Chapter 140 - Erythema Elevatum Diutinum

Question 1

Type of EED seen in young women with personal/family history of rheumatism

Answer 1

Bury type

Question 2

Type of EED is seen in older men with gout

Answer 2

Hutchinson type

Question 3

Chronic LCV typified by symmetric, erytheamtous, violaceous, or yellow brown papules, nodules, pr plaques

Answer 3

Erythema Elevatum Diutinum

Question 4

Sites of involvement include (3)

Answer 4

Skin overlying joints of hands or fingers
Extensor surfaces
Achilles tendon

Question 5

The trunk and mucosal membranes are usually spared in EED.

True or False

Answer 5

True

Question 6

Diseases associated with EED (5)

Answer 6

Monoclonal paraproteinemia
Lymphoproliferative disorders
Chronic infections
Autoimmune conditions
Connective tissue diseases

Question 7

Diutinum means

Answer 7

Long lasting

Question 8

EED presents in ____ to _____ decades

Answer 8

4th to 6th

Question 9

Involvement of retroauricular area or palmoplantar skin is common
True or False

Answer 9

False, uncommon

Question 10

Spontaneous resolution can transpire, usually ____ years of disease activity, while in other cases, fixed lesions last decades

Answer 10

5 to 10 years

Question 11

Hypothesized pathogenesis of EED

Answer 11

Formation of Ag-Ab complexes

Question 12

Chemotaxis of neutrophils

Answer 12

IL8

Question 13

Infections associated with EED (5)

Answer 13

Streptococcus
Hep B
Syphilis
Tb
HIV

Question 14

The most frequent association with EED was

Answer 14

IgA paraproteinemia

Question 15

Severity of EED was not dependent on total paraprotein levels.
True or False

Answer 15

True

Question 16

Diagnosis of EED is established by

Answer 16

Full thickness (of dermis) skin biopsy

Question 17

Central feature of disease process

Answer 17

Leukocytoclastic vasculitis

Question 18

Early EED often shows

Answer 18

Neutrophilic infiltrates and neutrophilic pyknotic debris

Question 19

DIF finding

Answer 19

Deposition of Ig G, M,A, C3 and fibrin in perivascular lesions

Question 20

There are multiple lab studies to confirm EED.

True or False

Answer 20

False, no

Question 21

Presence of red brown to violaceous papules and plaques with peau d’orange appearance

Answer 21

Granuloma faciale

Question 22

Matching type

1. LCV with concentric perivascular fibrosis
2. Eosinophils + admixed plasma cells
3. Histiocytes + granulomatous areas
4. Localized chronic fibrosing vasculitis

A. EED
B. GF

Answer 22

1. Both
2. B
3. A
4. Both

Question 23

First line agents

Answer 23

Sulfone- based therapies

1. Dapsone
2. Sulfapyridine

Question 24

Rapid improvement may transpire within first ___ hours with complete resolution of all lesions

Answer 24

48 hours

Question 25

MOA of sulfone based therapies

Answer 25

Inhibition of chemotaxis and function of neutrophils

Question 26

Medications associated with EED

Answer 26

Rifampin INH PZA Streptomycin

Question 27

HPx, reveals a __ in early stage; and ___ in late stage

Answer 27

LCV; | Mixed inflammation and fibrosis

Question 28

EED is uncommon on 4 areas

Answer 28

Face Ears Buttocks Genitals

Question 29

Lesions of EED become more __, __, and __ in the evening hours

Answer 29

Firm, raised, erythematous

Question 30

__ weather can cause new lesions or exacerbate symptoms

Answer 30

Cold

Question 31

Inflammatory disorders associated with EED

Answer 31

Crohn disease Ulcerative colitis Mixed cryoglobulinemia Pyoderma gangrenosum

Question 32

Palisaded interstitial granulomatous infiltrate without vasculitis; associated with DM

Answer 32

Disseminated granuloma Annulare

Question 33

Early DDx of EED

Answer 33

``` GA Sweet syndrome Rheumatoid neutrophilic dermatitis Palisade and granulomatous neutrophilic dermatoses Neutrophilic dermatoses of hands ```

Question 34

Histologically with cells of characteristic muddy rose or ground glass cytoplasm

Answer 34

Multicentric reticulohistiocytoma

Question 35

Goals of therapy (3)

Answer 35

Symptomatic relief Clearing of lesions Appropriate management

Question 36

Addition of __ in recalcitrant cases or if with dapsone-associated anemia

Answer 36

Prednisone

Question 37

Chronic fibrotic lesions have good prognosis | True or False

Answer 37

False, unlikely to respond to therapy

Question 38

Examples of ddx for chronic EED

Answer 38

Dermatofibroma Xanthoma Rheumatoid nodules Multicentric reticulohistiocytoma

Question 39

Serum protein electrophoresis has been advocated as one of lab tests for EED. True or False

Answer 39

False, immunofixation electrophoresis

Question 40

Tests to consider for patient with EED

Answer 40

``` CBC Comprehensive metabolic panel HIV screening Immunofixation electrophoresis Streptozyme test Hep B and C panel ANA ANCA APAS UA CXR ```