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(Ch25) Eosinophilic dermatoses Flashcards

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

A

deck-chair sign

23
Q

deck-chair sign ddx

A

Papuloerythroderma of Ofuji

Waldenstrom’s Macroglobulinemia

24
Q

Which ethnic group at higher risk for Papuloerythroderma of Ofuji

A

2/3 – elderly Japanese men (avg 72yo)

25
Q

Papuloerythroderma of Ofuji histopath

A

Eosinophilia
Lymphopenia

26
Q

Papuloerythroderma of Ofuji Rx

A

PUVA +/- acitretin  2/3 cleared + ¼ improved
UVB, cyclosporine, etretinate and azathiopurine

27
Q

which Tx is ineffective for Papuloerythroderma of Ofuji

A

TCS

28
Q

Well’s Syndrome other name

A

eosinophilic cellulitis

29
Q

Well’s synd Triggers

A

insect bites drugs, allergic contact dermatitis, malignancy, and infections

30
Q

which Histopth sign is characteristic but not diagnostic nor pathognmenic for Well’s

A

flame figures

31
Q

What are flame Figures

A

eosinophil degranulation onto collagen

32
Q

Which part of skin is normal in histopth of Well’s

A

Epidermis

33
Q

Ddx for Flame figures

A

FLAMEED
Fungus/dermatophyte infections

Lung (bronchiogenic) carcinoma

Arthropod bites/stings, scabies

Mastocytomas, bullous pemphigoid

Eczema, HES, prurigo nodularis

Drug

34
Q

Rx of Well’s

A

Steroids
Cyclosporine
Colchicine
Dapsone
Mepolizumab

35
Q

Hypereosinophilic Syndrome types

A
  1. Myeloproliferative( primary/neoplastic)
  2. Lymphoproliferative (2ndry)
  3. Overlap
36
Q

Hypereosinophilic Syndrome diagnostic criteria

A

1.Evidence of Eosinophilia
-peripheral >1500(x2 1m)
and/or
- BM bx >20% eosin
and/or
-Path extensive eosin

  1. End organ damage/dysfunction due to eosin
  2. exclusion of 2ndry causes
37
Q

Myeloproliferative HES mutation and chromosome

A

FIP1L1-PDGFRA (chrom 4)

38
Q

FIP1L1-PDGFRA results in?

A

activated tyrosine kinase

39
Q

mention the 4Ms associations of Myeloproliferative HES

A

Male predominance
Mastocytosis
endoMyocardial dis
Mucosal inovelevment

40
Q

what other findings in Myeloproliferative HES

A

inc tryptase and Vit B12,
tissue fibrosis, splenomegaly, and bone marrow biopsies with  CD25+ atypical spindle mast cells

41
Q

Associations of 2ndy lymph HES?

A

Pruritus
eczema
erythroderma
urticaria and angioedema
Lymphadenopathy

42
Q

Pathogenesis of 2ndry HES

A

T-cell clone elev IL-5
(Th2)

43
Q

mention overlap synd of HES

A

Gleich syndr
NERDS syndrome
Eosinophilic gastroenteritis
Eosinophilic vasculitis

44
Q

labs with 2ndry HES ?

A

elevated IgE

± lymphoma


45
Q

Primary immunodeficiencies As/w HES

A

Omenn syndrome
hyper IgE syndrome

46
Q

if patient Fits criteria (after criteria labs) for HES what is the next test?

A

Test for FIP1L1-PDGFRA (prim vs 2ndry)

47
Q

labs to r/o parasite infection in HES

A

R/O Parasitic infection
* anti-Strongyloides Ab
* 3x stool samples for ova and parasites
* IgE (>500 IU/ml ->helminth)

48
Q

Rx for HES?

A

Pred 1mg/kg/d +/-ivermctin if Strongyloides exp

1ry:
- tyrosine kinase inh
-Jak inh
-IFN

2ndry:
-SCS
-Mepolizumab
-IFN

49
Q

Angiolymphoid hyperplasia (ALH) with Eosinophilia histopath signs

A

hobnail’ endothelial cells (protrude into lumen) + eosin

50
Q

Ddx for hobnail

A

Hobnail hemangioma, Dabska’s tumor, retiform hemangioendothelioma, multifocal lymphangioendotheliosis, angiosarcoma (well-differentiated)

51
Q

ddx for ALH

A

cutaneous lymphoid hyperplasia, lymphoma cutis, sarcoidosis, angiosarcoma, metastatic tumors. Kimura’s disease (SEE TABLE below comparing)

52
Q

cutaneous conditions As/w IgG4

A

Granuloma faciale
Kimura
ALH with eosin
cutanous plasmocytosis

53
Q

Systemic disease with IgG4

A

pancreatitis Autoimmune
thyroid
sclerosis cholangitis

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