RESUMO
A healthy 12-month-old female presented with relapsing and remitting urticaria since birth that was resistant to treatment with antihistamines. A thorough history revealed extensive rheumatic disease on the father's side of the family, and subsequent genetic testing was positive for a missense variant of NLRP3, indicating cryopyrin-associated periodic fever syndrome (CAPS). CAPS encompasses a spectrum of diseases, all related to a defect in the same gene; manifestations vary in severity and presentation, but most are associated with recurrent rash and fever. Because the patient's only presenting symptom was rash, this case highlights the importance of having a high index of suspicion for cryopyrin-associated periodic fever syndrome in infants with persistent, early urticaria.
Assuntos
Síndromes Periódicas Associadas à Criopirina , Exantema , Urticária , Lactente , Humanos , Feminino , Proteína 3 que Contém Domínio de Pirina da Família NLR/genética , Mutação , Síndromes Periódicas Associadas à Criopirina/complicações , Síndromes Periódicas Associadas à Criopirina/diagnóstico , Síndromes Periódicas Associadas à Criopirina/tratamento farmacológico , Febre , Urticária/diagnóstico , Urticária/tratamento farmacológico , Urticária/etiologiaRESUMO
The sunscreens we see today were paved by our ancestors' sun protection methods, followed by scientific discovery in the more recent era and the trials and errors of the sunscreen formulations that followed. This history highlights sun protection methods used over the centuries and the varying degrees of agreeable sensorial properties or comforts in these methods.
RESUMO
ABSTRACT: Cutaneous/systemic plasmacytosis (C/SP) is a plasma cell disorder characterized by reddish-brown patches, lymphadenopathy, and hypergammaglobulinemia. The degree to which C/SP overlaps with other plasma cell proliferative disorders and neoplasms is incompletely understood. We present the case of a patient with a several-year history of cutaneous plasmacytosis and evidence of systemic involvement with concurrent idiopathic multicentric Castleman disease (iMCD) involving a lymph node. There have been only a few reports of systemic iMCD preceded by a long, asymptomatic phase of cutaneous manifestations. We discuss the relationship between C/SP and iMCD and elaborate on the pathophysiological overlap of these 2 conditions and potential similarities in their pathogenesis. We suggest that the 2 diseases may represent the same entity presenting on a spectrum, with individuals diagnosed with C/SP at risk for progression to iMCD.