mejia, Suaza, Gloria Andrea Vargas, Restrepo, Juan David Ruiz, Martinez-Gomez, Manuel
Format: Article
Language:English
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
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Summary: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.
ISSN:0961-2033
1477-0962
DOI:10.1177/0961203320950814