Clinical Features of Paediatric Inflammatory Epidermolysis Bullosa Acquisita: A Case Series Study.
Acta Derm Venereol
; 104: adv11917, 2024 Jan 25.
Article
en En
| MEDLINE
| ID: mdl-38270257
ABSTRACT
Epidermolysis bullosa acquisita (EBA) rarely develops in childhood. This study retrospectively recruited paediatric patients with EBA (age ≤ 16 years), diagnosed by clinical and histopathological features and results of immunofluorescence, immunoblotting and enzyme-linked immunosorbent assay (ELISA), and reviews their clinical manifestations, histopathology, immunological features, and responses to various treatments. All 7 included patients presented with inflammatory EBA. Among them, 3 had a bullous pemphigoid-like phenotype. Pathologically, in addition to dermal-epidermal blistering, in all patients, the distribution of neutrophils was superficial perivascular or interstitial, or in the dermal papilla. Mixed neutrophils and eosinophils were detected in 2 of the 3 patients with bullous pemphigoid-like phenotypes. In addition to treatment with glucocorticoids, dapsone was administered in 4 patients, while thalidomide and sulfasalazine were administered in 1 patient. All patients responded to the these therapies. Relapse was mainly due to reduction and cessation of glucocorticoids. In conclusion, EBA in childhood may be unique, and thus distinct from its adult counterpart. Specific treatment and follow-up protocols are required for therapy of this rare autoimmune skin disease in children.
Texto completo:
1
Colección:
01-internacional
Banco de datos:
MEDLINE
Asunto principal:
Enfermedades Autoinmunes
/
Epidermólisis Ampollosa Adquirida
/
Penfigoide Ampolloso
Tipo de estudio:
Diagnostic_studies
/
Guideline
/
Observational_studies
Límite:
Adolescent
/
Adult
/
Child
/
Humans
Idioma:
En
Revista:
Acta Derm Venereol
Año:
2024
Tipo del documento:
Article
País de afiliación:
China