À propos d’un cas rare d’acro-ostéolyse idiopathique

Abstract

Acro-osteolysis is characterized by bone resorption in hands and feet. It may be caused by several diseases. Joseph and Shinz or phalangeal acro-osteolysis is a rare idiopathic form, we report one probable case. Case report. – F.J., 13 years old has presented two years ago a swelling and ulceration of the second and the third left fingers and the second right finger. Physical exam showed short fingers and hypertrophic nails without any other associated abnormalities. X-rays showed terminal phalanges resorption in hands and feet. Inflammatory and immunological laboratory investigations were normal. Lesions biopsy showed skin fibrosis without inflammatory signs. Neurological exam and the electromyographic study were normal. Syphilis serology was negative and the phosphorus/calcium balance studies were normal. The probable diagnosis was Joseph and Shinz or phalangeal idiopathic acro-osteolysis. Discussion. – Joseph and Shinz or phalangeal acro-osteolysis is an hereditery form of acro-osteolysis with autosomal dominant or recessive transmission. It affects distal extremities of last phalanges of hands and feet, but may progress to other bones.