Non-langerhan cell histiocytoses

Question 1

All non-langerhan cell histiocytoses stain positive for _____ and negative for _____

Answer 1

CD68+

Langerin -

Question 2

JXG presents as:

Answer 2

one to few pink to red/yellow lesions on the head/neck > trunk/extremities

Question 3

What is age of onset and the clinical course/prognosis of JXG?

Answer 3

• 75% present in first year of life

- spontaneous resolution in 3-6 years

Question 4

What are the risk factors for ocular involvement with JXG?

Answer 4

multiple cutaneous JXG’s and age < 2 y/o

Question 5

What is the “triple association” of JXG?

Answer 5

JXG + NF-1 and 20x increased risk of juvenile myelomonocytic leukemia

Question 6

Histopath of JXG:

Answer 6

Well-circumscribed dermal infiltrate of foamy lipidized histiocytes with touton giant cells

Question 7

All patients with multiple JXG should be referred to_____

Answer 7

ophthalmologist

Question 8

Benign cephalic histiocytosis presents as:

Answer 8

numerous red-brown-yellow papules on the face/neck

Question 9

Is there oral/visceral involvement in Benign cephalic histiocytosis?

Answer 9

No! (unlike other histiocytoses)

Question 10

What is the clinical course/prognosis of Benign cephalic histiocytosis?

Answer 10

benign, self-limiting, resolve between 2-8 years old

Question 11

Gerneralized eruptive histiocytosis presents as:

Answer 11

recurrent eruption of hundreds of small red-brown-yellow papules in axial distribution (likely a JXG variant)

Question 12

What is clinical course/prognosis of Gerneralized eruptive histiocytosis?

Answer 12

self-limited, no internal or mucosal involvement

Question 13

Reticulohistiocytosis presents at what age?

Answer 13

30s-40s

Question 14

Multicentric Reticulohistiocytosis presents as:

Answer 14

Red-brown-yellow papules on acral sites (dorsal hands) and head w/ nasopharyngeal lesions and destructive arthritis

- coral bead appearance of lesions

Question 15

Multicentric Reticulohistiocytosis is a/w _____ in 30% of cases

Answer 15

solid organ malignancy

Question 16

Solitary Reticulohistiocytosis clinical course/prognosis?

Answer 16

Self resolving, no systemic involvment (unlike multicentric form which has severe joint involvment and underlying malignancy in 30% of patients)

Question 17

Xanthoma disseminatum presents with triad of:

Answer 17

• cutaneous xanthomas (hundreds of papules coalescing into oddly-patterned plaques favoring head and intertrigenous sites)
• mucosal xanthomas
• diabetes insipidus

Question 18

Xanthoma disseminatum is a/w_____

Answer 18

Monoclonal gammopathy, plasma cell dyscrasia