GENERALIZED PSEUDOXANTHOMA ELASTICUM WITH DEFICIENCY OF VITAMIN-K-DEPENDENT CLOTTING FACTORS

Introduction. Pseudoxanthoma elasticum is a connective tissue disease currently classed in 4 forms. Two forms are inherited via dominant aut osomal transmission and the other two via recessive autosomal transmis sion. The generalized form of pseudoxanthoma elasticum is the most unc ommon form and corresponds to recessive type II. Clinical manifestatio ns include the typical generalized ''peau d'orange'' skin associated w ith hyperlaxity of the skin. Usually, there is no systemic manifestati on. Case report. We report a case of a patient with generalized pseudo xanthoma elasticum associated with deficiency of vitamin-g dependent f actors II, VII, IX, and X. A search for other causes of vitamin-K depe ndet factor deficiency was negative. Discussion. The association of ge neralized pseudoxanthoma elasticum with deficiency of vitamin-g depend ent clotting factors has been reported previously in very rare cases a nd is probably not fortuitous. It could led to the definition of a sub -group of recessive autosomal pseudoxanthoma elasticum.