Editor,—Homozygous protein C deficiency is rare with an estimated incidence of one in 500 000 to one in 750 000.1 It presents shortly after birth with life threatening thromboses involving the central nervous system, eyes, kidneys, and skin (purpura fulminans).2 Protein C activity, in affected individuals, is usually less than 1% (normal 70–140%).3 Management, in the acute phase, is with intravenous protein C concentrate (ImmunoAG, Vienna, Austria).4 Untreated cases usually result in death.

We present a family exhibiting a spectrum of features caused by protein C deficiency.