Management of adults with idiopathic cutaneous small vessel vasculitis
- Nicole Fett, MD
Nicole Fett, MD
- Assistant Professor
- Oregon Health and Sciences University
Cutaneous small vessel vasculitis (CSVV) is a disorder characterized by leukocytoclastic vasculitis involving the small blood vessels in the skin. By definition, vasculitis affecting other organ systems must be absent. CSVV may develop in association with a wide variety of factors, including drugs, infections, systemic disease, or malignancy. An inciting factor is not detected in around one-third to one-half of patients. Such patients are designated here as having idiopathic CSVV.
Idiopathic CSVV often resolves within a few weeks, and thus, the management of most patients involves confirming the lack of an identifiable cause and the alleviation of symptoms. Systemic immunomodulatory therapy to arrest the disease process is usually reserved for the subset of patients who develop complications such as hemorrhagic bullae, ulceration, or chronic or recurrent disease.
The management of patients with idiopathic CSVV will be reviewed here. The evaluation of patients with cutaneous lesions of vasculitis, and the clinical features and treatment of specific disorders that may present with vasculitis involving small vessels of the skin are discussed separately. (See "Evaluation of adults with cutaneous lesions of vasculitis" and "Overview of cutaneous small vessel vasculitis" and "Urticarial vasculitis" and "Henoch-Schönlein purpura (immunoglobulin A vasculitis): Management" and "Treatment of rheumatoid vasculitis" and "Treatment and prognosis of eosinophilic granulomatosis with polyangiitis (Churg-Strauss)" and "Initial immunosuppressive therapy in granulomatosis with polyangiitis and microscopic polyangiitis" and "Treatment of the mixed cryoglobulinemia syndrome".)
Other terms that have been used to refer to cutaneous small vessel vasculitis (CSVV) include cutaneous leukocytoclastic vasculitis, cutaneous leukocytoclastic angiitis, and cutaneous necrotizing venulitis, all of which refer to the characteristic histopathologic findings in CSVV. The term hypersensitivity vasculitis has also been used to refer to CSVV, but is most appropriately applied to CSVV caused by a known drug or infection. (See "Evaluation of adults with cutaneous lesions of vasculitis", section on 'Terminology' and "Overview of cutaneous small vessel vasculitis".)
The 2012 Revised International Chapel Hill Consensus Conference Nomenclature classifies CSVV as a single-organ vasculitis .
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- CLINICAL FEATURES AND EVALUATION
- DISEASE COURSE
- Acute and uncomplicated idiopathic CSVV
- Complicated or chronic idiopathic CSVV
- - First-line therapy
- Systemic glucocorticoids
- - Second-line therapy
- - Refractory disease
- - Other therapies
- - Duration of therapy
- SUMMARY AND RECOMMENDATIONS