Chapter 26 - Eosinophilic Dermatoses Flashcards Preview

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Flashcards in Chapter 26 - Eosinophilic Dermatoses Deck (49):
1

Is the histologic dye eosin basic or acidic?

Acidic
*Eosinophils were named this way because eosin binds to them. This is because eosinophils contain basic intracellular granules.

2

What molecule is constitutively expressed by eosinophils that binds to vascular cell adhesion molecule 1 (VCAM-1) on endothelial cells?

Very late antigen 4 (VLA-4)

3

What is the name of the protein that comprises the main constituent of eosinophilic granules?

Major basic protein 1 (MBP1)

4

True or false: major basic protein 1 (MBP1) released from eosinophil granules stimulates the release of histamine from basophils.

True

5

True or false: granuloma faciale can occur on non-facial skin.

True; although when this occurs, there is usually also involvement of the face

6

List the systemic diseases associated with granuloma faciale.

There are none

7

True or false: a Grenz zone is typically seen in biopsies of granuloma faciale.

True

8

What condition is at the top of the clinical (and histological!) differential diagnosis for extra-facial granuloma faciale?

Erythema elevatum diutinum (EED)
*EED has a predilection for skin overlying joints
**EED typically lacks a Grenz zone

9

What is first-line therapy for granuloma faciale?

Intralesional kenalog
*Granuloma faciale is often very treatment resistant
**Oral dapsone or clofazimine can be tried

10

What population is most commonly affected by granuloma faciale?

White middle-aged men

11

What is the most common systemic complaint in patients with Well's syndrome?

Malaise, but fever can also occur (in less than 25% of cases)

12

True or false: in patients with Well's syndrome (eosinophilic cellulitis), eosinophils are present both peripherally and in the biopsy tissue.

True

13

True or false: flame figures are specific to Well's syndrome (eosinophilic cellulitis).

False; they can also be seen in arthropod bites and stings, mastocytomas, scabies, prurigo nodularis, and eczema

14

What are the two most common clinical mimics of Well's syndrome?

Bacterial cellulitis and erysipelas

15

What is first-line therapy for Well's syndrome?

Oral steroids; there's usually a dramatic improvement in a few days
*Tapering of the steroid over one month is usually well tolerated

16

True or false: the FIP1L1-PDGFRA fusion gene responsible for hypereosinophilic syndrome is over 100 times more sensitive to imatinib than the BCR-ABL kinase fusion gene responsible for some cases of CML.

True

17

True or false: patients with the FIP1L1-PDGFRA positive hypereosinophilia syndrome are almost always female.

False; they're almost always male (with a few female case reports)

18

What is the major cause of death in patients with hypereosinophilic syndrome?

Restrictive cardiomyopathy

19

True or false: all cases of hypereosinophilic syndrome are related to FIP1L1-PDFRA fusion genes, and will thus be sensitive to imatinib.

False; only some cases are due to this fusion gene, and thus, not all patients will be appropriate candidates for imatinib

20

What are some of the products of eosinophil during inflammation?

Reactive oxygen intermediates, enzymes, garnule-derived proteins, lipid mediators, cytokines, chemokines

21

What are 2 members of the C-C chemokine gene superfamily that act as chemoattractants for eosinophils?

1. Eotaxin family; 2. regulated on activation normal T cell expressed and secreted (RANTES) family

22

Are eotaxins 1-3 chemotactic for eosinophils only?

Yes

23

Are RANTES chemotactic for eosinophils only?

No. They are also chemotactic for monocytes, T lymphocytes, NK cells, basophils, NOT neutrophils

24

Which of the chemoattractants are stronger at inducing eosinophil function? Eotaxin 1, 2, 3 or RANTES?

Eotaxins 1 and 2 > 3 > RANTES

25

Which epidermal and dermal cells produce which chemoattractants?

Dermal fibroblasts produce Eoataxin 1-3, RANTES; keratinocytes produce RANTES

26

Give one example of an integrin that helps eosinophils transmigrate across blood vessels.

Very late antigen 4 (VLA-4); its corresponding ligand is vascular cell adhesion molecule 1 (VCAM-1)

27

Which integrin expression is critical for eosinophil effector function, eg. degranulation?

CD11b/CD18 (MAC-1)

28

In addition to T cells, what other cells also produce cytokines that contribute to eosinophil activation?

Mast cells, NK cells, eosinophils, all via cytokines

29

How are eosinophil granules released?

Cytolytic degranulation, piece-meal degranulation, regulated secretion

30

What are the components of eosinophil granules?

1) Major basic protein 1 (MBP1), direct damage of helminths, mammalian cells and tissues; also stimulate histamine release from basophils; induce neutrophil release of superoxide and lysozyme. 2) Eosinophil cationic protein (ECP/RNase3), potent toxin for parasites. 3) Eosinophil derived neurotoxin (EDN/RNase2), neurotoxin, antiviral activity against RNA viruses.

31

In addition to MBP1, ECP, EDN, what are other products of eosinophil that help in the anti-microbial/iflammatory response?

Eosinophil peroxidase (EPO) = hydrogen peroxide kills microorganisms; also a platelet agonist causing serotonin release and increase clotting

32

What is the nasal mucosal variant of granuloma faciale?

Eosinophilic angiocentric fibrosis

33

What might you see on histology for granuloma faciale?

Dermal infiltrate made up of eosinophil and other lymphocytes, neutrophils, plasma cells; Grenz zone; normal epidermis

34

What are flame figures?

Hallmark, but not specific for Well's syndrome. On histology, eosinophilic staining figures consisting of non-necrobiotic collagen fibers coated with eosinophil granules

35

What is the diagnostic criteria for hypereosinophilic syndrome (HES)?

1. Peripheral blood eosinophilia (ie. >1500 eosinophils/uL) x 6mths -or- <6mths + organ damage; 2. no other cause of eosinophilia; 3. organ involvement

36

What are some of the subtypes of HES?

Myeloproliferative type (FIP1L1-PDGFRA fusion gene mutation), lymphoproliferative type, eosinophilic vasculitis, episodic angioedema with eosinophilia (EAE), NERDS syndrome (nodules, eosinophilia, rheumatism, dermatitis, swelling)

37

What are possible complications of HES according to their subtypes?

Generally speaking, all HES subtypes can cause eosinophilic endomyocardial disease from prolonged eosinophilia, and embolic events. The leading cause of death is CHF, followed by sepsis. For the myeloproliferative type - leukemia; for the lymphoproliferative type - lymphoma.

38

What is the 1st line treatment for those with FIP1L1-PDGFRA fusion gene mutation vs those without this mutation?

Imatinib mesylate (Gleevec) vs prednisone

39

True or false: granuloma faciale has been associated with systemic disease

False

40

True or false : granz zone a leukocytoclastic vasculitis are present in granuloma faciale

True

41

What is the first line of treatment for granuloma faciale

Intralesional triamcinolone 2.5-5 mg/ml

42

What is characteristic about well's syndrome pathology?

Flame figures

* not specific

43

What are the possible triggers for well's syndrome?

Myeloproliferative diseases
Infections
Infestations
Insect bites
Drugs

44

What are the most common mimics of well's syndrome!

Cellulitis
Erysipelas

45

What is the first line of treatment for well's syndrome?

Prednisone 10-80 mg daily.

Tapered over 1 month

46

What cytokine is mainly produced in lymphocytic form of HES?

IL-5

47

What is NERDS syndrome

Nodules
Esinophilia
Rheumatism
Dermatitis
Swelling

48

What is the major cause of death in HES?

CHF from restrictive cardiomyopathy

49

What are the Th1 cytokines

IL-2
IL-12
IFN-gamma
TNF-a