RESUMO
Cryoglobulins are immunoglobulins that precipitate in cold conditions. Type I cryoglobulinemic vasculitis is associated with hematological malignancies. We herein report a case of steroid-resistant type 1 cryoglobulinemic vasculitis associated with monoclonal gammopathy of undetermined significance (MGUS) in a 47-year-old woman. By immunofixation of cryoglobulin, we found that the main component of cryoglobulin was the M protein due to MGUS, so treatment of MGUS was needed. Bortezomib+dexamethasone therapy resulted in a rapid decrease in cryoglobulin and improvement in the symptoms of cryoglobulinemic vasculitis. In refractory type I cryoglobulinemic vasculitis, treatment of the underlying gammaglobulinopathy should be considered.
Assuntos
Crioglobulinemia , Gamopatia Monoclonal de Significância Indeterminada , Paraproteinemias , Vasculite , Feminino , Humanos , Pessoa de Meia-Idade , Bortezomib/uso terapêutico , Gamopatia Monoclonal de Significância Indeterminada/complicações , Gamopatia Monoclonal de Significância Indeterminada/tratamento farmacológico , Gamopatia Monoclonal de Significância Indeterminada/diagnóstico , Crioglobulinas , Paraproteinemias/complicações , Crioglobulinemia/complicações , Crioglobulinemia/tratamento farmacológico , Dexametasona/uso terapêutico , Vasculite/complicações , Vasculite/tratamento farmacológicoRESUMO
BACKGROUND: Streptococcal toxic shock syndrome (STSS) is diagnosed based on signs of shock with multiorgan system involvement, a generalized erythematous macular rash, and rapidly progressive and destructive soft tissue infection. CASE REPORT: The patient was a 2-year-old girl with intramuscular venous malformation in the neck in which an infection occurred, developing into STSS. WHY SHOULD AN EMERGENCY PHYSICIAN BE AWARE OF THIS?: Group A streptococcal infections are common in children and usually have a mild clinical presentation, but may be life threatening in severe cases. Patients with venous malformations are known to have slow-flow anomalies with venous pooling, which can result in hypoxia and possible immune cell dysfunction. Thus, clinicians should be aware of STSS when a patient with venous malformation has a rapidly progressive infection.
Assuntos
Choque Séptico , Infecções Estreptocócicas , Feminino , Humanos , Criança , Pré-Escolar , Streptococcus pyogenes , Infecções Estreptocócicas/complicações , Infecções Estreptocócicas/diagnóstico , Dor no PeitoRESUMO
Infantile hemangiomas arising in the palate are rare. The authors describe a case of ulcerated infantile hemangioma of the hard palate with feeding difficulty. To our knowledge, this is the first reported case of immunohistochemically diagnosed palatal infantile hemangioma successfully treated using oral propranolol.
