Purpura fulminans. A cutaneous manifestation of severe protein C deficiency
M. J. Auletta and J. T. Headington
Department of Dermatology, University of Michigan Medical School, Ann Arbor 48109-0602.
Protein C, when activated, is a vitamin K-dependent serine protease that
has anticoagulant and profibrinolytic activities. An increased risk of
thrombotic disease is associated with deficiency of this enzyme. A neonate
developing purpura fulminans with internal thrombotic complications and
congenital absence of protein C is described. Family studies showed partial
protein C deficiency in the subject's symptom-free parents and two of three
siblings. Clinically and histopathologically similar lesions are also seen
in two acquired conditions in which there is a thrombotic tendency:
coumarin necrosis and purpura fulminans of childhood, both of which are
probably the result of transient protein C deficiency. We conclude that
purpura fulminans can represent a cutaneous marker of acquired or
congenital protein C deficiency.
