Familial cheiroarthropathy without juvenile onset diabetes mellitus

Cheiroarthropathy is a recently recognised complication of juvenile onset diabetes mellitus. It comprises inability to extend fully the fingers, contracted tendons, and waxy thickening of the skin overlying the fingers and to a lesser extent the hands. We report two families in which one parent and a number of siblings had the typical features of cheiroarthropathy without juvenile onset diabetes mellitus. The changes developed gradually during childhood and did not progress after adolescence. There were no other abnormal clinical findings, no persistently abnormal laboratory tests, and no association with a specific HLA phenotype. There are some similarities with scleroderma and its recognition is important to prevent unnecessary treatment and to reassure patients.