PERFORATING MILIA-LIKE IDIOPATHIC CALCINO SIS CUTIS OF THE EXTREMITIES IN DOWNS-SYNDROME

Introduction : Several skin diseases can be seen in patients with tris omy 21. We report a case of miliary calcinosis of the extremities. Cas e report : A 15-year old adolescent with Down's syndrome presented sma ll papular miliary lesions which had developed over 18 months and tend ed to discharge a chalk-like substance via the epidermis. Approximatel y 15 lesions were present on the hands and feet. Histologically, there was a well-delimited calcium deposit in the superficial dermis. There was no alteration in phosphorus/calcium metabolism. Brain CT-scan and cardiac echography did not reveal any calcifications. Discussion : Mi liary calcinosis cutis may not be exceptional in Down's syndrome, alth ough only 9 observations have been reported. Preferential localization s include the hands, wrists and feet. Association with syringoma has b een noted but would appear to be fortuitous. Transepidermal eliminatio n of the calcium deposits is frequent. Pathogenic hypotheses include p recipitation of calcium salts in sudation products and/or increased sy nthesis by fibroblasts. The association with trisomy 21 appears to be significant since only three cases have been reported in patients with normal karyotypes. This entity should be individualized as ''perforat ing milia-like idiopathic calcinosis cutis of the extremities''.