Macrophage Disorders Flashcards Preview

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Flashcards in Macrophage Disorders Deck (22):
1

GA recurrence rate

40%

2

Women with localized GA

Incr risk of autoimmune thyroiditis

Uveitis also described with localized GA

3

Acute onset painful acral GA

F with rapid onset acral lesions
May be dusky and resemble Em
Arthritis, fever, diarrhea
Steroids or plaquinel may tx

4

MC GA in HIV

Generalized

5

Meds associated with GA

IFN, TNF inh, mesotherapy

Also borrelia, TB, Lyme

6

GA tx

PDL, Fraxel, doxy +\- rifampin, fumaric acid, severe tnf -

7

Interstitial granulomatous drug eruption

Lipid lowering agents, ACE inh, diuretics, NSAIDS, antihistamines, aeds, antidepr, allopurinol, senna, thalidomide, gancyclovir, TNF -

Have more interface and atypical lymphos vs GA

8

Granuloma multiforme (leiker)

Africa
Upper trunk, arms, sun exposed
Like actinic granuloma

9

Sarcoidosis HLA

DQB1*0201 and DRB1*0301 - acute sarcoidosis with good prognosis

Butyrophilin-like 2 gene (BTNL2) associated with sarcoid

TNF mutations and LTA associated with EN In Sarcoid

10

MC morphology of sarcoid

Papular sarcoid

11

Pt with hep c receiving IFN

High risk of developing sarcoidal rxns after fillers

12

Meds that induce sarcoid

Tnf inh, IFN in the setting of hep c, haart (enhance helper T cell number), SCT, alemtuzumab and ipilimumab

13

Most common Lch

Jxg

14

Jxg stains

XIIIa, vimentin, fascin, MS-1, CD68

15

Generalized eruptive histiocytoma

Widespread papules of trunk and prox ext, spares flexures
Rare MM, no visceral
Progressive new crops of lesions until involution
Young adulthood
May follow illness, be seen in malignancy (leak or lymphoma)

Differentiated from xanthoma disseminatum my by lack of visceral dz, benign course, and scalloped macrophages in XD

Vimentin, CD68, usually XIIIa

PUVA or accutane may tx

16

Papular nodular histiocytosis

40-60
Diffuse, spare flexures
Leonine facies
Spindled histiocytes

17

Papular xanthoma

M>F
No flexursl predilection
No visceral inv and no lipid Abn
Neg XIIIa
Doxy may tx

18

Erdheim chester disease

Non LCH primarily visceral with cutaneous inv
BL and symmetric sclerosis of the metaphyseal and diaphyseal bone
DI
Retroperitoneal fibrosis affecting kidney
Often fatal from pulmonary fibrosis or cardiac failure

19

Progressive mucinous histiocytosis in women

AD
Pea sized red brown papules of face, arms, forearms, hands, leg
No visceral or mucosal
+ acid mucopoly
Lack of lipidized and MNGC
CD68 and XIIIa pos

20

Indeterminate cell histiocytosis

Stain for LCH but no birbeck granules

21

Sea blue histiocytosis

May be familial
Giemsa stains blue green
May-gruenwald stains blue
Infiltrates reticuloendothelial system
Facial waxy papules, eyelid swelling patchy gray pigmentation of face and upper trunk

Similar histo in myelogenous leukemia, nieman pick, sphimgomyelinase def, apo E mutation, prolonged IV fat suppl

22

Adult LCH

Bone MC
90% are tobacco and MJ smokers
Pneumothorax in 25%
Association with leukemia and solid organ CA thought to be 2' from chemo