Chapter 140 - Erythema Elevatum Diutinum Flashcards
Type of EED seen in young women with personal/family history of rheumatism
Bury type
Type of EED is seen in older men with gout
Hutchinson type
Chronic LCV typified by symmetric, erytheamtous, violaceous, or yellow brown papules, nodules, pr plaques
Erythema Elevatum Diutinum
Sites of involvement include (3)
Skin overlying joints of hands or fingers
Extensor surfaces
Achilles tendon
The trunk and mucosal membranes are usually spared in EED.
True or False
True
Diseases associated with EED (5)
Monoclonal paraproteinemia Lymphoproliferative disorders Chronic infections Autoimmune conditions Connective tissue diseases
Diutinum means
Long lasting
EED presents in ____ to _____ decades
4th to 6th
Involvement of retroauricular area or palmoplantar skin is common
True or False
False, uncommon
Spontaneous resolution can transpire, usually ____ years of disease activity, while in other cases, fixed lesions last decades
5 to 10 years
Hypothesized pathogenesis of EED
Formation of Ag-Ab complexes
Chemotaxis of neutrophils
IL8
Infections associated with EED (5)
Streptococcus Hep B Syphilis Tb HIV
The most frequent association with EED was
IgA paraproteinemia
Severity of EED was not dependent on total paraprotein levels.
True or False
True
Diagnosis of EED is established by
Full thickness (of dermis) skin biopsy
Central feature of disease process
Leukocytoclastic vasculitis
Early EED often shows
Neutrophilic infiltrates and neutrophilic pyknotic debris
DIF finding
Deposition of Ig G, M,A, C3 and fibrin in perivascular lesions
There are multiple lab studies to confirm EED.
True or False
False, no
Presence of red brown to violaceous papules and plaques with peau d’orange appearance
Granuloma faciale
Matching type
- LCV with concentric perivascular fibrosis
- Eosinophils + admixed plasma cells
- Histiocytes + granulomatous areas
- Localized chronic fibrosing vasculitis
A. EED
B. GF
- Both
- B
- A
- Both
First line agents
Sulfone- based therapies
- Dapsone
- Sulfapyridine
Rapid improvement may transpire within first ___ hours with complete resolution of all lesions
48 hours
MOA of sulfone based therapies
Inhibition of chemotaxis and function of neutrophils
Medications associated with EED
Rifampin
INH
PZA
Streptomycin
HPx, reveals a __ in early stage; and ___ in late stage
LCV;
Mixed inflammation and fibrosis
EED is uncommon on 4 areas
Face
Ears
Buttocks
Genitals
Lesions of EED become more __, __, and __ in the evening hours
Firm, raised, erythematous
__ weather can cause new lesions or exacerbate symptoms
Cold
Inflammatory disorders associated with EED
Crohn disease
Ulcerative colitis
Mixed cryoglobulinemia
Pyoderma gangrenosum
Palisaded interstitial granulomatous infiltrate without vasculitis; associated with DM
Disseminated granuloma Annulare
Early DDx of EED
GA Sweet syndrome Rheumatoid neutrophilic dermatitis Palisade and granulomatous neutrophilic dermatoses Neutrophilic dermatoses of hands
Histologically with cells of characteristic muddy rose or ground glass cytoplasm
Multicentric reticulohistiocytoma
Goals of therapy (3)
Symptomatic relief
Clearing of lesions
Appropriate management
Addition of __ in recalcitrant cases or if with dapsone-associated anemia
Prednisone
Chronic fibrotic lesions have good prognosis
True or False
False, unlikely to respond to therapy
Examples of ddx for chronic EED
Dermatofibroma
Xanthoma
Rheumatoid nodules
Multicentric reticulohistiocytoma
Serum protein electrophoresis has been advocated as one of lab tests for EED.
True or False
False, immunofixation electrophoresis
Tests to consider for patient with EED
CBC Comprehensive metabolic panel HIV screening Immunofixation electrophoresis Streptozyme test Hep B and C panel ANA ANCA APAS UA CXR
