Thevenard Disease: Rare Association of 3 Cases in the Same Family (3 Sisters)

Abstract

Thevenard's disease is a rare familial ulceromutilative acropathy, responsible for sensory peripheral neuropathy associated with dysautonomous syndrome. The delay in diagnosis and major comorbidities make this condition disabling. We report here a case of 3 sisters, with a history of parental consanguinity, received in Orthopedic and Traumatology Department for the management of a sensitive polyneuropathy, associated with pressure ulcers and ulcers arthropathies of the feet. Functionally, walking was possible without dodging lameness. The electromyogram was in favor of a sensory polyneuropathy of the four limbs. An artisan shoe footfeeding shoe has been proposed, in the face of delayed healing of the wound, associated with local care and sensorimotor rehabilitation. Thévenard's Disease is a rare nosological entity that requires multidisciplinary management.