Vascular Disorders Flashcards
Hereditary vascular disorders
- Hereditary hemorrhagic Telangiectasia (Rendy-Osler-Weber Syndrome)
- Hemangioma-Thrombocytopenia Syndrome (Kasabach-Meritt Syndrome)
- Ehlers-Danlos Syndrome and Other Genetic Disorders
Acquired Vascular Disorders
- Allergic Purpura (Henoch-Schönlein Purpura)
- Paraproteinemia and Amyloidosis
- Senile Purpura
- Drug – Induced Vascular Purpuras
- Scurvy
Characterized by thin-walled blood vessels with discontinuous endothelium, inadequate smooth muscle, and inadequate / missing elastin in the surrounding stoma
Hereditary hemorrhagic Telangiectasia (Rendy-Osler-Weber Syndrome)
Universal finding of Hereditary hemorrhagic Telangiectasia (Rendy-Osler-
Weber Syndrome)
epistaxis (nosebleed)
dilated superficial blood vessels that create small, focal red lesions
Telangiectasia
Vascular tumor (Giant cavernous hemangioma), thrombocytopenia, bleeding diathesis
Hemangioma-Thrombocytopenia Syndrome (Kasabach-Meritt Syndrome)
Sequestration of platelets in the hemangiomas
Hemangioma-Thrombocytopenia Syndrome
(Kasabach-Meritt Syndrome)
May be transmitted as an autosomal dominant, recessive, or X-linked trait.
Ehlers-Danlos Syndrome
It is manifested by Hyperextensible skin, Hypermobile joints, Joint laxity
Ehlers-Danlos Syndrome
Be ascribed to defects in collagen production, structure, or cross-linking, with resulting inadequacy of the connective tissues
Ehlers-Danlos Syndrome
Generally applied to a group of nonthrombocytopenic purpura characterized by allergic manifestations including skin rash and edema
Allergic Purpura (Henoch-Schönlein Purpura)
More appropriately applied when the conditions an acute IgA-mediated disorder
Allergic Purpura (Henoch-Schönlein Purpura)
Implicates autoimmune microvascular injury
Allergic Purpura (Henoch-Schönlein Purpura)
Platelet function can be inhibiting by myeloma proteins
Paraproteinemia
IgA myeloma & IgG myeloma and Recurrent Purpurua
Dysproteinemia
Fibrous protein consisting of rigid, linear, non-branching, aggregated fibrils
Amyloid
The deposition of abnormal quantities of amyloid protein in tissues, may be primary or secondary, and localized or systemic
Amyloidosis
Common in elderly men due to lack of collagen support for small blood vessels.
Senile Purpura
Dark blotches are flattened. Do not blanch with pressure. Often leaving a brown stain in the skin (age spots)
Senile Purpura
Deficiency of Vitamin C
Scurvy
Cause defect the synthesis of collagen in the walls of small blood vessels
Scurvy
“Corkscrew” hairs.
Scurvy
