Background. Fahr syndrome is defined by the presence at the brain base of strio-pallido-notched non-arteriosclerotic bilateral and symmetric calcifications. We report an observation of Fahr syndrome revealed by a grow...Background. Fahr syndrome is defined by the presence at the brain base of strio-pallido-notched non-arteriosclerotic bilateral and symmetric calcifications. We report an observation of Fahr syndrome revealed by a growing pyodermitis in direct relation with hypocalcemia. Observation. A 17 year-old girl was admitted to the Dermatology Department for a growing pyodermitis of the large folds. The patient was known to be epileptic and had undergone surgery for a left eye cataract a year before. Microbiologic and mycologic examinations of the pus and of the cutaneous fragments were negative. During hospitalization,thepatienthadanattackoftetany,alaryngalspasm and a generalized erythema thrust studded with pustules. The phosphocalcic assessment showed severe hypocalcemia and hyperphosphoremia. Parathormone srum rate was decreased. The brain tomodensitometry conveyed bilateral calcifications of the central gray nucleus. The diagnosis of a growing amicrobial pustulosis secondary to an idiopathic hypoparathyroidism and responsible for the Fahr syndrome was maintained. Treatment was composed of calcium (2 g/day) and vitamin D3. The evolution was favorable with a relapse at 2 years. Discussion. We report an idiopathic hypoparathyroidism associated with Fahr syndrome conveyed by growing amicrobial pustulosis. We discuss the dermatologic manifestations of Fahr syndrome associated with hypoparathyroidism and the physiopathologic mechanisms of the dermatologic lesions during hypocalcemia.展开更多
文摘Background. Fahr syndrome is defined by the presence at the brain base of strio-pallido-notched non-arteriosclerotic bilateral and symmetric calcifications. We report an observation of Fahr syndrome revealed by a growing pyodermitis in direct relation with hypocalcemia. Observation. A 17 year-old girl was admitted to the Dermatology Department for a growing pyodermitis of the large folds. The patient was known to be epileptic and had undergone surgery for a left eye cataract a year before. Microbiologic and mycologic examinations of the pus and of the cutaneous fragments were negative. During hospitalization,thepatienthadanattackoftetany,alaryngalspasm and a generalized erythema thrust studded with pustules. The phosphocalcic assessment showed severe hypocalcemia and hyperphosphoremia. Parathormone srum rate was decreased. The brain tomodensitometry conveyed bilateral calcifications of the central gray nucleus. The diagnosis of a growing amicrobial pustulosis secondary to an idiopathic hypoparathyroidism and responsible for the Fahr syndrome was maintained. Treatment was composed of calcium (2 g/day) and vitamin D3. The evolution was favorable with a relapse at 2 years. Discussion. We report an idiopathic hypoparathyroidism associated with Fahr syndrome conveyed by growing amicrobial pustulosis. We discuss the dermatologic manifestations of Fahr syndrome associated with hypoparathyroidism and the physiopathologic mechanisms of the dermatologic lesions during hypocalcemia.
