A peculiar involvement of the interphalangeal joints of both hands with palmar flexion of the fingers has been observed in
11 insulin-treated, nonrheumatoid, juvenile diabetics. The onset of diabetes occurred between 1 and 12 years of age. Painless
deformities of the fingers with progressive stiffness and impaired extension started 4 to 10 years later. One patient complained
of articular pain and swelling. X-ray and circulatory changes were absent or minimal. Prepubertal patients showed delayed
puberty and stunted growth, adult patients had normal sexual development. Rheumatic or rheumatoid signs were absent. Electromyography
showed minor abnormalities of the motor units, normal or subnormal motor nerve conduction velocity, increased median nerve
terminal latency, in the absence of muscular atrophy or thickening of palmar tendons. Vibratory sensitivity was impaired in
1 subject. Juvenile cheiroarthropathy is associated with:
a) early onset and poor control of diabetes;
b) stunted growth;
c) hepatomegaly;
d) delayed puberty;
e) long standing administration of insulin. The articular changes are distinct from previously known forms of ‘diabetic hand’,
such as atrophic neuropathy, osteoarthropathy, Dupuytren’s contracture, carpal tunnel syndrome.
Key-words Diabetic cheiroarthropathy - Diabetic hand - Hand stiffness - Mauriac’s syndrome - Neurodystrophic fingerjoints - Prepubertal complications