Ulcerative-mutilating acropathy occurs as an inherited (Thevenard's disease
) and a sporadic (Bureau-Barriere syndrome) variant. We report a retrospect
ive study in nine patients with the sporadic variant. All nine had painless
foot ulcers with trophic disorders and severe skeletal alterations. Electr
odiagnostic testing showed polyneuropathy with sensory disorders in seven p
atients. Motor conduction velocity was normal. Radiological changes were co
nfined to the lower limbs. The clinical course and treatment of sporadic ul
cerative-mutilating acropathy are presented.