Paraneoplastic pemphigus and Castleman's disease: a case report and a revision of the literature.
Ital J Pediatr
; 49(1): 33, 2023 Mar 20.
Article
em En
| MEDLINE
| ID: mdl-36941723
ABSTRACT
BACKGROUND:
In literature, a few reports described an association between paraneoplastic pemphigus (PNP) and Castelman's disease (CD), but no consensus have been proposed for the diagnostic-therapeutical approach. Aim of this study is to present a case report and explore the relationship between PNP and CD in pediatric patients, focusing on clinical manifestations, histopathological findings, treatment and outcome to find elements for an early diagnosis. CASE PRESENTATION We present the clinical case of a 13 years old girl with a challenging diagnosis of PNP and CD who underwent therapy at first with Rituximab and then with Siltuximab, for the control of symptoms.CONCLUSIONS:
Reviewing literature, 20 clinical cases have been described in the pediatric age. Diagnosis may be challenging, requiring an average of 3 months (range from 3 weeks to 2 years). In all cases, the initial manifestations were mucocutaneous lesions, especially oral lesions with poor response to conventional treatment. Systemic symptoms may be present as well. Therapeutical approach is still discussed with no consensus. Almost all patients received corticosteroids with poor response. Other drugs including azathioprine, methotrexate, cyclosporine and monoclonal antibodies have been evaluated for the control of the disease. Further studies and experimental trials urge to define the diagnostic criteria and therapy protocol.Palavras-chave
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Hiperplasia do Linfonodo Gigante
/
Pênfigo
Tipo de estudo:
Diagnostic_studies
/
Etiology_studies
/
Guideline
/
Screening_studies
Limite:
Adolescent
/
Child
/
Female
/
Humans
Idioma:
En
Revista:
Ital J Pediatr
Assunto da revista:
PEDIATRIA
Ano de publicação:
2023
Tipo de documento:
Article
País de afiliação:
Itália