Dermatology
PREDOMINANTLY SMALL AND MEDIUM-SIZED VESSEL VASCULITIDES
Built from Dermatology, 5th Edition

What’s inside
11 sections · 142 slides
Overview
- Scope of this topic
- How this topic is organised
Background: CSVV and IgA Vasculitis
Two baseline small-vessel entities that every disease in this topic is compared against
- Two entities used as the yardstick
- Cutaneous small vessel vasculitis
- Digital infarcts in cutaneous small vessel vasculitis
- Vesiculobullous variant of cutaneous small vessel vasculitis
- IgA vasculitis (Henoch-Schonlein purpura)
- Ulcerations in IgA vasculitis (Henoch-Schonlein purpura)
- IgA vasculitis with renal involvement in an adult
- IgA vasculitis lesions in an adult
- Hemorrhagic Sweet syndrome mimicking vasculitis
Acute Hemorrhagic Edema of Infancy
A dramatic-looking but benign small-vessel vasculitis of infants and toddlers
- What is acute hemorrhagic edema of infancy
- Epidemiology of acute hemorrhagic edema of infancy
- Pathogenesis of acute hemorrhagic edema of infancy
- Triggers in acute hemorrhagic edema of infancy
- Triggers in acute hemorrhagic edema of infancy
- Clinical features of AHEI: the rash
- What the rash of AHEI looks like
- Acute hemorrhagic edema of infancy: thigh and sole lesions
- Clinical course of acute hemorrhagic edema of infancy
- Pathology of acute hemorrhagic edema of infancy
- Differential diagnosis of acute hemorrhagic edema of infancy
- Treatment of acute hemorrhagic edema of infancy
Urticarial Vasculitis
Hive-like lesions that are actually true blood vessel inflammation
- What is urticarial vasculitis
- Epidemiology of urticarial vasculitis
- How urticarial vasculitis develops
- Conditions associated with urticarial vasculitis
- Conditions associated with urticarial vasculitis
- Skin findings in urticarial vasculitis
- What urticarial vasculitis looks like
- Hypocomplementemic urticarial vasculitis syndrome (HUVS)
- Organ involvement in urticarial vasculitis and HUVS
- Laboratory findings in urticarial vasculitis
- Pathology of urticarial vasculitis
- Differential diagnosis of urticarial vasculitis
- Treatment of urticarial vasculitis
Erythema Elevatum Diutinum
A rare, long-lasting small-vessel vasculitis that scars into fibrous nodules over joints
- What is erythema elevatum diutinum
- Epidemiology of erythema elevatum diutinum
- How erythema elevatum diutinum develops
- Conditions associated with erythema elevatum diutinum
- Clinical features of erythema elevatum diutinum
- What EED lesions look like
- Course and eye disease in erythema elevatum diutinum
- Erythema elevatum diutinum: histopathologic features
- Differential diagnosis of erythema elevatum diutinum
- Treatment of erythema elevatum diutinum
Granuloma Faciale
A chronic, usually solitary, facial small-vessel vasculitis
- What is granuloma faciale
- Epidemiology and pathogenesis of granuloma faciale
- Clinical features of granuloma faciale
- What granuloma faciale looks like
- Pathology of granuloma faciale
- Granuloma faciale: histopathologic features
- Differential diagnosis of granuloma faciale
- Treatment of granuloma faciale
Cryoglobulinemic Vasculitis
A mixed small- and medium-vessel vasculitis driven, in most cases, by hepatitis C infection
- What are cryoglobulins
- Classification of cryoglobulins
- Classification of cryoglobulins: I
- Classification of cryoglobulins: II / III
- Epidemiology of cryoglobulinemic vasculitis
- How cryoglobulinemic vasculitis develops
- Skin and organ findings in cryoglobulinemic vasculitis
- What cryoglobulinemic vasculitis looks like
- Laboratory testing in cryoglobulinemic vasculitis
- Cutaneous vasculitis due to mixed cryoglobulinemia
- Pathology and differential diagnosis of cryoglobulinemic vasculitis
- Treatment of cryoglobulinemic vasculitis
ANCA-Associated Vasculitides
Three overlapping small-to-medium vessel vasculitides defined by anti-neutrophil cytoplasmic antibodies
- What are the ANCA-associated vasculitides
- The two ANCA staining patterns
- Comparing GPA, MPA, and EGPA
- ANCA-associated vasculitides: Granulomatosis with polyangiitis (GPA; formerly Wegener granulomatosis) - most often c-ANCA/anti-PR3 antibody
- ANCA-associated vasculitides: Microscopic polyangiitis (MPA) - most often p-ANCA/anti-MPO antibody
- ANCA-associated vasculitides: Eosinophilic granulomatosis with polyangiitis (EGPA; formerly Churg-Strauss syndrome) - most often ANCA-negative or p-ANCA/anti-MPO antibody
- Epidemiology and mechanism of the ANCA-associated vasculitides
- What is granulomatosis with polyangiitis
- Epidemiology and pathogenesis of granulomatosis with polyangiitis
- Skin and mucosal findings in granulomatosis with polyangiitis
- What granulomatosis with polyangiitis looks like
- Airway and lung findings in granulomatosis with polyangiitis
- Kidney and other organ findings in granulomatosis with polyangiitis
- Additional testing for suspected ANCA-associated vasculitis
- Additional diagnostic testing for suspected ANCA-associated vasculitis
- Leg ulcer in granulomatosis with polyangiitis
- Churg-Strauss nodules of the elbow in granulomatosis with polyangiitis
- Pathology of granulomatosis with polyangiitis
- Distinguishing GPA from its differential diagnosis
- Key disorders in the differential diagnosis of ANCA-associated vasculitis
- Key disorders in the differential diagnosis of ANCA-associated vasculitis (continued)
- Treatment of granulomatosis with polyangiitis: induction
- Treatment of granulomatosis with polyangiitis: maintenance
- What is microscopic polyangiitis
- Epidemiology and skin findings in microscopic polyangiitis
- What microscopic polyangiitis looks like
- Organ disease, pathology, and treatment of microscopic polyangiitis
- What is eosinophilic granulomatosis with polyangiitis
- Epidemiology of eosinophilic granulomatosis with polyangiitis
- How eosinophilic granulomatosis with polyangiitis develops
- The three phases of eosinophilic granulomatosis with polyangiitis
- Skin and organ findings in eosinophilic granulomatosis with polyangiitis
- What eosinophilic granulomatosis with polyangiitis looks like
- Labs, pathology, and differential diagnosis of EGPA
- Treatment of eosinophilic granulomatosis with polyangiitis
Polyarteritis Nodosa
A multi-system vasculitis of predominantly medium-sized arteries, with a milder skin-limited form
- What is polyarteritis nodosa
- Epidemiology of polyarteritis nodosa
- How polyarteritis nodosa develops
- Clinical features of systemic (classic) polyarteritis nodosa
- Skin findings in systemic polyarteritis nodosa
- What polyarteritis nodosa looks like
- Cutaneous polyarteritis nodosa
- Laboratory and imaging findings in polyarteritis nodosa
- Cutaneous polyarteritis nodosa: histopathologic features
- Differential diagnosis of polyarteritis nodosa
- Lymphocytic thrombophilic arteritis, a PAN look-alike
- Treatment of polyarteritis nodosa
Giant Cell (Temporal) Arteritis
A large-vessel vasculitis that occasionally reaches the skin of the scalp and tongue
- Temporal arteritis and the skin
- Temporal arteritis
- Diagnosis and treatment of temporal arteritis
Diagnostic Approach to Suspected Vasculitis
Correlating the clinical picture with pathology, screening for systemic disease, and matching treatment to severity
- Approach to a patient with suspected vasculitis
- Physical examination in suspected vasculitis
- Morphologic approach to suspected cutaneous vasculitis
- Skin biopsy in suspected vasculitis
- Laboratory evaluation of suspected vasculitis
- Approach to suspected cutaneous small vessel vasculitis
- Principles of vasculitis treatment
- Summary: telling these vasculitides apart
- Key take-home points
- References
- References (continued)
- References (continued)
- References (continued)
- References (continued)
- References (continued)
- References (continued)
- References (continued)
- References (continued)
- References (continued)
- References (continued)
- References (continued)
- References (continued)
- References (continued)
- References (continued)
- References (continued)
- Dermatology, 5th Edition (2-Volume Set)