(Ch25) Eosinophilic dermatoses Flashcards
DDx EOSINOPHILIC SPONGIOSIS ?
HAAAPPPIED
Herpes gestationis
Arthropod bite
ACD
AD
Pemphigus
Pemphigoid
Polymorphous eruption of pregnancy (PEP)
Incontinentia pigmenti (stage 1 and 2)
Erythema toxicum
Drug
GROWTH FACTORS for Eosin
- IL-3
- GM-CSF
- IL-5
Most potent growth factor for Eosin
GM-CSF
Most Selective growth factor for Eosin
IL-5: JAK2/STAT5 pathway
Factors chemoattract/recurit Eosin to tissue
- Platelet activaing factor (PAF)
- CCL11 (eotaxin),
- CCL24 (eotaxin 2),
- CCL26 (eotaxin3)
Most potent eosin chemoattractant
Platelet activaing factor (PAF)
Release of Eosin granules in 3 ways mention them?
- cytolytic degranulation - organelle rupture,
- Piecemeal degranulation
- Exocytosis
Eosin Primary Granules
Charcot Leyden crystal (Galactin-10)
Eosin 2˚/Secretory Granules 5 components
Major basic protein 1
Major basic protein 2
Eosinophil derived neurotoxin
Eosinophil catonic protein
Eosinophil peroxidase (most numerous)
Eosin Small granules
Acid phosphatase
Arylsulfatase
Eosin Secretory vesicles
Albumin
Eosin Non-membrane bound lipid bodies
Arachidonic acid
Cyclooxygenease
5&15 lipooxygenase
LTC4
Ddx for PSEUDOCELLULITIS
- Infections and bites
- Arthropod bite reactions (e.g. insect, spider)
-Erythema migrans
-Herpes zoster
-Toxin-mediated erythema (e.g. recurrent toxin-mediated perineal erythema
- Neutrophilic dermatoses
- Sweet’s syndrome
- Neutrophilic panniculitis (Pancreatic, Facticial)
- Familial Mediterranean fever
- Arthropod bite reactions (e.g. insect, spider)
- Drug reactions
- Fixed drug eruptions (esp Non-Pigmenting Adverse rx: NSAIDs, Pseudoepherine, Acetaminophen)
- Vaccine/injection site reactions
- Toxic erythema of chemotherapy
- Metabolic disorders
Gout - Malignancy
Erysipeloid skin metastases
ddx for persistent red facial plaque with epidermal changes
DLE
Subacute SLE
Tinea
SD
Perioral dermatitis
BCC
SCC
Leprosy
ddx for persistent red facial plaque without epidermal changes
LLLLLL
Lymphoma
Leukemia cutis
Lupus tumidus
Polymorphous Light Eruption
Lyme
syphilis
Granuloma faciale
Granuloma faciale clinical appearance
Prominent follicular openings
2/3 solitary
Granuloma faciale variant with Sinonasal mucosal involvement
esoinophilic angiocentric fibrosis
Typical histopath of Granuloma faciale
Grenz zone
+ LCV, Eosin, neut
Grenz zone ddx
lymphoma/leukemia
Granuloma faciale
Erythema elevatum diutinum
Rx for Granuloma faciale
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
which patients are at higher risk/ As/w Exaggerated Insect Bite and Insect Bite-Like reactions
CLL > other myeloproliferative disorders
EBV associated NK/T-cell lymphoma, nasal type
Papuloerythroderma of Ofuji clinical sign
deck-chair sign
deck-chair sign ddx
Papuloerythroderma of Ofuji
Waldenstrom’s Macroglobulinemia
Which ethnic group at higher risk for Papuloerythroderma of Ofuji
2/3 – elderly Japanese men (avg 72yo)
Papuloerythroderma of Ofuji histopath
Eosinophilia
Lymphopenia
Papuloerythroderma of Ofuji Rx
PUVA +/- acitretin 2/3 cleared + ¼ improved
UVB, cyclosporine, etretinate and azathiopurine
which Tx is ineffective for Papuloerythroderma of Ofuji
TCS
Well’s Syndrome other name
eosinophilic cellulitis
Well’s synd Triggers
insect bites drugs, allergic contact dermatitis, malignancy, and infections
which Histopth sign is characteristic but not diagnostic nor pathognmenic for Well’s
flame figures
What are flame Figures
eosinophil degranulation onto collagen
Which part of skin is normal in histopth of Well’s
Epidermis
Ddx for Flame figures
FLAMEED
Fungus/dermatophyte infections
Lung (bronchiogenic) carcinoma
Arthropod bites/stings, scabies
Mastocytomas, bullous pemphigoid
Eczema, HES, prurigo nodularis
Drug
Rx of Well’s
Steroids
Cyclosporine
Colchicine
Dapsone
Mepolizumab
Hypereosinophilic Syndrome types
- Myeloproliferative( primary/neoplastic)
- Lymphoproliferative (2ndry)
- Overlap
Hypereosinophilic Syndrome diagnostic criteria
1.Evidence of Eosinophilia
-peripheral >1500(x2 1m)
and/or
- BM bx >20% eosin
and/or
-Path extensive eosin
- End organ damage/dysfunction due to eosin
- exclusion of 2ndry causes
Myeloproliferative HES mutation and chromosome
FIP1L1-PDGFRA (chrom 4)
FIP1L1-PDGFRA results in?
activated tyrosine kinase
mention the 4Ms associations of Myeloproliferative HES
Male predominance
Mastocytosis
endoMyocardial dis
Mucosal inovelevment
what other findings in Myeloproliferative HES
inc tryptase and Vit B12,
tissue fibrosis, splenomegaly, and bone marrow biopsies with CD25+ atypical spindle mast cells
Associations of 2ndy lymph HES?
Pruritus
eczema
erythroderma
urticaria and angioedema
Lymphadenopathy
Pathogenesis of 2ndry HES
T-cell clone elev IL-5 (Th2)
mention overlap synd of HES
Gleich syndr
NERDS syndrome
Eosinophilic gastroenteritis
Eosinophilic vasculitis
labs with 2ndry HES ?
elevated IgE
± lymphoma
Primary immunodeficiencies As/w HES
Omenn syndrome
hyper IgE syndrome
if patient Fits criteria (after criteria labs) for HES what is the next test?
Test for FIP1L1-PDGFRA (prim vs 2ndry)
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)
Rx for HES?
Pred 1mg/kg/d +/-ivermctin if Strongyloides exp
1ry:
- tyrosine kinase inh
-Jak inh
-IFN
2ndry:
-SCS
-Mepolizumab
-IFN
Angiolymphoid hyperplasia (ALH) with Eosinophilia histopath signs
hobnail’ endothelial cells (protrude into lumen) + eosin
Ddx for hobnail
Hobnail hemangioma, Dabska’s tumor, retiform hemangioendothelioma, multifocal lymphangioendotheliosis, angiosarcoma (well-differentiated)
ddx for ALH
cutaneous lymphoid hyperplasia, lymphoma cutis, sarcoidosis, angiosarcoma, metastatic tumors. Kimura’s disease (SEE TABLE below comparing)
cutaneous conditions As/w IgG4
Granuloma faciale
Kimura
ALH with eosin
cutanous plasmocytosis
Systemic disease with IgG4
pancreatitis Autoimmune
thyroid
sclerosis cholangitis
