Histiocytoses

Question 1

Langerhans cells are antigen presenting cells in the epidermis, bronchi and mucosa

Answer 1

True

Question 2

Skin biopsy of LCH demonstrates an inflammatory infiltrate of eosinphils, histiocytes and lymphocytes

Answer 2

True

Question 3

Stains of LCH demonstrate positivity to CD1a,CD117

Answer 3

False = not CD117, which is for mast cells, but CD207 +

Question 4

Hand-Schuller-Christian is self healing

Answer 4

False. This is Hashimoto-Pritzker .

- Hand Schuller Christian has exophthalmos, diabetes insipidus and lytic bone lesions and is low-risk multisystem LCH

Question 5

Benign cephalic histiocytosis occurs in infants >1 year of age most commonly

Answer 5

False - <1year of age

Question 6

Benign cephalic histiocytosis mostly occurs by age 1 and always within the first 3 years

Answer 6

T

Question 7

What are the 3 histologic patterns for benign cephalic histiocytosis

Answer 7

PDL

• papillary dermal
• diffuse
• lichenoid

Question 8

Generalised eruptive histiocytoma affects adults more than children

Answer 8

True

Question 9

Treatment is generally required for generalised eruptive histiocytoma

Answer 9

False - generally NOT required

Question 10

Indeterminate cell histiocytosis is positive for s100 and CD1a which are LCH markers, and non LCH markers CD68+ and CD163+

Answer 10

True

Question 11

Indeterminate cell histiocytosis has two variants : solitary and generalised

Answer 11

True

Question 12

Indeterminate cell histiocytosis is Langerin positive (CD207)

Answer 12

False - Langerin negative

Question 13

Touton giant cells present in indeterminate cell histiocytosis

Answer 13

True

Question 14

Juvenile xanthogranuloma is the most common histiocytosis

Answer 14

True

Question 15

JXG associated with hyperlipidaemia

Answer 15

False - reason for progressive lipidation of histiocytes in absence of hyperlipidaemia is unclear

Question 16

JXG Fatalities have been reported with liver involvement and CNS involvement

Answer 16

True

Question 17

Ocular JXG is usually bilateral

Answer 17

False- unilateral and develops in <0.5% of patients

Question 18

Oral JXG is common

Answer 18

False - rare; usually lateral tongue or midline of the hard palate

Question 19

JXG have a firm and rubbery consistency

Answer 19

True

Question 20

JXG stains

Answer 20

— CD68+
— CD163+
— Factor XIIIa 
— HAM56
— Fascin
— sometimes s100

Question 21

JXG histo

Answer 21

Sheet like nodular aggregates of histiocytes, lymphocytes, eosinophils with admixed Touton -like giant cells in the dermis

Question 22

JXG can exhibit needle shaped clefts

Answer 22

False - unlikely sclerema neonatorum, SC fat necrosis, post steroid panniculus and cholesterol emboli

Question 23

NXG average age of onset is the eighth decade

Answer 23

False - sixth decade

Question 24

NXG- males to female ratio 1:1

Answer 24

True

Question 25

NXG associated with IgA monoclonal gammopathy

Answer 25

False- IgG

Question 26

NXG have ocular involvement in 5%

Answer 26

False - 50% have ocular involvement with orbital masses, ectropion , ptosis, conjunctival lesions, keratitis and scleritis , anterior uveitis, proptosis

Question 27

SC nodules of NXG need to be distinguished from nodules of RA and SC granuloma annulare

Answer 27

True

Question 28

Reticulohistiocytosis can be a single lesion or multicentric with systemic involvement

Answer 28

True

Question 29

Reticulohistiocytosis pathology shows…

Answer 29

Histiocytes characteristic with abundant Eosinophilic cytoplasm, which is homogenous and finely granular, creating a “ground glass” effect

Question 30

Rosai-Dorfman affects the elderly and not children and young adults

Answer 30

FALSE - affects children and young adults most commonly

— except skin limited form affects adult women most commonly

Question 31

In Rosai-Dorfman, fever and polyclonal hypergammaglobulinaemia are often seen

Answer 31

True

Question 32

Emperipolesis is a common histologic finding of Rosai Dorfman in the skin but not the lymph nodes

Answer 32

False - both

Question 33

Rosai Dorfman and H syndrome have mutations in the same gene SLC29A3

Answer 33

True

Question 34

Males are overall more affected by Rosai Dorfman than females

Answer 34

True

Question 35

Emperipolesis refers to..

Answer 35

Taking up of intact lymphocyte sand plasma cells by histiocytes

Question 36

Rosai Dorfman stains..

Answer 36

CD68, CD163, CD11c, CD14, laminin 5 and lysozyme

Question 37

Xanthoma disseminatum triad.. ?

Answer 37

Cutaneous xanthoma, diabetes insipidus, mucous membrane xanthomas

Question 38

In xanthoma disseminatum, most patients have abnormal lipid levels

Answer 38

False - normal

Question 39

Xanthoma disseminatum can be treated with radiotherapy

Answer 39

True for airway obstructive disease