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VASCULITIDES &
VASO-OCCLUSIVE
DISORDERS
Broward Health Medical Center
Dermatology Residency Program
Program Director: Carlos Nousari, M.D.
Trevor Batty, D.O., Sasha Chediak, D.O., Jennifer
Conde, D.O., Lisa Diaz, D.O., June Kunaparredy,
D.O., Brandon Nickle, D.O., Pamela Sheridan, D.O.,
Brittany Smirnov, D.O., Miguel Villacorta, D.O.
Disclosure
• We have no financial interests or
relationships to disclose.
CUTANEOUS SMALL-VESSEL VASCULITIS
A. Hypersensitivity Vasculitis/Leukocytoclastic
Vasculitis
B. Henoch-Schonlein Purpura
C. Acute Hemorrhagic Edema of Infancy
D. Urticarial Vasculitis
E. Cryoglobulinemic Vasculitis
F. Erythema Elevatum Diutinum
• All ages & sexes, MC in adults• Etiology: immune complex
deposition• Presentation: palpable purpura,
erythematous papules, urticarial lesions involving dependent areas– Koebner phenomenon– Fever, weight loss, myalgias with
flares
• Pathology:– H&E: LCV, karyorrhexis,
extravasated RBC– DIF: C3, IgM, IgA and/or IgG
granular deposits in vessels
HYPERSENSITIVITY VASCULITIS / CUTANEOUS
LEUKOCYTOCLASTIC VASCULITIS (LCV)
Bolognia, Jean, Joseph L. Jorizzo, and Julie V.
Schaffer. Dermatology. Philadelphia: Elsevier Saunders,
2012. Print.
• Secondary LCV• Idiopathic (50%)• Infection (15-20%)• Inflammatory Disorders (15-20%)• Drug Exposure (10-15%)• Neoplasms (2-5%)• Genetic Disorders (Rare)
• Treatment:– Acute
• Often resolves without treatment• Avoid trigger• Supportive care
– Chronic (>4 wks)• Colchicine, dapsone,
corticosteroids
HYPERSENSITIVITY VASCULITIS / CUTANEOUS
LEUKOCYTOCLASTIC VASCULITIS (LCV)
Bolognia, Jean, Joseph L. Jorizzo, and Julie V.
Schaffer. Dermatology. Philadelphia: Elsevier Saunders,
2012. Print.
• MC children • Etiology: bacterial & viral
infections• Presentation: palpable purpura,
arthralgias, abdominal pain, renal disease
• Pathology:– H&E: LCV– DIF: IgA vasculitis
• Treatment: systemic corticosteroids, immunosuppressants, ACE-I
• Prognosis: monitor for chronic renal insufficiency
HENOCH-SCHONLEIN PURPURA
IGA VASCULITIS
Gaskill, Neil; Guido, Bruce; & Mago, Cynthia. (2016).
Recurrent adult onset Henoch-Schonlein Purpura: a case
report. Dermatology Online Journal, 22(8). doj_32191.
• Children < 2 yrs• Etiology: likely infectious• Presentation: cockade, annular,
purpuric plaques involving the face, ears, distal extremities– No systemic findings
• Pathology:– H&E: LCV
– DIF: IgA vasculitis
• DDx: Child abuse, urticaria, urticarial vasculitis, erythema multiforme
• Treatment: Resolves spontaneously in 1-3 weeks
ACUTE HEMORRHAGIC EDEMA OF CHILDHOOD
Mreish S, Al-Tatari H. Hemorrhagic Lacrimation and
Epistaxis in Acute Hemorrhagic Edema of Infancy
Case Rep Pediatr. 2016;2016:9762185.
• Adults, peak 50s, F>M• Etiology: unknown• Variants:
– Normocomplementemic– Hypocomplementemic
• Associations:– CTD (Sjögren’s, SLE)– Serum sickness– Cryoglobulinemia– Infections (HBV, HCV, EBV, Lyme)– Medications – Hematologic & solid malignancies:
colon & renal cell cancer
URTICARIAL VASCULITIS
James W, Berger T, Elston D. (2011) Andrews’ Diseases of
Skin (11th ed.) Edinburgh, London, New York, Oxford,
Philadelphia, St Louis, Sydney, Toronto, Elsevier Inc.
• Presentation: – Erythematous annular or
targetoid wheals resemble urticaria → progress to purpura with residual hyperpigmentation
– Favor trunk or proximal extremities
– > 24 hours– Recurrent lesions– Painful or burning– Systemic findings: angioedema,
arthralgias, abdominal or chest pain, fever, dyspnea, uveitis, glomerulonephritis & Raynaud’s
URTICARIAL VASCULITIS
Bolognia J, Jorizzo J, Schaffer J. et al. (2009) Dermatology
(3rd ed.) Edinburgh, London, New York, Oxford, Philadelphia,
St Louis, Sydney, Toronto, Elsevier Inc.
3 clinical features distinguish skin lesions of urticarial
vasculitis from true urticaria:
1. Lesions are often painful, rather than pruritic.
2. Lesions >24 hrs and are fixed, rather than migrating.
3. Post-inflammatory purpura or hyperpigmentation.
• Diagnosis: skin biopsy– H&E: < 48 hours in onset
– DIF: < 24 hours in onset
• Pathology: – H&E: LCV + neutrophilic infiltrate
– Hypo DIF: C3 granular pattern in BV & BM
• Treatment: – 1st line: antihistamines, NSAIDs
– Alt: colchicine, hydroxychloroquine, dapsone
– Systemic tx: steroids, azathioprine, mycophenolate mofetil, rituximab
• Prognosis: – Chronic and benign ~ 3 years
URTICARIAL VASCULITIS
Rapini, R. (2012). Practical Dermatopathology (2nd ed.)
Edinburgh, London, New York, Oxford, Philadelphia, St
Louis, Sydney, Toronto, Elsevier Inc.
CRYOGLOBULINEMIC VASCULITIS
• Vasculitis is ONLY seen with types II and III
– Type I can present with vasculopathy
• Small & medium-sized vessels, but preferentially involves
small vessels
• Association with HCV & HBV infections
Bolognia, Jean., et al. eds. Dermatology.
[Philadelphia] :: Elsevier Saunders, 2012. Print.
CRYOGLOBULINEMIC VASCULITIS
• Palpable purpura MC
• Other findings: erythematous
papules, ecchymoses, nodules,
urticaria, livedo reticularis,
necrosis, ulcerations, bullae
• Extracutaneous findings:
– Arthralgias/arthritis (70%)
– Peripheral sensory neuropathy (40%)
– GI symptoms or hepatitis (30%)
– Membranoproliferative
glomerulonephritis (25%)
1Berera SR et al. A Rare Case of Hepatitis C-Associated Cryoglobulinemic
Duodenal Vasculitis. ACG Case Reports Journal. 2016;3(4):e134.2 Dalai et al. An Unusual Case of HCV Negative Cryoglobulinemia
Presenting as Symmetrical Peripheral Gangrene. Journal of Clinical and
Diagnostic Research 2016;10(4):18-19.
CRYOGLOBULINEMIC VASCULITIS
• Laboratory Evaluation:
– Often falsely negative, need to test multiple times
– Blood samples should be kept at 37C (98.6F) while being transported to
lab
• 70% are RF (+); 20% are ANA (+)
• Low C4 levels - do not correlate with disease activity
• Treatment: treat underlying disease!
– Hep C (+): new antivirals (i.e Harvoni), Ribavirin, Interferon
– Plasma exchange or cyclophosphamide with corticosteroids may be
needed for severe renal or neurological involvement
– Rituximab
Bolognia, Jean., et al. eds. Dermatology. [Philadelphia] ::
Elsevier Saunders, 2012. Print.
ERYTHEMA ELEVATUM DIUTINUM
• Symmetric red-violet to red-
brown papules & plaques
• Persistent lesions that develop
on extensor surfaces/small
joints
– Trunk generally spared
– A/w infections, hematologic,
rheumatologic diseases
• Limited to skin
• Pathology: LCV with fibrinoid
necrosis
• Treatment: typically responds
promptly to Dapsone or
sulfapyridine
Momen, S.E., Jorizzo, J. and Al-Niaimi, F. (2014), Erythema
elevatum diutinum: a review of presentation and treatment. J
Eur Acad Dermatol Venereol, 28: 1594–1602.
doi:10.1111/jdv.12566
MIXED (MEDIUM & SMALL) VESSEL VASCULITIS
A. Connective Tissue Disease Associated (usually
rheumatoid vasculitis)
B. Septic Vasculitis
C. ANCA-Associated
1. Microscopic Polyangiitis
2. Granulomatosis with Polyangiitis
3. Allergic Granulomatosis (Churg-Strauss)
A. Polyarteritis Nodosa (benign & systemic)
MEDIUM-VESSEL VASCULITIS
• Classic PAN (25% cases have skin
findings): palpable purpura, livedo
reticularis, retiform purpura,
“punched-out” ulcers
• Cutaneous PAN (10%): painful
subcutaneous nodules & ulcerations
over the lower extremities, particularly
near malleoli
• Extracutaneous manifestations:
– Fever
– Arthralgias
– Myalgias
– Paresthesias
– Abdominal pain
– Orchitis
– Renovascular hypertension
POLYARTERITIS NODOSA
Matteoda MA, Stefano PC, Bocián M, Katsicas MM, Sala J,
Cervini AB. Cutaneous polyarteritis nodosa. Anais
Brasileiros de Dermatologia. 2015;90(3 Suppl 1):188-190.
doi:10.1590/abd1806-4841.20153856.
POLYARTERITIS NODOSA
• Cutaneous variant has chronic, more
benign course
– Often a/w strep infection in children
• HBV, HCV, infections, inflammatory
diseases, malignancies (hairy cell
leukemia) & medications
• Pathology: Segmental necrotizing
vasculitis in subcutaneous tissue
• Treatment– Classic PAN – systemic corticosteroids
(1 mg/kg/day of prednisone)
– Cutaneous PAN – topical or intralesional
steroids, may need systemic steroids if
progressive or extensive
Morgan, A. J. and Schwartz, R. A. (2010), Cutaneous
polyarteritis nodosa: a comprehensive review. International
Journal of Dermatology, 49: 750–756. doi:10.1111/j.1365-
4632.2010.04522.x
• Rare, late complication in patients with
longstanding, erosive, deforming RA
• High morbidity and mortality
• Risk Factors:
– Smoking, HLA-DRB1/similar epitopes,
uncontrolled RA, high +RF titer and anti-
CCP, PVD
• Presentation:
– Purpura, cutaneous ulcers (upper or lower
ext), rheumatoid nodules, digital infarcts,
nail fold infarcts
– Extracutaneous: severe erosive arthritis,
ocular, cardiovascular, pulmonary, renal,
GI, & CNS findings
• Treatment:
– No established guidelines to help guide
therapy, high-dose glucocorticoids +
cyclophosphamide has shown promising
results
RHEUMATOID VASCULITIS
Makol A, Matteson EL, Warrington KJ. Rheumatoid
vasculitis: an update. Curr Opin Rheumatol. 2015
Jan;27(1):63-70.
• Presentation: purpuric papules, macules,
retiform purpura, cutaneous ulceration, livedo,
rarely urticaria
– Systemic: fever, weight loss, myalgias,
arthralgias, segmental necrotizing and
crescentic glomerulonephritis, with pulmonary
involvement, pulmonary capillaritis, vasculitis
neuropathy, eye disease
• Pathology: necrotizing LCV
• Laboratory findings: ANCA + (70%), p-ANCA >
c-ANCA
• Treatment: systemic corticosteroids
– Localized: TMP/SMX + corticosteroids
– Generalized: MTX + CS
– Organ involvement: cyclophosphamide then MMF,
MTX, or azathioprine, IVIG and anti-TNF
(refractory)
MICROSCOPIC POLYANGIITIS
Andrews’ Diseases of the Skin 11th edition.
Bolognia 3rd edition
• Rare, potentially life-threatening PR3-ANCA
associated necrotizing vasculitis of small to medium-sized vessels
and extravascular necrotizing granulomatous inflammation
• F>M; peak age 45-65 years
• Triad
– 1) Necrotizing granulomatous inflammation of upper &
lower respiratory tracts
– 2) Glomerulonephritis
– 3) Necrotizing small to medium-vessel vasculitis
• Presentation:
– Palpable purpura followed by oral ulcers/friable gums
“strawberry gums”
– Painful SQ nodules resemble pyoderma gangrenosum
– Upper or lower respiratory tract involvement
– Glomerulonephritis
– Other: musculoskeletal, ocular, neurological, GI, and cardiac
– Saddle-nose deformity due to mucosal necrotizing
granulomas
GRANULOMATOSIS WITH POLYANGIITIS
R. Heera, Kanaram Choudhary, V. T. Beena, and Simon.R.
Strawberry gingivitis: A diagnostic feature of gingival
Wegener's granulomatosis. Dent Res J (Isfahan). 2012 Dec;
9(Suppl 1): S123–S126.
• Labs:– (+) c-ANCA in 80-90%, generalized
disease, 60% in localized– RF, ESR, CRP, anemia,
leukocytosis, anti-MPO Ab in 10%, proteinuria, RBC casts
• Pathology: – LCV-like changes, palisading
granulomas, granulomatous vasculitis surrounding foci of basophilic necrobiosis
• Treatment: – Corticosteroids + cyclophosphamide
= 75% remission– Corticosteroids + rituximab may be
equally effective
GRANULOMATOSIS WITH POLYANGIITIS
Bolognia, Jean, Joseph L. Jorizzo, and Julie V.
Schaffer. Dermatology. Philadelphia: Elsevier
Saunders, 2012. 3rd edition
• Presentation: palpable purpura,
subcutaneous nodules of extremities and
scalp, firm non-tender purpuric papules of
fingertips, urticaria
• 3 phases:1. Initial: allergic rhinitis, nasal polyps, asthma (35 yo)
2. Secondary (2-12 years later): fever, eosinophilia,
with pneumonia and gastroenteritis
3. Tertiary: diffuse angiitis of the liver, spleen, kidneys,
intestines, and pancreas, mononeuritis multiplex
common
• Medication triggers: vaccination,
desensitization, leukotriene inhibitors,
azithromycin, nasal fluticasone, rapid d/c
corticosteroids
• Death from CHF d/t granulomatous
inflammation of myocardium
CHURG-STRAUSS SYNDROME
Andrews’ Diseases of the Skin 11th edition.
Bolognia 3rd edition
• Laboratory findings: peripheral
eosinophilia, p-ANCA (anti-
myeloperoxidase ab) positive, c-ANA (anti-
PR3) sometimes positive
• Pathology: small and medium LCV, Wells
syndrome with flame figures, palisaded
granulomas lacking giant cells with central
eosinophils
• Treatment: corticosteroids,
cyclophosphamide with corticosteroids if
neuro, renal, myocardial, or
gastrointestinal involvement
– MTX or other steroid-sparing agents can be
used to maintain remission
CHURG-STRAUSS SYNDROME
Ratzinger et al. Eosinophilic leukocytoclastic vasculitis: a
spectrum ranging from wells’ syndrome to Churg-Strauss?
European Journal of Dermatology. 2014 Sept-Oct. 24(5):
603-10.
• HLA-B51
• Diagnostic Criteria:
– Oral ulcers 3 times in 12 month period plus 2 of
below
• Recurrent genital ulcers
• Positive pathergy test
• Uveitis, retinal vasculitis
• Skin findings: Ulcerations, acneiform,
papulopustular, EN-like
• Clinical Features:
– Painful
– CNS lesions (MS-like)
– Thrombophlebitis
– Thrombosis of SVC
– Asymmetric, non-erosive polyarthritis
• Histology:
– Vasculitis may involve small and medium vessels
– Predominantly neutrophilic infiltrate
• Treatment:
– Colchicine, dapsone, thalidomide, TNF-inhibitors
BEHCET’S DISEASE
Rokutanda R, Kishimoto M, Okada M. Update on the diagnosis
and management of Behçet’s disease. Open Access
Rheumatology : Research and Reviews. 2015;7:1-8.
doi:10.2147/OARRR.S46644.
Thank You