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cutaneous leukocytoclastic vasculitis
A group of disorders with vasculitis of the small vessels of the skin. Skin manifestations are predominant.
Etiology:
1) hypersensitivity reaction
2) immune complex formation
3) foreign antigens
a) drug reaction (20%)
b) microbe or foreign protein (22%)
4) self antigen, idiopathic (40%)
a) DNA
b) immunoglobulin
5) malignancy - lymphoproliferative disorders
6) occurs in patients with connective tissue diseases (12%)
a) systemic lupus erythematosus
b) rheumatoid arthritis [3]
Pathology:
1) vasculitis of small vessels
2) postcapillary venules most commonly involved
3) leukocytoclasis in & around vessels
4) mononuclear infiltrates in chronic phase
Clinical manifestations:
1) predominance of skin involvement
a) skin lesions
- palpable purpura (most common)
- macules
- papules
- vesicles
- bullae
- subcutaneous nodules
- ulcers
b) urticaria
- recurrent or chronic
- may become painful
c) edema may accompany skin lesions
d) hyperpigmentation occurs in regions of chronic lesions
- livedo reticularis
e) distribution
- vasculitis typically occurs on lower extremities, especially below the knees or where tight-fitting clothing is worn
- involvement of upper extremities, trunk, head or neck suggest severe disease or associated disorder [3]
f) symptoms of medication-induced cutaneous vasculitis generally manifest 7-21 days after initial exposure to the triggering agent [3]
2) systemic symptoms may be present
- fever
- malaise
- myalgia
- anorexia
* images [5]
Laboratory:
1) leukocytosis (mild)
2) eosinophilia may be present
3) elevated erythrocyte sedimentation rate (ESR)
4) cryoglobulinemia may be present
5) rheumatoid factor may be positive
6) biopsy shows cutaneous vasculitis (see pathology)
Differential diagnosis:
1) drug-induced vasculitis
2) serum-sickness
3) cryoglobulinemia
4) vasculitis associated with underlying disease
a) systemic lupus erythematosus
b) rheumatoid arthritis
c) Sjogren's syndrome
5) disorders with vasculitis as a minor component
a) EBV infection
b) AIDS
c) bacterial endocarditis
d) chronic active hepatitis
e) ulcerative colitis
f) primary biliary cirrhosis
g) alpha-1 antitrypsin deficiency
Management:
1) general measures
- most cases resolve spontaneously
- recurrences generally remit completely
- remove antigenic stimuli
- antimicrobials for infection
- treat associated systemic illnesses
2) pharmacologic agents
- prednisone 1 mg/kg QD tapered to clinical response
- cyclophosphamide for glucocorticoid-resistant disease
- methotrexate or azathioprine may be used as glucocorticoid-sparing agents
- dapsone, NSAIDs, antihistamines or colchicine may be useful [3]
- dramatic response to any agent is rare
3) plasmapheresis for fulminant cases
Interactions
disease interactions
Related
bulla; bleb
cryoglobulinemia (cryoglobulinemic vasculitis)
eosinophilia
leukocytoclasis
macule
papule
rheumatoid arthritis (RA)
Sjogren's syndrome (autoimmune epitheliitis)
systemic lupus erythematosus
urticaria (hives)
vesicular lesion; blister
General
cutaneous vasculitis; predominantly cutaneous vasculitis
leukocytoclastic vasculitis; hypersensitivity vasculitis; trisymptome
References
- Harrison's Principles of Internal Medicine, 14th ed.
Fauci et al (eds), McGraw-Hill Inc. NY, 1998, pg 1919-20
- Weiner S, In: UCLA Intensive Course in Geriatric Medicine
& Board Review, Marina Del Ray, CA, Sept 12-15, 2001
- Medical Knowledge Self Assessment Program (MKSAP) 15, 16.
American College of Physicians, Philadelphia 2009, 2012
- Chen KR, Carlson JA.
Clinical approach to cutaneous vasculitis.
Am J Clin Dermatol. 2008;9(2):71-92.
PMID: 18284262
- Eastham ABW, Diamond HS (images)
Medscape: Leukocytoclastic Vasculitis.
http://emedicine.medscape.com/article/333891-overview