Dermatological Manifestations in Primary Biliary Cirrhosis Patients: A Case Control Study

“…Dermographism and melanosis were also very common. In as many as 38.7% of patients the presenting symptom was a dermatologic lesion [213] . An increased portal pressure defined as the difference between the wedge and free pressure in the hepatic veins is frequently found in PBC.…”

Section: Clinical Manifestationsmentioning

confidence: 99%

Primary biliary cirrhosis: From bench to bedside

Notas

2015

WJGPT

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Primary biliary cirrhosis (PBC) is a chronic nonsuppurative destructive intrahepatic cholangitis leading to cirrhosis after a protractive non cirrhotic stage. The etiology and pathogenesis are largely unknown and autoimmne mechanisms have been implicated to explain the pathological lesions. Many epitopes and autoantigens have been reported as crucial in the pathophysiology of the disease and T and B cells abnormalities have been described, the exact pathways leading to the destruction of small intrahepatic ductules are mostly speculative. In this review we examined the various epidemiologal and geoepidemiological data as well as the complex pathogenetic aspects of this disease, focusing on recent in vivo and in vitro studies in this field. Initiation and progression of PBC is believed to be a multifactorial process with strong infuences from the patient's genetic background and by various environmental factors. The role of innate and adaptive immunity, including cytokines, chemokines, macrophages and the involvement of apoptosis and reactive oxygen species are outlined in detailed. The current pathogenetic aspects are presented and a novel pathogenetic theory unifying the accumulated clinical information with in vitro and in vivo data is formulated.A review of clinical manifestations and immunological and pathological diagnosis was presented. Treatment modalities, including the multiple mechanisms of action of ursodeoxycholate were finally discussed. Core tip: Primary biliary cirrhosis (PBC) is a chronic nonsuppurative destructive intrahepatic cholangitis leading to cirrhosis of largely unknown pathophysiology. We examined the epidemiological and geoepidemiological data as well as the pathogenetic aspects of PBC. A novel pathogenetic theory unifying the accumulated clinical information with in vitro and in vivo data is formulated.

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“…A xerose foi, em outros estudos, demonstrada como uma das alterações dermatológicas mais comuns em hepatopatas, notada em 72% de pacientes com cirrose hepática 20 e em 70% de pacientes com CBP 26 . O achado de xerose com prurido foi encontrado ainda em um estudo de monitoramento das reações adversas ao tratamento da hepatite C: a queixa de "pele seca", avaliada como sintoma, foi relatada por 83% dos pacientes durante o tratamento com o IFN-α, com aumento progressivo a cada trimestre de tratamento 27 .…”

Section: Materiais E Métodosunclassified

“…Sabe-se, porém, que algumas terapias podem influenciar no aparecimento de infecções bacterianas, virais e fúngicas. Em uma série de 49 casos de CBP, foram evidenciadas infecções fúngicas de pele em 31,5% dos pacientes 26 . O uso de imunossupressores parece aumentar o risco de infecções e sepse 35 .…”

Section: Materiais E Métodosunclassified