[Association of hepatic adenomatosis and hepatoportal sclerosis in a woman with incontinentia pigmenti]. / Association d'une adénomatose hépatique et d'une sclérose hépatoportale chez une femme atteinte d'incontinentia pigmenti.
Gastroenterol Clin Biol
; 21(2): 147-51, 1997.
Article
en Fr
| MEDLINE
| ID: mdl-9161481
ABSTRACT
We report the case of the association of three uncommon diseases in a young woman incontinentia pigmenti, portal hypertension due to hepatoportal sclerosis, and liver adenomatosis. Incontinentia pigmenti is a hereditary genodermatosis with pigmentary cutaneous lesions and dysmorphic malformations. In our patient, among liver abnormalities, there were blood biochemical alterations, portal hypertension, and initially neo hepatic nodular lesions. Histological examination of the surgical liver specimen showed several adenomas and fibrosis of the portal tracts with portal vascular changes. The etiopathogenic nature of the adenomatosis and hepatoportal sclerosis is unclear. As a general rule, hepatic adenomatosis is associated with normal liver. We hypothesize that the adenomas could be secondary to changes in hepatic vascularisation.
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Bases de datos:
MEDLINE
Asunto principal:
Sistema Porta
/
Incontinencia Pigmentaria
/
Adenoma de Células Hepáticas
/
Neoplasias Hepáticas
Tipo de estudio:
Etiology_studies
/
Risk_factors_studies
Límite:
Adolescent
/
Female
/
Humans
Idioma:
Fr
Revista:
Gastroenterol Clin Biol
Año:
1997
Tipo del documento:
Article