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Last updated: November 27, 2024
Keywords #
- cutaneous small vessel vasculitis (CSVV)
- leukocytoclastic vasculitis
- LCV
- small-vessel vasculitis
- immune-complex
Diagnosis #
Cutaneous small vessel vasculitis (CSVV) encompasses a group of vasculitic disorders characterized histopathologically by leukocytoclastic vasculitis (LCV) resulting from immune complex deposition within dermal capillaries and venules [1]. CSVV is a skin-predominant disorder; however, extra-cutaneous manifestations may occur in less than 30% of cases[2]. While the majority of cases are idiopathic, secondary causes include acute or chronic infections, medications, underlying systemic diseases (including connective tissue diseases), or malignancies[2]. The vast majority of cutaneous-limited cases spontaneously resolve within weeks to months—though chronic, relapsing cases occur.
Key Concepts #
- Cutaneous small vessel vasculitis is caused by immune-complex deposition in small vessels. Leukocytoclastic vasculitis is the histologic hallmark of CSVV.
- CSVV can be idiopathic or secondary to a medication, infection, underlying systemic disease, or malignancy.
- CSVV classically presents as palpable purpura on the lower extremities.
- Biopsy with H&E and DIF is the gold standard for diagnosis. Initial work-up should include a complete blood count, basic metabolic panel, and a urinalysis with microscopy. Further work-up should be guided by a review of systems and included if systemic involvement, or an underlying disorder, are suspected.
- Treatment of mild disease is supportive, with dapsone or colchicine used for more extensive or refractory cases. Systemic steroids are reserved for progressive or severe presentations.
Epidemiology #
The incidence of CSVV is approximately 21 cases per million, affecting both sexes equally [3]. CSVV most commonly affects the adult population, but can be seen in all ages [1].
Clinical Features #
- CSVV classically presents with non-blanching palpable purpura and petechia though erythematous papules, urticarial plaques, targetoid lesions, and/or hemorrhagic vesicles may be present. [3]
- CSVV predominantly affects the lower extremities, but can also involve the buttocks, upper extremities, and abdomen.
- Lesions may be asymptomatic or associated with burning, pain, or pruritus.
- In systemic cases, patients may develop fever, myalgias, arthralgias, malaise, lymphadenopathy, dyspnea, abdominal pain, melena, hematochezia, diarrhea, hematuria, lower extremity swelling, or paresthesias. [4]
Differential Diagnoses #
- Thrombocytopenic purpura
- Specific small vessel vasculitis subtypes presenting with LCV histopathologically and additional morphologic features or findings on direct immunofluorescence
- Henoch-Schonlein purpura/IgA vasculitis
- Erythema elevatum diutinum
- Cryoglobulinemic vasculitis
- Urticarial vasculitis
- Mixed vessel vasculitis/other systemic vasculitis
- Pigmented purpuric dermatoses
- Secondary purpura (as is seen with arthropod bites, viral exanthems, drug eruptions)
- Amyloidosis
- Scurvy
- Endocarditis
- Other infections
Diagnostic Workup #
Punch biopsy for routine histopathology and direct immunofluorescence studies should be performed if vasculitis is suspected. Histopathology classically reveals an infiltrate of inflammatory cells, predominantly neutrophils, and karyorrhexis around small vessels in the upper dermis and less frequently around the vessels of the deep dermis or hypodermis [2]. Fibrin deposition within vessel walls and erythrocyte extravasation are often present as well [1]. Direct immunofluorescence should be performed to help differentiate underlying disease.
Initial evaluation should include a complete blood count with differential, a basic metabolic panel, and a urinalysis with microscopy for all patients. Further work-up may be pursued to screen for systemic involvement or identify an underlying etiology when dictated by the history, review of systems, or examination. Additional testing may include a viral hepatitis panel, HIV testing, cryoglobulins, complement levels, ANCA studies, ANA, SPEP/IFE, and rheumatoid factor [2,4] .
Treatment #
Treatment depends on the severity and underlying etiology of the vasculitis. First steps involve discontinuing incriminated drugs, or treating the underlying infection, malignancy, or systemic disease when present.
For skin-limited, mild disease, supportive measures should be initiated including leg elevation, gentle compression, and rest [2]. Symptomatic cases can be treated with NSAIDs, antihistamines, or topical corticosteroids. More severe or chronic cases can be treated with systemic agents such as colchicine and dapsone. Systemic steroids are reserved for severe, progressing or recalcitrant cases and steroid-sparing agents may be necessary for those unable to taper successfully [3].
Slide Viewer #
https://utahderm.med.utah.edu/image-viewer/References #
- Lotti, T., et al., Cutaneous small-vessel vasculitis. J Am Acad Dermatol, 1998. 39(5 Pt 1): p. 667-87; quiz 688-90.
- Baigrie, D., et al., Leukocytoclastic Vasculitis (Hypersensitivity Vasculitis), in StatPearls. 2020, StatPearls Publishing
- Bolognia, J., J.V. Schaffer, and L. Cerroni, Dermatology. 2018.
- Carlson, J.A., L.F. Cavaliere, and J.M. Grant-Kels, Cutaneous vasculitis: diagnosis and management. Clin Dermatol, 2006. 24(5): p. 414-29.
