LEUKOCYTOCLASTIC VASCULITIS (SMALL VESSEL VASCULITIS)

By Gary M. White, MD

Leukocytoclastic vasculitis


Leukocytoclastic vasculitis (LCV) is a histologic description of a type of small vessel vasculitis. It has a myriad of causes although many cases are idiopathic.

Clinical

A shower of purpuric lesions, both palpable and non-palpable, concentrated on the legs, occurs in the classic presentation of leukocytoclastic vasculitis. The patient often feels well, but fever, malaise, nausea, and arthralgias may be associated. Other lesions may occur including purpuric nodules and/or papules, necrosis, ulceration, hemorrhage, bulla, EM-like lesions, and annular lesions. Acute, subacute, and chronic forms occur. Erythema elevatum diutinum, Henoch-Schonlein purpura, and urticarial vasculitis show LCV on biopsy. Systemic involvement should be considered including renal, GI, lung, and CNS. Erythema multiforme or septicemia with cutaneous lesions may be part of the differential diagnosis.

Often, the classic eruption of LCV comes and goes before a workup can be completed.

Causes

Many causes have been associated with this hypersensitivity vasculitis including inflammatory bowel disease, larger vessel vasculitis (e.g., PAN, Granulomatosis with polyangiitis), malignancy (e.g., lymphoma, myeloma, leukemia, solid tumors [e.g., ovarian JAAD 1999;40;287]), drugs (e.g., levamisole associated with cocaine use, Propylthiouracil can induce ANCA-associated microscopic polyangiitis or lupus-like disease, the nicotine patch [BJD 1996;134;361]), infection (e.g., urinary tract infection, Parvovirus B19, streptococcus, hepatitis B/C, mycobacterium leprae, Brucellosis), cryoglobulins, macroglobulinemia, collagen vascular disease (e.g., SLE, Sjögren's syndrome), rheumatoid vasculitis, chronic active hepatitis, and the food additive sodium benzoate [AD 1999;135;726].

Exercise

A study of long distance walking [BJD 1996;134;91] found purpuric lesions may develop in athletes after prolonged exercise. Erythema, purpura, and urticaria of the lower legs was seen. LCV was found on biopsy of the purpura. Such changes were much more common in those with signs of chronic venous insufficiency. See exercise-induced purpura.

Workup

For the patient who presents with classic palpable purpura without obvious signs of systemic disease or cutaneous necrosis, the initial workup may be as minimal as a good HP, with emphasis on any new drugs, signs of infection (e.g., sore throat, fever, sinusitis, dental symptoms), OTC medications, lymph node exam, CBC, urine analysis, and stool for occult blood. However, if the disease becomes chronic, the patient is systemically ill, or the skin involvement is severe, workup may also include a skin biopsy (the best lesion to biopsy is a well-formed papule about 24 hours old) and the following laboratory data.

Laboratory

CBC, stool guaiac, creatinine, LFT's, urine analysis, RF, ASO, ANA, ANCA, SPEP, Hepatitis B and C, ELISA for parvovirus B19, complement levels, cryoglobulins, anti C1Q antibody and IgA-fibronectin aggregates (if available), and CXR. For the acutely ill patient, the most important entities to exclude are septic emboli as a cause (e.g., Neisseria, Staphylococcus, RMSF, Streptococcus, Pseudomonas, Candida). One may aspirate tissue fluid from an early lesion for gram stain and culture.

Treatment of Acute LCV

Of course, any primary disease, infection, etc., should be addressed and eliminated if possible. If a cause is identified and eliminated (e.g., drug, infection), milder cases may be observed expectantly. Prednisone is highly effective but has significant side effects. Other steroid-sparing agents may be used. Minocycline 100 BID was very helpful combined with low-dose prednisone in a patient with rheumatoid arthritis and LCV [AD 1997;133;15].

Treatment of Chronic LCV

Colchicine has been recommended for patients with chronic, idiopathic LCV [AD 1998;134;355]. Dapsone may also be tried. Other options are every other day low-dose prednisone, weekly, low-dose methotrexate, mycophenolate mofetil, or azathioprine (e.g., 2 mg/kg/day).

Pediatric LCV

In one study of LCV in children [JEADV 2017;31;544], 48% of cases were IgA vasculitis (Henoch-Schonlein purpura), 34% small-vessel vasculitis, 9% urticarial vasculitis, 7% ANCA-associated vasculitis and 2% acute hemorrhagic edema of infancy. IgA vasculitis was associated with abdominal pain, whereas non-IgA vasculitis was associated with headache.

Additional Pictures

Leukocytoclastic vasculitis Leukocytoclastic vasculitis Leukocytoclastic vasculitis Leukocytoclastic vasculitis

Macular, purpuric almost targeted lesions.
Leukocytoclastic vasculitis

Necrotic bulla
Leukocytoclastic vasculitis

Annular Leukocytoclastic Vasculitis
Leukocytoclastic vasculitis Leukocytoclastic vasculitis

Whitish centers.
Leukocytoclastic vasculitis

Vasculitis of the ankle (leukocytoclastic vasculitis on biopsy) in a patient whose SLE was flaring. Exam showed necrotic, vesicular lesions.
Lupus Vasculitis

References

Leukocytoclastic vasculitis sparing a tattoo JAAD Case Reports September 2015 Volume 1, Issue 5, Pages 269–271

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