Mastocytosis

Question 1

What is urticaria pigmentosa?

Answer 1

Darker urticaria associated with mastocytosis.

Question 2

What is mastocytosis?

Answer 2

A rare disorder characterized by abnormal mast cell proliferation.

Question 3

What are the three broad categories of mastocytosis?

Answer 3

Cutaneous (urticaria pigmentosa)
Systemic
Malignant

Question 4

What lab test can be used to screen for cutaneous mastocytosis?

Answer 4

Tryptase

Question 5

What is Darier’s sign?

Answer 5

Wheal developed after gently stroking urticaria pigmentosa (cutaneous mastocytosis).

Question 6

Patients with mastocytosis should avoid triggers that cause mast cell degranulation. What are these typical triggers?

Answer 6

Cold
Heat
Alcohol
Aspirin
Opiates

Question 7

What medication can be given for mastocytosis?

Answer 7

Cromolyn which can help stabilize mast cells.

Question 8

A 7 year old is seen for large, hyperpigmented raised areas all over his back. Gently stroking an area causes a wheal. What serum test could help confirm the diagnosis?

Answer 8

Tryptase level > 20. This could be cutaneous mastocytosis (urticaria pigmentosa)