Langerhans Cell Histiocytosis

Question 1

What are langerhan cells and were are they predominantly found and what is their job?

Answer 1

Specialized dendritic cells predominate found in the skin and they present antigen to naive T cells.

Question 2

How do we characterize langerhan cell histocyotis and what is the classic/characteristic feature of EM?

Answer 2

Proliferation of langerhan cells

Birbeck (tennis racket) cells

Question 3

What is the immunohistochemistry of these birbeck cells?

Answer 3

CD1a+ and s100+

Question 4

What are the made up rules to remember for the 3 subtypes of langerhan cell histiocytosis?

Answer 4

If the disease name has someone’s name in it it is malignant
If there are two names in the disease name, it usually presents in an infant less than 2
If there are three names in the disease name it usually present sin kids 3 and older
If it is malignant, it usually involves this skin

Question 5

How do we characterize lettered siwe disease? 3 clinical features?

Answer 5

Malignant proliferation of langerhan cells

Skin rash, cystic skeletal defects in an infant less than 2, and can become fatal quick

Question 6

How do we characterize eosinophilic granuloma, 1 clinical feature to remember and what is not involved?

Answer 6

Benign proliferation of langerhan cells
Pathologic fractures in teenagers
No skin

Question 7

How do we characterize hand Schuller Christian disease? 4 clinical signs?

Answer 7

Malignant proliferation of langerhan cells.

Scal rash, lytic skull defects, diabetes insipidus, and exophthalmos in a child.