RESUMO
Idiopathic hypereosinophilic syndrome (HES) is a rare disorder, characterized by prominent blood or tissue eosinophilia with ensuing tissue damage, which is diagnosed after exclusion of underlying diseases known to cause HES. Sustained eosinophilia is associated with cardiac disease, including endomyocardial thrombosis and fibrosis, leading to restrictive cardiomyopathy, valvular dysfunction and increased thrombotic tendency. Aneurysmal coronary artery disease (CAD) occurring in the absence of stenotic atherosclerotic plaques is extremely rare. We present a case of idiopathic HES in a 30-year-old woman with asymptomatic multianeurysmal CAD and a sinus of Valsalva aneurysm, and discuss a brief review of the literature and management strategies.
Assuntos
Aneurisma Aórtico/complicações , Aneurisma Coronário/complicações , Exantema/etiologia , Síndrome Hipereosinofílica/complicações , Adulto , Gerenciamento Clínico , Feminino , HumanosAssuntos
Carotenoides/efeitos adversos , Citrullus/efeitos adversos , Citrus paradisi/efeitos adversos , Mãos/patologia , Dermatopatias/patologia , beta-Criptoxantina/sangue , Dietoterapia/métodos , Economia/estatística & dados numéricos , Humanos , Licopeno/sangue , Licopeno/metabolismo , Masculino , Pessoa de Meia-Idade , Dermatopatias/dietoterapia , Pigmentação da Pele/fisiologia , Resultado do TratamentoAssuntos
Antifúngicos/uso terapêutico , Dermatomicoses/tratamento farmacológico , Trichosporon/isolamento & purificação , Tricosporonose/tratamento farmacológico , Voriconazol/uso terapêutico , Adulto , Cistos/microbiologia , Dermatomicoses/diagnóstico , Dermatomicoses/microbiologia , Humanos , Masculino , Tricosporonose/diagnóstico , Tricosporonose/microbiologiaRESUMO
BACKGROUND: Primary cutaneous amyloidosis of the penis is a rare entity. OBJECTIVES: We describe two cases of primary penile cutaneous amyloidosis presenting as localized lesions on the penis and not associated with systemic amyloidosis. METHODS: A comprehensive literature review of all published cases. RESULTS: The rarest form of cutaneous amyloidosis, nodular amyloidosis, is the most frequent presentation of penis-limited cutaneous amyloid with a low incidence of systemic disease progression. CONCLUSIONS: Although primary nodular amyloidosis is a rare entity it seems to be the most frequent presentation of penile-limited cutaneous disease, with a low incidence of systemic involvement.
Assuntos
Amiloidose Familiar/patologia , Doenças do Pênis/patologia , Dermatopatias Genéticas/patologia , Humanos , Masculino , Pessoa de Meia-IdadeRESUMO
The hallmark of pancreatic tumours, the desmoplastic reaction, provides a unique microenvironment that affects pancreatic tumour behaviour, its ability to grow and metastasize as well as resist the effects of chemotherapy. Complex molecular interactions and pathways give rise to the desmoplastic reaction. Breakdown or penetration of the desmoplastic reaction may hold the key to overcoming the limits of delivery of efficacious chemotherapy or the development of new targeted treatments. Herein we discuss such new developments to fight the desmoplastic reaction, including inhibitors of the epidermal growth factor, fibroblast growth factor, the hedgehog pathway, as well as new molecular targets like CD40 agonist and its effects on T cells, extracellular matrix modifying enzymes such as LOXL2 inhibitor and novel tumour penetrating peptides for delivery of drugs.