Ehlers-Danlos is a hereditary disease of the whole connective tissue
initially described by dermatologists (Tscherchnogobov
Moscow 1892, Ehlers, Copenhagen, 1900, Danlos, Paris, 1908). They
emphasized the joint hyperlaxity and stretchiness of the skin
which has long summed up the clinical expression of this entity. In
recent decades, many other manifestations have been described
and gradually identified, mainly by rheumatologists (Grahame, London,
1960). Several of them concern the digestive tract, mainly
gastric reflux and constipation. They can be the cause of serious
accidents: bronchial flooding by gastric reflux or aspiration,
intestinal
obstruction, hernial constriction, eventration, intestinal rupture,
peritonitis of vesicular or appendicular origin, hemorrhages. It is
important that gastroenterologists know how to link these manifestations
to their etiology in order to adapt treatments, prevent
iatrogenic accidents and direct the patients towards the treatment of
other manifestations of Ehlers-Danlos disease. Nine clinical
signs, including digestive manifestations, allow diagnosis by their
significant grouping. The proof of hereditary origin is based
on the identification of other identical cases in the family,, even if
they are paucisymptomatic. A person affected by the disease
systematically transmits the disease to all his children. We have
verified this in all our patients.
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