cutaneous leukocytoclastic vasculitis
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Introduction
A group of disorders with vasculitis of the small vessels of the skin. Skin manifestations are predominant.
Etiology
- hypersensitivity reaction
- immune complex formation
- foreign antigens
- drug reaction (20%)
- microbe or foreign protein (22%)
- self antigen, idiopathic (40%)
- malignancy - lymphoproliferative disorders
- occurs in patients with connective tissue diseases (12%)
Pathology
- vasculitis of small vessels
- postcapillary venules most commonly involved
- leukocytoclasis in & around vessels
- mononuclear infiltrates in chronic phase
Clinical manifestations
- predominance of skin involvement
- skin lesions
- palpable purpura (most common)
- macules
- papules
- vesicles
- bullae
- subcutaneous nodules
- ulcers
- urticaria
- recurrent or chronic
- may become painful
- edema may accompany skin lesions
- hyperpigmentation occurs in regions of chronic lesions
- 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]
- symptoms of medication-induced cutaneous vasculitis generally manifest 7-21 days after initial exposure to the triggering agent[3]
- skin lesions
- systemic symptoms may be present
* images[5]
Laboratory
- leukocytosis (mild)
- eosinophilia may be present
- elevated erythrocyte sedimentation rate (ESR)
- cryoglobulinemia may be present
- rheumatoid factor may be positive
- biopsy shows cutaneous vasculitis (see pathology)
Differential diagnosis
- drug-induced vasculitis
- serum-sickness
- cryoglobulinemia
- vasculitis associated with underlying disease
- disorders with vasculitis as a minor component
Management
- general measures
- most cases resolve spontaneously
- recurrences generally remit completely
- remove antigenic stimuli
- antimicrobials for infection
- treat associated systemic illnesses
- 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
- plasmapheresis for fulminant cases
More general terms
Additional terms
- 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
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
- ↑ 3.0 3.1 3.2 3.3 3.4 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: https://www.ncbi.nlm.nih.gov/pubmed/18284262
- ↑ 5.0 5.1 Eastham ABW, Diamond HS (images) Medscape: Leukocytoclastic Vasculitis. http://emedicine.medscape.com/article/333891-overview