EHLERS-DANLOS-SYNDROMES - REVISED NOSOLOGY, VILLEFRANCHE, 1997

Categorization of the Ehlers-Danlos syndromes began in the late 1960s and was formalized in the Berlin nosology, Over time, it became appare nt that the diagnostic criteria established and published in 1988 did not discriminate adequately between the different types of Ehlers-Danl os syndromes or between Ehlers-Danlos syndromes and other phenotypical ly related conditions, In addition, elucidation of the molecular basis of several Ehlers-Danlos syndromes has added a new dimension to the c haracterization of this group of disorders, We propose a revision of t he classification of the Ehlers-Danlos syndromes based primarily on th e cause of each type, Major and minor diagnostic criteria have been de fined for each type and complemented whenever possible with laboratory findings, This simplified classification will facilitate an accurate diagnosis of the Ehlers-Danlos syndromes and contribute to the delinea tion of phenotypically related disorders. (C) 1998 Wiley-Liss, Inc.