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Non-HCV infectious mixed cryoglobulinemia vasculitis - Diagnosis, causes and management - French Vasculitis Study Group

< 5%
infectious mixed cryoglobulinemia ... , causes and management ... < 5% of mixed cryoglobulinemia ... vasculitis Clinical ... #Vasculitis #Rheumatology
Cryoglobulinemia Summary

Cryoglobulins:
 • Cryoglobulins are Igs in the serum that precipitate in vitro at temperatures below
Cryoglobulinemia ... , in Mixed Cryoglobulinemia ... Syndrome: • Clinical ... • Autoimmune diseases ... ) #Cryoglobulinemia
Dermatological semiology of Cutaneous Vasculitis 

Clinicopathologic correlations
 • Purpura, papules, pustules, necrosis: involvement of small vessels
Identification of causes ... Connective tissue disease ... hemopathies (4%) • Cryoglobulinemia ... #semiology #signs ... lesions #diagnosis #clinical
Purpura - Differential Diagnosis Framework

Non-blanchable, dark red to purple, hemorrhagic skin lesions that result from leakage
- Bone marrow disease ... Kasabach-Merritt syndrome ... Gardner-Diamond syndrome ... /HSP • Cryoglobulinemia ... Differential #Diagnosis #hematology
Vasculitis and Vasculitides - Differential Diagnosis Framework

When to Consider Vasculitis:
 • Purpura, ischemic skin lesions
 •
tract, and often causes ... ANCA +MPO) • Cryoglobulinemic ... • Cogan Syndrome ... purpura: strong sign ... Low levels mixed cryoglobulinemia
Dermatologic Manifestations of Granulomatosis with Polyangiitis (GPA)

Skin involvement in granulomatosis with polyangiitis (GPA) is common and
GPA causes disease ... Rarely dominate the clinical ... purpura • Mixed cryoglobulinemia ... • Goodpasture syndrome ... rash #diagnosis #rheumatology
Cryofibrinogenemia Summary

Cryofibrinogenemia Epidemiology:
 • 40-70 years with a modest female predominance

Cryofibrinogenemia:
 • The precipitation of a
malignancies, vasculitis ... , sepsis or cryoglobulinemia ... cryoglobulins • Negative causes ... Calciphylaxis • Cryoglobulinemia ... Antiphospholipid syndrome
Vasculitis - Differential Diagnosis Framework

When to Consider Vasculitis:
• Purpura, ischemic skin lesions
• Mononeuritis multiplex
• Hematuria, proteinuria,
purpura: Strong sign ... tract, and often causes ... / ANCA + MPO) Cryoglobulinemic ... • Behcet syndrome ... • Cogan syndrome
Sjogren's Syndrome Overview

Epidemiology:
• F > M: 9:1
• 5-6th Decades (can be any age)

Autoimmune exocrinopathy multisystemic disease
Hypocomplementemia • Can cause ... salivary ducts Clinical ... Nephrogenic DI Hematologic ... : • 7%, Cryoglobulinemia ... Diagnosis #Management
Behçet's Syndrome
Systemic disease associated with inflammation of multiple organs, small-vessel vasculitis and large-vessel vasculopathy
Epidemiology:
 • Young
association • M > F Clinical ... (Clinical Dx). ... #diagnosis #management ... #signs #symptoms ... #rheumatology #