[TAFRO syndrome and cutaneous necrotizing vasculitis]. / Syndrome TAFRO et vascularite nécrosante cutanée : une association inédite.
Rev Med Interne
; 42(2): 134-139, 2021 Feb.
Article
em Fr
| MEDLINE
| ID: mdl-33218790
ABSTRACT
INTRODUCTION:
TAFRO syndrome is a systemic inflammatory syndrome in the spectrum of Castleman's disease, associating thrombocytopenia, anasarca, fever, renal failure and/or reticulin myelofibrosis and organomegaly. Its association with necrotizing cutaneous vasculitis has not yet been reported. CASE REPORT A 69-year-old woman presented with weight loss, fever, anasarca, organomegaly, lymphadenopathy, anuria and extensive necrotic livedo occurring after acute diarrhea. Biology showed anemia, thrombocytopenia, renal failure, hypergammaglobulinemia, a circulating B-lymphocyte clone, hypoparathyroidism and autoimmune hypothyroidism. The skin biopsy showed small vessel vasculitis with fibrinoid necrosis. Methylprednisolone infusions associated with tocilizumab were ineffective and the patient became anuric. Rituximab and plasma exchanges associated to corticosteroids allowed remission for 2 months. Combination of rituximab, cyclophosphamide and dexamethasone resulted in a prolonged remission.CONCLUSION:
We report here the first case of severe cutaneous necrotizing vasculitis in a patient suffering from TAFRO syndrome. The possible resistance to tocilizumab should be known.Palavras-chave
Texto completo:
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Base de dados:
MEDLINE
Assunto principal:
Vasculite
/
Hiperplasia do Linfonodo Gigante
Tipo de estudo:
Diagnostic_studies
/
Risk_factors_studies
Limite:
Aged
/
Female
/
Humans
Idioma:
Fr
Ano de publicação:
2021
Tipo de documento:
Article