Malformation syndrome with connective tissue involvement Desbuquois syndrome orphanet OMIM:300881 ICD10:Q78.8 UMLS:C0432242 Desbuquois syndrome (DBQD) is an osteochondrodysplasia characterized by severe micromelic dwarfism, facial dysmorphism, joint laxity, multiple dislocations, vertebral and metaphyseal abnormalities and advanced carpotarsal ossification. Two forms have been distinguished on the basis of the presence (type 1) or the absence (type 2) of characteristic hand anomalies. Desbuquois dysplasia OMIM:251450 OMIM:615777 MESH:C535943 Primary bone dysplasia