Non-langerhan cell histiocytoses Flashcards
All non-langerhan cell histiocytoses stain positive for _____ and negative for _____
CD68+
Langerin -
JXG presents as:
one to few pink to red/yellow lesions on the head/neck > trunk/extremities
What is age of onset and the clinical course/prognosis of JXG?
- 75% present in first year of life
- spontaneous resolution in 3-6 years
What are the risk factors for ocular involvement with JXG?
multiple cutaneous JXG’s and age < 2 y/o
What is the “triple association” of JXG?
JXG + NF-1 and 20x increased risk of juvenile myelomonocytic leukemia
Histopath of JXG:
Well-circumscribed dermal infiltrate of foamy lipidized histiocytes with touton giant cells
All patients with multiple JXG should be referred to_____
ophthalmologist
Benign cephalic histiocytosis presents as:
numerous red-brown-yellow papules on the face/neck
Is there oral/visceral involvement in Benign cephalic histiocytosis?
No! (unlike other histiocytoses)
What is the clinical course/prognosis of Benign cephalic histiocytosis?
benign, self-limiting, resolve between 2-8 years old
Gerneralized eruptive histiocytosis presents as:
recurrent eruption of hundreds of small red-brown-yellow papules in axial distribution (likely a JXG variant)
What is clinical course/prognosis of Gerneralized eruptive histiocytosis?
self-limited, no internal or mucosal involvement
Reticulohistiocytosis presents at what age?
30s-40s
Multicentric Reticulohistiocytosis presents as:
Red-brown-yellow papules on acral sites (dorsal hands) and head w/ nasopharyngeal lesions and destructive arthritis
- coral bead appearance of lesions
Multicentric Reticulohistiocytosis is a/w _____ in 30% of cases
solid organ malignancy
Solitary Reticulohistiocytosis clinical course/prognosis?
Self resolving, no systemic involvment (unlike multicentric form which has severe joint involvment and underlying malignancy in 30% of patients)
Xanthoma disseminatum presents with triad of:
- cutaneous xanthomas (hundreds of papules coalescing into oddly-patterned plaques favoring head and intertrigenous sites)
- mucosal xanthomas
- diabetes insipidus
Xanthoma disseminatum is a/w_____
Monoclonal gammopathy, plasma cell dyscrasia
