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Behçet's Syndrome
Systemic disease associated with inflammation of multiple organs, small-vessel vasculitis and large-vessel vasculopathy
Epidemiology:
 • Young
, small-vessel vasculitis ... ulceration • Ocular ... meningitis), MCC CNS symptoms ... inhibitors • Uveitis- Ophthalmology ... management #signs #symptoms
Vasculitis and Vasculitides - Differential Diagnosis Framework

When to Consider Vasculitis:
 • Purpura, ischemic skin lesions
 •
, and ocular inflammation ... ulcers, cutaneous, ocular ... Syndrome: - Ocular ... to exclude are infections ... Presentation - Systemic Symptoms
Vasculitis Involving the Skin - Differential Diagnosis Framework

Primary: 50% Cutaneous Vasculitis: Leukocytoclastic Vasculitis
Secondary: CTD: SLE, RA
, infections, drug ... IgM deposits • Nodular ... : • Temporal arteritis ... • Takayasu's arteritis ... : • Behcet Disease
Vasculitis - Differential Diagnosis Framework

When to Consider Vasculitis:
• Purpura, ischemic skin lesions
• Mononeuritis multiplex
• Hematuria, proteinuria,
to exclude are infections ... Presentation: Systemic Symptoms ... • Progressive symptoms ... , recent viral infection ... Cogan syndrome: Ocular
Highlighted Conditions With Arteritis or Periarteritis of the Coronaries
Takayasu's arteritis:
 - Onset < 40 yrs
 -
yrs - Cranial symptoms ... Polyarteritis nodosa: - Skin ... multiplex ANCA vasculitis ... Leukocytoclastic vasculitis ... Ulceration GI tract - Ocular
Skin Conditions Associated with Joint Pain

Rash
	• Human parvovirus B19 infection

Malar rash
	• SLE
	• Human parvovirus B19 infection
	•
parvovirus B19 infection ... parvovirus B19 infection ... • Lyme disease ... Hypersensitivity vasculitis ... Antiphospholipid-antibody syndrome • Vasculitis
Extra-articular and Other Conditions Associated with Joint Pain

Eyes
  • Iritis or uveitis - Spondyloarthropathies, Sarcoidosis,
Bacterial or viral infection ... • Still's disease ... , Vasculitis (e.g ... Scleroderma Thickened skin ... Cardiomyopathies - Viral infection
Dermatologic Manifestations of Granulomatosis with Polyangiitis (GPA)

Skin involvement in granulomatosis with polyangiitis (GPA) is common and
• Pauci-immune vasculitis ... • Giant cell arteritis ... Infections include ... • Fungal infections ... Granulomatous vasculitis
Behcet's Syndrome - Diagnosis
Diagnostic Criteria for Behget's Syndrome (International Study Group for Behget's Disease):
 • Recurrent,
or retinal vasculitis ... - Skin lesions ... Wide variety of skin ... , retinal vasculitis ... ) • Arthritis (
Cryoglobulinemia Summary

Cryoglobulins:
 • Cryoglobulins are Igs in the serum that precipitate in vitro at temperatures below
- Purpura - Skin ... • Autoimmune diseases ... reticularis & skin ... arthralgia, and arthritis ... SLE), rheumatoid arthritis