24- Cutaneous Manifestations of Microvascular occlusion syndromes

Question 1

What is the pathogenesis of heparin necrosis?

Answer 1

• antibody binds to complex of heparin and platelet factor

This activates the platelet and causes platelet aggregation.

Question 2

What are the clinical features of heparin necrosis?

Answer 2

• well demarcated retiform area with purpuric or ecrotic centre.
  Occurs 5-10 days after initiation of heparin but may occur earlier.

Question 3

Which 2 myeloproliferative disorders can cause thrombocytosis?

Answer 3

• essential thrombocythema and polycythemia veria.

Question 4

What is erythromelalgia and what is it associated with?

Answer 4

-Erythromelalgia is a syndrome that can be idiopathic or secondary to myeloproliferative or myelodysplastic condition and poly cythemia vera/essential thrombocythemia.

It is characterised by burning hands and palmar erythema. Triggered by skin contact with a hot surface.

Question 5

What are the clinical features of thrombocytopenic purpura?

Answer 5

• simple petechiae formation
  in haemolytic uremic syndrome:
• fever, petechiae, thrombocytopenia, microangiopathic hemolytic anemia, renal disease and eurologic symptoms.

Question 6

What is the treatment of trhombotic thrombocytopenic purpura?

Answer 6

• plasmapheresis

plasma exchange or administration of fresh frozen plasm.