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Bolognia Book Club > Chapter 26 - Eosinophilic Dermatoses > Flashcards

Chapter 26 - Eosinophilic Dermatoses Flashcards

1
Q

Is the histologic dye eosin basic or acidic?

A

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

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2
Q

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

A

Very late antigen 4 (VLA-4)

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3
Q

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

A

Major basic protein 1 (MBP1)

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4
Q

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

A

True

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5
Q

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

A

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

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6
Q

List the systemic diseases associated with granuloma faciale.

A

There are none

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7
Q

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

A

True

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8
Q

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

A

Erythema elevatum diutinum (EED)

  • EED has a predilection for skin overlying joints
  • *EED typically lacks a Grenz zone
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9
Q

What is first-line therapy for granuloma faciale?

A

Intralesional kenalog

  • Granuloma faciale is often very treatment resistant
  • *Oral dapsone or clofazimine can be tried
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10
Q

What population is most commonly affected by granuloma faciale?

A

White middle-aged men

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11
Q

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

A

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

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12
Q

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

A

True

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13
Q

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

A

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

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14
Q

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

A

Bacterial cellulitis and erysipelas

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15
Q

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

A

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

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16
Q

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.

A

True

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17
Q

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

A

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

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18
Q

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

A

Restrictive cardiomyopathy

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19
Q

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

A

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

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20
Q

What are some of the products of eosinophil during inflammation?

A

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

21
Q

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

A
  1. Eotaxin family; 2. regulated on activation normal T cell expressed and secreted (RANTES) family
22
Q

Are eotaxins 1-3 chemotactic for eosinophils only?

A

Yes

23
Q

Are RANTES chemotactic for eosinophils only?

A

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

24
Q

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

A

Eotaxins 1 and 2 > 3 > RANTES

25
Q

Which epidermal and dermal cells produce which chemoattractants?

A

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

26
Q

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

A

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

27
Q

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

A

CD11b/CD18 (MAC-1)

28
Q

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

A

Mast cells, NK cells, eosinophils, all via cytokines

29
Q

How are eosinophil granules released?

A

Cytolytic degranulation, piece-meal degranulation, regulated secretion

30
Q

What are the components of eosinophil granules?

A

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
Q

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

A

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

32
Q

What is the nasal mucosal variant of granuloma faciale?

A

Eosinophilic angiocentric fibrosis

33
Q

What might you see on histology for granuloma faciale?

A

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

34
Q

What are flame figures?

A

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

35
Q

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

A
  1. Peripheral blood eosinophilia (ie. >1500 eosinophils/uL) x 6mths -or- <6mths + organ damage; 2. no other cause of eosinophilia; 3. organ involvement
36
Q

What are some of the subtypes of HES?

A

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

37
Q

What are possible complications of HES according to their subtypes?

A

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
Q

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

A

Imatinib mesylate (Gleevec) vs prednisone

39
Q

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

A

False

40
Q

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

A

True

41
Q

What is the first line of treatment for granuloma faciale

A

Intralesional triamcinolone 2.5-5 mg/ml

42
Q

What is characteristic about well’s syndrome pathology?

A

Flame figures

  • not specific
43
Q

What are the possible triggers for well’s syndrome?

A 
Myeloproliferative diseases
Infections
Infestations
Insect bites
Drugs
44
Q

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

A

Cellulitis

Erysipelas

45
Q

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

A

Prednisone 10-80 mg daily.

Tapered over 1 month

46
Q

What cytokine is mainly produced in lymphocytic form of HES?

A

IL-5

47
Q

What is NERDS syndrome

A 
Nodules
Esinophilia
Rheumatism
Dermatitis
Swelling
48
Q

What is the major cause of death in HES?

A

CHF from restrictive cardiomyopathy

49
Q

What are the Th1 cytokines

A

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

Bolognia Book Club (29 decks)

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