Macrophage Disorders Flashcards
GA recurrence rate
40%
Women with localized GA
Incr risk of autoimmune thyroiditis
Uveitis also described with localized GA
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
MC GA in HIV
Generalized
Meds associated with GA
IFN, TNF inh, mesotherapy
Also borrelia, TB, Lyme
GA tx
PDL, Fraxel, doxy +- rifampin, fumaric acid, severe tnf -
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
Granuloma multiforme (leiker)
Africa
Upper trunk, arms, sun exposed
Like actinic granuloma
Sarcoidosis HLA
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
MC morphology of sarcoid
Papular sarcoid
Pt with hep c receiving IFN
High risk of developing sarcoidal rxns after fillers
Meds that induce sarcoid
Tnf inh, IFN in the setting of hep c, haart (enhance helper T cell number), SCT, alemtuzumab and ipilimumab
Most common Lch
Jxg
Jxg stains
XIIIa, vimentin, fascin, MS-1, CD68
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
Papular nodular histiocytosis
40-60
Diffuse, spare flexures
Leonine facies
Spindled histiocytes
Papular xanthoma
M>F No flexursl predilection No visceral inv and no lipid Abn Neg XIIIa Doxy may tx
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
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
Indeterminate cell histiocytosis
Stain for LCH but no birbeck granules
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
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
