Macrophage Disorders

Question 1

GA recurrence rate

Answer 1

40%

Question 2

Women with localized GA

Answer 2

Incr risk of autoimmune thyroiditis

Uveitis also described with localized GA

Question 3

Acute onset painful acral GA

Answer 3

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

Question 4

MC GA in HIV

Answer 4

Generalized

Question 5

Meds associated with GA

Answer 5

IFN, TNF inh, mesotherapy

Also borrelia, TB, Lyme

Question 6

GA tx

Answer 6

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

Question 7

Interstitial granulomatous drug eruption

Answer 7

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

Have more interface and atypical lymphos vs GA

Question 8

Granuloma multiforme (leiker)

Answer 8

Africa
Upper trunk, arms, sun exposed
Like actinic granuloma

Question 9

Sarcoidosis HLA

Answer 9

DQB10201 and DRB10301 - acute sarcoidosis with good prognosis

Butyrophilin-like 2 gene (BTNL2) associated with sarcoid

TNF mutations and LTA associated with EN In Sarcoid

Question 10

MC morphology of sarcoid

Answer 10

Papular sarcoid

Question 11

Pt with hep c receiving IFN

Answer 11

High risk of developing sarcoidal rxns after fillers

Question 12

Meds that induce sarcoid

Answer 12

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

Question 13

Most common Lch

Answer 13

Jxg

Question 14

Jxg stains

Answer 14

XIIIa, vimentin, fascin, MS-1, CD68

Question 15

Generalized eruptive histiocytoma

Answer 15

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

Question 16

Papular nodular histiocytosis

Answer 16

40-60
Diffuse, spare flexures
Leonine facies
Spindled histiocytes

Question 17

Papular xanthoma

Answer 17

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

Question 18

Erdheim chester disease

Answer 18

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

Question 19

Progressive mucinous histiocytosis in women

Answer 19

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

Question 20

Indeterminate cell histiocytosis

Answer 20

Stain for LCH but no birbeck granules

Question 21

Sea blue histiocytosis

Answer 21

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

Question 22

Adult LCH

Answer 22

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