B-90/91/92/93 Histiocytosis/xanthomas/non-Infectious/fb Granulomas

Front 1

Name the four diseases that comprise Langerhans cell histiocytosis

Back 1

Letterer-Siwe Disease (0-2y/o)

Hand-Schuller-Christian Disease (2-6y/o); Triad (DI, bone lesions, exophthalmos)

Eosinophilic Granuloma (7-12y/o); bone lesions primarily

Congenital Self-Healing Reticulohistiocytosis (aka
Hashimoto-Pritzker disease) (congenital); self-healing, mainly skin involvment

Front 2

What is another name for Congenital Self-Healing Reticulohistiocytosis?

Back 2

Hashimoto-Pritzker Disease (may present as blueberry muffin baby)

Front 3

Name some LCH cell stains

Back 3

S100+
CD1a+
CD207-Langerin+
alpha-D-mannosidase+
CD45+
CD101+
HLA-DR+

Front 4

What endocrine disorder is assoc with LCH?

Back 4

Diabetes Insipidus

Front 5

What bone abnormality is seen in LCH?

Back 5

Osteolytic bone lesions are common

Front 6

What are the three 'histiocytes' of cutaneous importance?

Back 6

1. Langerhans cell
2. mononuclear cell/macrophages
3. dermal dendrocytes

Front 7

What is the triad of Hand-Schuller-Christian disease?

Back 7

Diabetes insipidus
Bone lesions
Exophthalmos

Front 8

Patients with LCH have increased risk of what malignancy?

Back 8

Solid Tumors and Leukemia (ALL or Acute non-lymphoblastic leukemia)

Front 9

What conditions have comma-shaped bodies and worm-like particles?

Back 9

LCH, juvenile xanthogranuloma, generalized eruptive histiocytoma, and Rosai-Dorfman disease

Front 10

Histiocytosis with intracytoplasmic worm-like bodies is aka?

Back 10

Benign Cephalic Histiocytosis

Front 11

Name the Dz:
Infants < 1 y/o
Red to brown macules and papules on face and neck
Self-limiting dz
worm-like bodies

Back 11

Benign Cephalic Histiocytosis

Front 12

This dz:
affects adults more than children
recurrent crops of red to brown papules
widespread axial distribution
self-limited

Back 12

Generalized eruptive histiocytoma

Front 13

Immunophenotype for Indeterminante Cell Histiocytosis?

Back 13

S100 and CD1a (like in LCH) as well as non-LCH (CD68+)

Front 14

Juvenile Xanthogranuloma (MC histiocytosis) -- can be assoc with what dz?

Back 14

rare assoc with:
NF type 1 (JXG assoc with CALM)
Juvenile myelomonocytic leukemia
(ocular lesions can cause blindness)

Front 15

What is the most common histiocytosis?

Back 15

Juvenile Xanthogranuloma

Front 16

What immunohistochemical stains are seen in JXG?

Back 16

Positive HAM56, CD68, factor XIIIa
Sometimes S100+
CD1a usually negative

Front 17

What is the most common extracutaneous invovlvement in JXG?

Back 17

#1. Eyes - can cause Hyphema & Glaucoma, blindness
#2. Lung

Front 18

What are the key clinical features and findings in Necrobiotic Xanthogranuloma?

Back 18

1. Avg Age of onset = 6th decade
2. Strong assoc with with Paraproteinemia (IgG monoclonal gammopathy in 80%of pts)
3. Increased risk of plasma cell dyscrasias and lymphoproliferative disorders

Front 19

What are the key features in Reticulohistiocytosis?

Back 19

1. single to multicentric
2. primarily seen in adults (caucasian)
3. 50% develop mucous membrane lesions (oral and nasopharyngeal -- can cause leonine faces)
4. Assoc with destructive arthritis and solid-organ malignancies (bronchial, breast, stomach and cervical carcinomas are most common)
5. Periungual lesions can have "ground glass" appearance

Front 20

Sinus histiocytosis with massive lymphadenopathy Aka?

Back 20

Rosai-Dorfman Disease

Front 21

what are key clinical findings in Rosai-Dorman dz?

Back 21

UNKNOWN ETIOLOGY, ?assoc with HHV 6
Children and young adults

1. massive, but painless bilateral cervical LAD
2. fever/anemia/elevated ESR
3. neutrophilia and polyclonal hypergammaglobulinemia

Front 22

Most common extranodal involvement in Rosai-Dorfman?

Back 22

After Lymph nodes...
skin and soft tissue, the eyelid and orbit, the upper respiratory tract, major salivary glands, CNS and bone

Front 23

What is emperipolesis?

Back 23

taking up of intact lymphocytes and plasma cells by histiocytes -- see in rosai-dorfman

Front 24

Immunohistochemical stains in Rosai-Dorfman?

Back 24

S100+
CD11c+, CD14+, CD68+
Factor XIIIa+
CD1a negative

Front 25

Disseminated Xanthosiderohistiocytosis aka?

Back 25

Xanthoma Disseminatum

Front 26

Key features of Xanthoma Disseminatum

Back 26

1. Classic Triad: Cutaneous xanthomas, mucous membrane xanthomas, and diabetes insipidus
2. symmetric distribution
3. flexural and intertriginous involvement
4. upper airway lesions

Front 27

What % of patients have mucous memb involvement in Xanthoma Disseminatum? CNS?

Back 27

40-60% have MM
40% have CNS involvement (hypothalamus and pituitary gland)

Front 28

What three clinical outcomes are seen in Xanthoma Disseminatum?

Back 28

1. rare self-healing form
2. common persistent form
3. rare progressive form

Front 29

What is Langerin?

Back 29

CD207
Transmembrane cell surface receptor

Front 30

What two diseases has JXG been associated with?

Back 30

NF1 and Juvenile CML

Front 31

Most common site for Necrobiotic Xanthogranuloma

Back 31

Periorbital region -- Ophthalmic manifestations common

Front 32

What gammopathy is associated with NXG?

Back 32

Associated IgG monoclonal gammopathy (one case of IgA); 40% with cryoglobulinemia

Front 33

What virus is found in many Rosai Dorfman cases (in the LNs)?

Back 33

HHV6

Front 34

Most common association/involvement in Rosai Dorfman (besides LN)?

Back 34

Uveal involvement

Front 35

Eruptive Xanthomas can be seen in which hyperlipidemias?

Back 35

Seen in hypertriglyceridemia:
Type I, Type IV, V

Front 36

What are the skin findings in:
Type I Hyperlipoproteinemias?
Lab findings?

Back 36

Eruptive Xanthoma

slow chylomicron clearance, reduced LDL and HDL
elevated TGs

Front 37

What are the skin findings in:
Type II Hyperlipoproteinemias?
Lab findings?

Back 37

Tendinous, tuberoeruptive, tuberous, plane (xanthelasma, intertriginous areas, interdigital web spaces)

Reduced LDL Clearance
Hypercholesterolemia

Front 38

What are the skin findings in:
Type III Hyperlipoproteinemias?
Lab findings?

Back 38

Tuberoeruptive, tuberous, plane (palmar creases), tendinous

Elevated levels of chylomicron remnants and IDLs
Hypercholesterolemia
HyperTGs

Front 39

What are the skin findings in:
Type IV Hyperlipoproteinemias?
Lab findings?

Back 39

Eruptive Xanthoma

Increased VLDLs
HyperTGs

Front 40

What are the skin findings in:
Type V Hyperlipoproteinemias?
Lab findings?

Back 40

Eruptive Xanthoma

Decreased LDLs and HDLs
HyperTGs

Front 41

What is the role of Apolipoprotein C-II?

Back 41

Activates lipoprotein lipase

Front 42

What is the role of Apolipoprotein E/B-100?

Back 42

Binds LDL receptor allowing uptake in the liver

Front 43

What is the role of Apolipoprotein A-I?

Back 43

Required for activation of Lecithin: Cholesterol acyltransferase (LCAT)

Front 44

What is the role of Apolipoprotein B-48?

Back 44

Exclusively found in chylomicrons

Front 45

Lofgren's syndrome

Back 45

Erythema Nodosum
Hilar Adenopathy
Fever
Arthritis

Front 46

Heerfordt's syndrome

Back 46

Parotid gland enlargement, uveitis, fever, cranial nerve palsy

Front 47

Mikulicz Disease

Back 47

parotitis, salivary/lacrimal gland enlargement, xerostomia, decreased lacrimation

Front 48

Darier-Roussy disease

Back 48

subcutaneous sarcoidosis

Front 49

What HLAs are assoc with Sarcoid?

Back 49

Genetic susceptibility associated with HLA-1, HLA B-8, and HLA-DR3 alleles

Front 50

Is CD4 or CD8 predominant in Sarcoid granulomas?

Back 50

Cutaneous granulomas with a predominance of CD4+ helper T cells and a paucity of CD8+ suppressor T cells, B cells, and immunoglobulin

Front 51

What is Kveim-Siltzbach antigen?

Back 51

Suspension of sarcoidal spleen injected in the skin leads to formation of typical granuloma in sarcoidosis patients

Front 52

Lupus pernio?
Assoc with what complications of sarcoid?

Back 52

papulonodules and plaques of the nose, ears and cheeks
important to recognize this finding is associated with chronic pulmonary sarcoidosis (75%) and upper airway sarcoidosis (50%)

Front 53

Childhood sarcoid has what triad?

Back 53

Arthritis, uveitis, and cutaneous lesions

Front 54

What % of pts have elevated ESR in Sarcoid?

Back 54

40%

Front 55

What % of sarcoid pts have hilar and/or paratracheal LAD?

Back 55

90%

Front 56

What are Asteroid bodies?

Back 56

engulfed collagen = eosinophilic stellate inclusions

Front 57

What are Schaumann bodies?

Back 57

rounded basophilic inclusions = degenerating lysosomes

Front 58

What are some common lab findings in sarcoid patients?

Back 58

elevated ANA in 30%; elevated serum ACE in 60%; also check for elevated ESR, anemia, eosinophilia, lymphopenia, and hypercalcemia

Front 59

Demographic for Granuloma Annulare?

Back 59

kids & young adults (2/3s under age 30) and more common in females (2:1)

Front 60

What diseases are associated with GA (only rarely though!)

Back 60

DM & Lymphoma

Front 61

HLA Associated with generalized GA?

Back 61

HLA-Bw35

Front 62

Annular Elastolytic Giant Cell Granuloma is aka?

Back 62

Actinic granuloma (of O’Brein)

Giant cell elastophagocytosis

Miescher’s granuloma of the face

Atypical (annular) necrobiosis lipoidica of the face and scalp

Front 63

Name the condition:
Asymptomatic annular plaques on the head, neck, and other sun exposed areas
Borders are elevated and erythematous with slightly atrophic and hypopigmented center
Non-palisading granulomas with FBT giant cells, histiocytes, and lymphocytes
Elastic fibers are absent in the center of lesions, and some elastic fibers are found within giant cells
No collagen alteration, mucin, or lipid deposition; no vascular changes

Back 63

Annular Elastolytic Giant Cell Granuloma

Front 64

What % of Crohn's patients will present with skin findings?

Back 64

20%
No correlation with skin findings and intestinal activity

Front 65

% with genital or extra genital crohns?

Back 65

2/3 of kids have genital findings
1/2 of adults have genital findings

Front 66

What % have pt's have continguous crohn's (connecting to intestinal crohns)?

Back 66

1/3 of pts have Contiguous Crohns

Front 67

What % of pt's have oral dz in crohns?

Back 67

5-20% (90% granulomatous oral findings)

Front 68

Other Cutaneous Manifestations of Crohns?

Back 68

PAN
EN
EM
LCCV
EBA
Pathergy
Pyoderma gangrenosum
Zinc deficiency - acrodermatitis enteropathica-like syndrome

Front 69

What can cause introduction of the following FBs into the body for:
Silica
Talc
Zirconium
Berylium
Aluminum
Zinc

Back 69

Silica - blast injuries
Talc - surgical procedures
Zirconium - deodorants
Berylium - Inhalation
Aluminum - Vaccines
Zinc - Insulin-zinc preparations

Front 70

Name the Tatoo color:
Cinnabar and mercury

Back 70

Red

Front 71

Name the Tatoo color: Cobalt

Back 71

Blue

Front 72

Name the Tatoo color: Chromium

Back 72

Green

Front 73

Name the Tatoo color: Cadmium

Back 73

Yellow

Front 74

Name the Tatoo color: Manganese

Back 74

Purple

Front 75

Name the Tatoo color: Ferric Hydrate

Back 75

Ochre (can be a purple ochre or red ochre)

Front 76

Name the Laser for: Blue/Black

Back 76

Q-switched ruby (694 nm), Q-switched alexandrite (755 nm), Q-switched Nd:YAG (1064 nm)

Front 77

Name the Laser for: Green

Back 77

Q-switched alexandrite (755 nm), QS-ruby (694 nm)

Front 78

Name the Laser for: Red/Orange/Purple

Back 78

Q-switched, frequency-doubled Nd:YAG (532 nm); pigmented pulsed dye (510 nm)

Front 79

Name the Laser for: Yellow

Back 79

Pigmented pulsed dye (510 nm)

Front 80

How can you distinguish silicone from paraffin on histology?

Back 80

Silicone will not stain with lipid stains

Front 81

If you see someone with granulomas around the umbilical stump, intertriginous areas and IV drug sites -- what might you think about?

Back 81

Talc (magnesium silicate)

Front 82

What can you use to detect zirconium particles?

Back 82

Energy-dispersive X-Ray analysis

Front 83

What is paler staining, less fibrillar, and is not birefrignent under polarized light?

Back 83

implanted bovine collagen

Front 84

What is Sabra dermatitis?

Back 84

skin colored papules with black dot resulting from cactus spine exposure