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Diagnosis and Management of Hypocomplementemic Urticarial Vasculitis (HUV)

Diagnosis of HUV
1) Chronic urticaria 
2) Leukocytoclastic vasculitis 
3)
Management of Hypocomplementemic ... Urticarial Vasculitis ... Leukocytoclastic vasculitis ... 3) Hypocomplementemia ... #Urticarial #Vasculitis
Pathophysiology of Hypocomplementemic Urticarial Vasculitis

C1q/anti-C1q antibiody ->
Immune Complex Formation - Complement Pathway Activation (C3a and C5a)
Pathophysiology of Hypocomplementemic ... Urticarial Vasculitis ... Formation -> Vasculitis ... TerrierBen #Hypocomplementemic ... #Urticarial #Vasculitis
Vasculitis and Vasculitides - Differential Diagnosis Framework

When to Consider Vasculitis:
 • Purpura, ischemic skin lesions
 •
Vasculitis and Vasculitides ... When to Consider Vasculitis ... SMALL VESSEL VASCULITIS ... often involved • Hypocomplementemic ... Urticaria and hypocomplementemia
Vasculitis - Differential Diagnosis Framework

When to Consider Vasculitis:
• Purpura, ischemic skin lesions
• Mononeuritis multiplex
• Hematuria, proteinuria,
Vasculitis - Differential ... When to Consider Vasculitis ... abnormalities Vasculitis ... Elevated AST ALT • Hypocomplementemia ... ) • Hypocomplementemic
IgG4-Related Disease
Clinical history:  Man, 60 years, multiorgan involvement, unique or multiple inflammatory pseudotumor
4 phenotypes:
 •
rheumatoid factor), ± hypocomplementemia ... No necrosis, No vasculitis
Sjogren's Syndrome Overview

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

Autoimmune exocrinopathy multisystemic disease
Cytopenia: 2% • Hypocomplementemia ... ) • Cutaneous vasculitis ... : 2% Systemic Vasculitis
Small Vessel Vasculitides
 • Granulomatosis with polyangiitis - Granulomatous manifestations (e.g., nasal crusting, destructive sinusitis, pulmonary
Small Vessel Vasculitides ... Cryoglobulinemic vasculitis ... syndrome • IgA vasculitis ... Small #Vessel #Vasculitides ... #vasculitis #differential
A System for Vasculitides 

#Diagnosis #IM #Rheum #Vasculitis #Small #Medium #Large #Vessel #Size #Classification #ANCA #Differential
A System for Vasculitides ... Diagnosis #IM #Rheum #Vasculitis
Vasculitis: In this instance, the vasculitic skin lesions were secondary to Haemophilus endocarditis.
Photo credit, Josh Fierer,
Vasculitis: In this ... instance, the vasculitic ... Dermatology #SkinRash #Vasculitis
Vasculitis - Break down based on vessel size 
Distribution of vessel involvement by large vessel vasculitis,
Vasculitis - Break ... by large vessel vasculitis ... medium vessel vasculitis ... and small vessel vasculitis ... Large vessel vasculitis