Skin Changes in Congenital Hemostasis Disorders

Abstract

The article examines skin changes and patterns of hemorrhagic manifestations in congenital disorders of hemostasis. It describes the hematoma type of bleeding in hemophilia A and B, characterized by deep, painful hemorrhages into the subcutaneous tissue, and the petechial-spotted type in thrombocytopathies, with petechiae and ecchymoses. Disturbances of pigmentation are noted in Hermansky-Pudlak and Chediak-Higashi syndromes, while a mixed hemorrhagic pattern occurs in several hereditary coagulopathies. The severity of skin manifestations depends on the degree of coagulation-factor deficiency.