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(Ch25) Eosinophilic dermatoses Flashcards

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1
Q

DDx EOSINOPHILIC SPONGIOSIS ?

A

HAAAPPPIED
Herpes gestationis
Arthropod bite
ACD
AD
Pemphigus
Pemphigoid
Polymorphous eruption of pregnancy (PEP)
Incontinentia pigmenti (stage 1 and 2)
Erythema toxicum
Drug

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2
Q

GROWTH FACTORS for Eosin

A
  • IL-3
  • GM-CSF
  • IL-5
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3
Q

Most potent growth factor for Eosin

A

GM-CSF

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4
Q

Most Selective growth factor for Eosin

A

IL-5: JAK2/STAT5 pathway

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5
Q

Factors chemoattract/recurit Eosin to tissue

A
  • Platelet activaing factor (PAF)
  • CCL11 (eotaxin),
  • CCL24 (eotaxin 2),
  • CCL26 (eotaxin3)
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6
Q

Most potent eosin chemoattractant

A

Platelet activaing factor (PAF)

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7
Q

Release of Eosin granules in 3 ways mention them?

A
  1. cytolytic degranulation - organelle rupture,
  2. Piecemeal degranulation
  3. Exocytosis
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8
Q

Eosin Primary Granules

A

Charcot Leyden crystal (Galactin-10)

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9
Q

Eosin 2˚/Secretory Granules 5 components

A

Major basic protein 1
Major basic protein 2
Eosinophil derived neurotoxin
Eosinophil catonic protein
Eosinophil peroxidase (most numerous)

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10
Q

Eosin Small granules

A

Acid phosphatase
Arylsulfatase

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11
Q

Eosin Secretory vesicles

A

Albumin

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12
Q

Eosin Non-membrane bound lipid bodies

A

Arachidonic acid
Cyclooxygenease
5&15 lipooxygenase
LTC4

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13
Q

Ddx for PSEUDOCELLULITIS

A
  1. Infections and bites
    • Arthropod bite reactions (e.g. insect, spider)
      -Erythema migrans

      -Herpes zoster

      -Toxin-mediated erythema (e.g. recurrent toxin-mediated perineal erythema
    1. Neutrophilic dermatoses
      - Sweet’s syndrome
      - Neutrophilic panniculitis
(Pancreatic, Facticial)
      - Familial Mediterranean fever
  2. Drug reactions
    • Fixed drug eruptions (esp Non-Pigmenting Adverse rx: NSAIDs, Pseudoepherine, Acetaminophen)

    • Vaccine/injection site reactions

    • Toxic erythema of chemotherapy
    1. Metabolic disorders
      Gout

    2. Malignancy
      Erysipeloid skin metastases
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14
Q

ddx for persistent red facial plaque with epidermal changes

A

DLE
Subacute SLE
Tinea
SD
Perioral dermatitis
BCC
SCC
Leprosy

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15
Q

ddx for persistent red facial plaque without epidermal changes

A

LLLLLL
Lymphoma
Leukemia cutis
Lupus tumidus
Polymorphous Light Eruption
Lyme
syphilis
Granuloma faciale

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16
Q

Granuloma faciale clinical appearance

A

Prominent follicular openings
2/3 solitary

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17
Q

Granuloma faciale variant with Sinonasal mucosal involvement

A

esoinophilic angiocentric fibrosis

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18
Q

Typical histopath of Granuloma faciale

A

Grenz zone
+ LCV, Eosin, neut

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19
Q

Grenz zone ddx

A

lymphoma/leukemia
Granuloma faciale
Erythema elevatum diutinum

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20
Q

Rx for Granuloma faciale

A

Resistant
Intralesional triamcinolone (2.5-5mg/ml)
Dapsone (50-150 mg/d)
Clofazimine (300mg daily) - ?availability
Topical PUVA or calcineurin inhbitors
Physical: excision, cryosurgery, dermabrasion, electrosurgery and CO2 or 595 PDL  can recur

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21
Q

which patients are at higher risk/ As/w Exaggerated Insect Bite and Insect Bite-Like reactions

A

CLL > other myeloproliferative disorders
EBV associated NK/T-cell lymphoma, nasal type

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22
Q

Papuloerythroderma of Ofuji clinical sign

Study These Flashcards
A

deck-chair sign

23
Q

deck-chair sign ddx

Study These Flashcards
A

Papuloerythroderma of Ofuji

Waldenstrom’s Macroglobulinemia

24
Q

Which ethnic group at higher risk for Papuloerythroderma of Ofuji

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A

2/3 – elderly Japanese men (avg 72yo)

25
Papuloerythroderma of Ofuji histopath
Eosinophilia Lymphopenia
26
Papuloerythroderma of Ofuji Rx
PUVA +/- acitretin  2/3 cleared + ¼ improved UVB, cyclosporine, etretinate and azathiopurine
27
which Tx is ineffective for Papuloerythroderma of Ofuji
TCS
28
Well's Syndrome other name
eosinophilic cellulitis
29
Well's synd Triggers
insect bites drugs, allergic contact dermatitis, malignancy, and infections
30
which Histopth sign is characteristic but not diagnostic nor pathognmenic for Well's
flame figures
31
What are flame Figures
eosinophil degranulation onto collagen
32
Which part of skin is normal in histopth of Well's
Epidermis
33
Ddx for Flame figures
**FLAMEED** Fungus/dermatophyte infections Lung (bronchiogenic) carcinoma Arthropod bites/stings, scabies Mastocytomas, bullous pemphigoid Eczema, HES, prurigo nodularis Drug
34
Rx of Well's
Steroids Cyclosporine Colchicine Dapsone Mepolizumab
35
Hypereosinophilic Syndrome types
1. Myeloproliferative( primary/neoplastic) 2. Lymphoproliferative (2ndry) 3. Overlap
36
Hypereosinophilic Syndrome diagnostic criteria
1.Evidence of Eosinophilia -peripheral >1500(x2 1m) and/or - BM bx >20% eosin and/or -Path extensive eosin 2. End organ damage/dysfunction due to eosin 3. exclusion of 2ndry causes
37
Myeloproliferative HES mutation and chromosome
FIP1L1-PDGFRA (chrom 4)
38
FIP1L1-PDGFRA results in?
activated tyrosine kinase
39
mention the 4Ms associations of Myeloproliferative HES
Male predominance Mastocytosis endoMyocardial dis Mucosal inovelevment
40
what other findings in Myeloproliferative HES
inc tryptase and Vit B12, tissue fibrosis, splenomegaly, and bone marrow biopsies with  CD25+ atypical spindle mast cells
41
Associations of 2ndy lymph HES?
Pruritus eczema erythroderma urticaria and angioedema Lymphadenopathy
42
Pathogenesis of 2ndry HES
T-cell clone elev IL-5
(Th2)
43
mention overlap synd of HES
Gleich syndr NERDS syndrome Eosinophilic gastroenteritis Eosinophilic vasculitis
44
labs with 2ndry HES ?
elevated IgE
 ± lymphoma

45
Primary immunodeficiencies As/w HES
Omenn syndrome hyper IgE syndrome
46
if patient Fits criteria (after criteria labs) for HES what is the next test?
Test for FIP1L1-PDGFRA (prim vs 2ndry)
47
labs to r/o parasite infection in HES
R/O Parasitic infection * anti-Strongyloides Ab * 3x stool samples for ova and parasites * IgE (>500 IU/ml ->helminth)
48
Rx for HES?
Pred 1mg/kg/d +/-ivermctin if Strongyloides exp 1ry: - tyrosine kinase inh -Jak inh -IFN 2ndry: -SCS -Mepolizumab -IFN
49
Angiolymphoid hyperplasia (ALH) with Eosinophilia histopath signs
hobnail’ endothelial cells (protrude into lumen) + eosin
50
Ddx for hobnail
Hobnail hemangioma, Dabska’s tumor, retiform hemangioendothelioma, multifocal lymphangioendotheliosis, angiosarcoma (well-differentiated)
51
ddx for ALH
cutaneous lymphoid hyperplasia, lymphoma cutis, sarcoidosis, angiosarcoma, metastatic tumors. Kimura’s disease (SEE TABLE below comparing)
52
cutaneous conditions As/w IgG4
Granuloma faciale Kimura ALH with eosin cutanous plasmocytosis
53
Systemic disease with IgG4
pancreatitis Autoimmune thyroid sclerosis cholangitis

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