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

< 5%
vasculitis - Diagnosis ... , causes and management ... vasculitis Clinical ... #Vasculitis #Rheumatology ... #Diagnosis #Management
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 ... hemopathies (4%) • Cryoglobulinemia ... #dermpath #dermatology ... #semiology #signs ... #clinical #manifestations
Cryofibrinogenemia Summary

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

Cryofibrinogenemia:
 • The precipitation of a
, sepsis or cryoglobulinemia ... Manifestations ... • Negative causes ... Calciphylaxis • Cryoglobulinemia ... hematology
Dermatologic Manifestations of Granulomatosis with Polyangiitis (GPA)

Skin involvement in granulomatosis with polyangiitis (GPA) is common and
Dermatologic Manifestations ... GPA causes disease ... Rarely dominate the clinical ... purpura • Mixed cryoglobulinemia ... Polyangiitis #GPA #dermatology
Vasculitis and Vasculitides - Differential Diagnosis Framework

When to Consider Vasculitis:
 • Purpura, ischemic skin lesions
 •
tract, and often causes ... ANCA +MPO) • Cryoglobulinemic ... purpura: strong sign ... Low levels mixed cryoglobulinemia ... Serologies for Cryoglobulins
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
Anti-NXP-2 Dermatomyositis

What?
DM with generalized subcutaneous edema
Pts have more myalgias, more severe weakness, and an increased prevalence
auricular papules Clinical ... Muscle atrophy may cause ... had more severe disease ... #diagnosis #management ... dermatology