Caffey-Silverman syndrome

Description: Also known as: Caffey's syndrome I Caffey-Smyth syndrome Smyth-Caffey syndrome De Toni-Caffey syndrome De Toni-Caffey-Silverman syndrome De Toni-Silverman-Caffey syndrome Roske-De Toni-Caffey syndrome Roske-De Toni-Caffey-Silverman syndrome Roske-De Toni-Caffey-Smyth syndrome Description: Probably familial disease of infants affecting skeleton and adjacent tissues. It is characterized by fever, irritability, swelling of soft tissues, and cortical bone thickening. The mandible is usually involved, less commonly the clavicle, tibia, ulna, femur ribs, humerus, and fibula with periodic proliferation in the first 3 months of life. Jaw and forearm are the most common sites of thickening. Tenderness and movement limitation of affected parts. Puffy jaws and cheeks give the face a characteristic appearance. Also ocular signs. Although cases have been identified in utero, the syndrome may usually be recognized during the first six months of life. Spontaneous healing within few weeks. Etiology unknown. Inheritance is autosomal domninant.