Chapter 26 - Eosinophilic Dermatoses Flashcards
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.
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)
What is the name of the protein that comprises the main constituent of eosinophilic granules?
Major basic protein 1 (MBP1)
True or false: major basic protein 1 (MBP1) released from eosinophil granules stimulates the release of histamine from basophils.
True
True or false: granuloma faciale can occur on non-facial skin.
True; although when this occurs, there is usually also involvement of the face
List the systemic diseases associated with granuloma faciale.
There are none
True or false: a Grenz zone is typically seen in biopsies of granuloma faciale.
True
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
What is first-line therapy for granuloma faciale?
Intralesional kenalog
- Granuloma faciale is often very treatment resistant
- *Oral dapsone or clofazimine can be tried
What population is most commonly affected by granuloma faciale?
White middle-aged men
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)
True or false: in patients with Well’s syndrome (eosinophilic cellulitis), eosinophils are present both peripherally and in the biopsy tissue.
True
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
What are the two most common clinical mimics of Well’s syndrome?
Bacterial cellulitis and erysipelas
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
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
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)
What is the major cause of death in patients with hypereosinophilic syndrome?
Restrictive cardiomyopathy
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
What are some of the products of eosinophil during inflammation?
Reactive oxygen intermediates, enzymes, garnule-derived proteins, lipid mediators, cytokines, chemokines
What are 2 members of the C-C chemokine gene superfamily that act as chemoattractants for eosinophils?
- Eotaxin family; 2. regulated on activation normal T cell expressed and secreted (RANTES) family
Are eotaxins 1-3 chemotactic for eosinophils only?
Yes
Are RANTES chemotactic for eosinophils only?
No. They are also chemotactic for monocytes, T lymphocytes, NK cells, basophils, NOT neutrophils
Which of the chemoattractants are stronger at inducing eosinophil function? Eotaxin 1, 2, 3 or RANTES?
Eotaxins 1 and 2 > 3 > RANTES
