[Ultrastructural study of hereditary benign telangiectasia. Differential diagnosis from Osler Rendu disease].
BACKGROUND
There are two hereditary forms of primary telangiectasia with a totally opposite prognosis. In Rendu-Osler disease, also called hereditary haemorrhagic telangiectasia, there is a major risk of severe haemorrhage in adults. In benign hereditary telangiectasia there is no such risk.


METHODS
We report two brothers who were diagnosed as having Rendu-Osler disease. Under standard electron microscopy, a biopsy of a telangiectasia taken from the right arm pit showed that the vessels of the superficial reticular derma were dilated with thick walls but no dehiscences as in Rendu-Osler disease.


CONCLUSIONS
This ultrastructure explains why there had been no haemorrhages in our two cases and favoured a diagnosis of hereditary benign telangiectasia.
Tsianakas
P
P
Département d'Histologie, Faculté de Médecine Lariboisière-Saint-Louis, Paris.
Teillac-Hamel
D
D
Fraitag
S
S
De Prost
Y
Y
Prost
C
C
D002648:Child; D002675:Child, Preschool; D003937:Diagnosis, Differential; D006801:Humans; D008297:Male; D008854:Microscopy, Electron; D011379:Prognosis; D012867:Skin / Q000648:ultrastructure*; D013683:Telangiectasia, Hereditary Hemorrhagic / Q000175:diagnosis*; D013684:Telangiectasis / Q000175:diagnosis / Q000235:genetics* / Q000473:pathology
Etude ultrastructurale des télangiectasies héréditaires bénignes. Un diagnostic différentiel de la maladie de Rendu-Osler.