Chapter 140 - Erythema Elevatum Diutinum Flashcards

1
Q

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

A

Bury type

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2
Q

Type of EED is seen in older men with gout

A

Hutchinson type

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3
Q

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

A

Erythema Elevatum Diutinum

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4
Q

Sites of involvement include (3)

A

Skin overlying joints of hands or fingers
Extensor surfaces
Achilles tendon

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5
Q

The trunk and mucosal membranes are usually spared in EED.

True or False

A

True

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6
Q

Diseases associated with EED (5)

A
Monoclonal paraproteinemia
Lymphoproliferative disorders
Chronic infections
Autoimmune conditions
Connective tissue diseases
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7
Q

Diutinum means

A

Long lasting

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8
Q

EED presents in ____ to _____ decades

A

4th to 6th

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9
Q

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

A

False, uncommon

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10
Q

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

A

5 to 10 years

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11
Q

Hypothesized pathogenesis of EED

A

Formation of Ag-Ab complexes

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12
Q

Chemotaxis of neutrophils

A

IL8

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13
Q

Infections associated with EED (5)

A
Streptococcus
Hep B
Syphilis
Tb
HIV
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14
Q

The most frequent association with EED was

A

IgA paraproteinemia

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15
Q

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

A

True

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16
Q

Diagnosis of EED is established by

A

Full thickness (of dermis) skin biopsy

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17
Q

Central feature of disease process

A

Leukocytoclastic vasculitis

18
Q

Early EED often shows

A

Neutrophilic infiltrates and neutrophilic pyknotic debris

19
Q

DIF finding

A

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

20
Q

There are multiple lab studies to confirm EED.

True or False

A

False, no

21
Q

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

A

Granuloma faciale

22
Q

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

A
  1. Both
  2. B
  3. A
  4. Both
23
Q

First line agents

A

Sulfone- based therapies

  1. Dapsone
  2. Sulfapyridine
24
Q

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

A

48 hours

25
Q

MOA of sulfone based therapies

A

Inhibition of chemotaxis and function of neutrophils

26
Q

Medications associated with EED

A

Rifampin
INH
PZA
Streptomycin

27
Q

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

A

LCV;

Mixed inflammation and fibrosis

28
Q

EED is uncommon on 4 areas

A

Face
Ears
Buttocks
Genitals

29
Q

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

A

Firm, raised, erythematous

30
Q

__ weather can cause new lesions or exacerbate symptoms

A

Cold

31
Q

Inflammatory disorders associated with EED

A

Crohn disease
Ulcerative colitis
Mixed cryoglobulinemia
Pyoderma gangrenosum

32
Q

Palisaded interstitial granulomatous infiltrate without vasculitis; associated with DM

A

Disseminated granuloma Annulare

33
Q

Early DDx of EED

A
GA
Sweet syndrome
Rheumatoid neutrophilic dermatitis 
Palisade and granulomatous neutrophilic dermatoses 
Neutrophilic dermatoses of hands
34
Q

Histologically with cells of characteristic muddy rose or ground glass cytoplasm

A

Multicentric reticulohistiocytoma

35
Q

Goals of therapy (3)

A

Symptomatic relief
Clearing of lesions
Appropriate management

36
Q

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

A

Prednisone

37
Q

Chronic fibrotic lesions have good prognosis

True or False

A

False, unlikely to respond to therapy

38
Q

Examples of ddx for chronic EED

A

Dermatofibroma
Xanthoma
Rheumatoid nodules
Multicentric reticulohistiocytoma

39
Q

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

A

False, immunofixation electrophoresis

40
Q

Tests to consider for patient with EED

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