Title: GZ / The Grenz Zone Season 2 / 30 Vasculopathies
Tags: GZ DermnetNZ

Q: Vasculopathies refer to conditions with blood vessel damage in the [presence or absence] of vasculitis
A: absence
Back Extra: Often due to problems with clotting, typically an inherited or acquired issue with platelets or the coagulation pathway

Some causes of vasculopathy include inherited coagulopathies, acquired coagulopathies, increased platelet destruction, abnormal platelet function, embolic disorders, and other miscellaneous diseases
Back Extra: Inherited coagulopathies: factor V Leiden, protein C/S deficiency, antithrombin III mutation, hyperhomocysteinemia, sick cell disease
Acquired coagulopathies: anti-phospholipid syndrome, liver disease, type 1 cryoglobulinemia, purpura fulminans
Increased platelet destruction: idiopathic thrombocytopenic purpura, thrombotic thrombocytopenic purpura, DIC, HIT
Abnormal platelet function: medications, myeloproliferative disorders, renal disease
Embolic disorders: cholesterol emboli, oxalate emboli
Miscellaneous (Pigmented Purpuras): Schambergs disease, Lichenoid purpura of Gougerot and Blum, purpura annularis telangiectodes, Lichen Aureus, Eczematous Dermatitis of Doucas and Kapetanakis

Q: Factor V Leiden is an inherited coagulopathy where factor V is resistant to degradation by [specific protein] which leads to activation of the coagulation cascade
A: protein C

Q: Protein C/S deficiency is an inherited coagulopathy where you are unable to inactivate factors [2 answers] leading to activation of the coagulation cascade
A: V & VIII
Back Extra: Protein C/S are brakes that stop coagulation. Think of it as "broken brakes"

Q: Antithrombin III mutation is an inherited coagulopathy where you are unable to block factors [2 answers]
A: II & X
Back Extra: Normally, antithrombin III blocks factors II and X, leading to inhibition of the coagulation cascade. Heparin activates antithrombin III

Q: What are the 3 anti-phospholipid antibodies?
[...]
A: anti-cardiolipin, lupus anticoagulant, and anti-beta2 glycoprotein
Back Extra: Patients with anti-phospholipid syndrome are more prone to forming clots, therefore look for a hx of stroke, MI, DVT, PE, miscarriages. Cutaneous changes are related to occlusion, including livedo reticularis, splinter hemorrhages, or retiform purpura.

Q: Type 1 cryoglobulinemias is an acquired coagulopathy that is a lymphoproliferative disorder making monoclonal [specific Ig] leading to a vasculopathy
A: IgM
Back Extra: Patients will present with livedo reticularis, purpura, and classic cold-induced lesions on the ears.
REMEMBER: Type 2 and 3 cryoglobulinemias are vasculitis diseases, whereas type 1 is a vasculopathy

Some risk factors for acquired coagulation include immobilization, obesity, cancer, pregnancy, smoking, oral contraceptives

Q: ITP is an autoimmune disease causing destruction of platelets due to [...] autoantibodies coating platelets and leading to macrophage destruction in the spleen
A: IgG
Back Extra: ITP = idiopathic thrombocytopenic purpura

Q: TTP is caused by a deficiency in [specific enzyme] that cleaves von Willebrand factor multimers leading to platelet aggregation and thrombosis
A: ADAMS TS13
Back Extra: TTP = Thrombotic thrombocytopenic purpura
FAT RN: Fever, MAHA (Microangiopathic Hemolytic Anemia), Thrombocytopenia, Renal disease, Neurologic disease

Q: Schambergs disease affects the lower legs of middle aged adults and appears as petechiae with [color] hemosiderin staining
A: golden-brown

Q: Lichenoid purpura of Gougerot and Blum presents as [color] or [color] lichenoid papules on the legs and trunk of older men
A: rust-colored; violaceous

Q: Purpura annularis telangiectodes presents as 1-3 cm [...] with petechiae on the legs of younger women
A: annular patches

Q: Lichen Aureus presents as solid [color] macules or papules usually overlying a varicose vein
A: golden to rust colored

Q: Eczematous Dermatitis of Doucas and Kapetanakis presents with similar purpura as Schambergs disease, however they complain of [...]
A: itching

Q: Diagnosis of vasculopathies will demonstrate fibrin thrombi with [...] inflammation and NO leukocytoclastic vasculitis
A: minimal
Back Extra: Screening labs include: PT, PTT, INR, Protein C/S, Factor V Leiden, Antithrombin III, Prothrombin 22-10 gene mutations, anti-prothrombin antibodies, homocysteine levels

Q: Treatment of vasculopathies consists of treating the [...], often in conjunction with a hematologist
A: underlying disease
Back Extra: For pigmented purpuras, treatment includes topical steroids + vitamin C 500mg BID + rutoside 50mg BID
Pigmented purpuras include: Schambergs disease, Lichenoid purpura of Gougerot and Blum, purpura annularis telangiectodes, Lichen Aureus, Eczematous Dermatitis of Doucas and Kapetanakis

Q: Abnormal platelet function can be caused by medications like [2 answers], myeloproliferative disorders, and renal disease, which can lead to a vasculopathy
A: aspirin/NSAIDs
Back Extra: Myeloproliferative disorder example: Multiple Myeloma
Renal disease causes a decrease in erythropoetin production leading to bruising, petechiae, and GI bleeding. Use of dialysis improves platelet function

Q: What is a common lab abnormality associated with cholesterol emboli?
[...]
A: Eosinophilia