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Ulcero-mutilating familial acropathy

Three cases of ulcero-mutilating familial acropathy (also known as Thévenard's disease or heredo-sensitive radicular neuropathy of Denny-Brown) are reported. The familial features of the disease are stressed and a genealogic study of the family is made. The similarity of sensitive-trophic lesions in all cases is noteworthy. The obscure pathogenesis has some features in common with heredodegenerative conditions. According to bibliographic references, syringomielia as a cause of the disease may be excluded by histopathologic data. Differential diagnosis must include leprosy and some other conditions where plantar ulcers are present. The disease is characterized by recurrent episodes of necrosis of the distal extremities. Treatment is only symptomatic.


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