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Leukocytoclastic vasculitis in Child
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Leukocytoclastic vasculitis in Child

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Contributors: Andrew Walls MD, Belinda Tan MD, PhD, Susan Burgin MD
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Synopsis

Leukocytoclastic vasculitis (LCV) is a small vessel vasculitis that predominantly affects postcapillary venules in the dermis.

LCV can occur in all ages and in both sexes. It is more commonly found in adults, but up to 10% of cases are in the pediatric population. The clinical hallmark of LCV is palpable purpura: purpuric papules erupt symmetrically on the shins 7-10 days after an inciting factor. Other parts of the lower extremities may be involved including thighs and dorsal feet. Less frequently, the buttocks, upper extremities, and abdomen are involved. Initially, purpuric macules may be seen that give way to palpable purpura. In more advanced cases, bullae and ulcers may be seen. While the majority of cases are asymptomatic, LCV can be associated with pruritus, pain, or burning.

A variety of inciting factors have been identified, including medications (especially antibiotics, NSAIDs, and diuretics), pathogens (hepatitis viruses, human immunodeficiency virus [HIV], Epstein-Barr virus, and streptococci), malignancy, inflammatory bowel disease, or connective tissue disease (systemic lupus erythematosus [SLE], Sjögren syndrome, and rheumatoid arthritis). Up to 50% of cases, however, have no identifiable cause and are considered idiopathic.

Pathogenically, antigen-antibody complexes bind to the endothelium. The complement cascade is subsequently activated, effecting neutrophil chemotaxis. Vessel wall damage then ensues secondary to proteolytic enzymes and reactive oxygen species released by neutrophils. The physician will need to differentiate skin-limited LCV from a systemic vasculitis and should suspect the latter if fever, myalgias, malaise, lymphadenopathy, abdominal pain, melena, hematochezia, diarrhea, hematuria, lower extremity swelling, or paresthesias are noted. The level of systemic involvement will largely influence the prognosis. Over 90% of patients with LCV limited to the skin will experience spontaneous resolution over several weeks to months.

Codes

ICD10CM:
L95.8 – Other vasculitis limited to the skin

SNOMEDCT:
60555002 – Leukocytoclastic vasculitis

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

The differential diagnosis for LCV is extensive. A skin biopsy will largely aid in the diagnosis.

Other small vessel vasculitis:
  • Henoch-Schönlein purpura – Acute onset of palpable purpura usually seen in children aged younger than 10 years in the lower extremities / buttocks 1-2 weeks after a respiratory infection. Fever, arthralgias, and renal and gastrointestinal involvement are commonly seen. Can occur in adults. Renal sequelae are of significant concern in these patients.
  • Urticarial vasculitis (UV) – Recurrent, painful eruptions of urticarial lesions that last for more than 24 hours (assists in differentiating from typical urticaria) with or without angioedema. Fever, malaise, myalgias, and arthritis are commonly associated. UV has a strong association with autoimmune disease, such as SLE and Sjögren syndrome, and these entities should be investigated. Complement levels can predict systemic involvement; normal levels are seen with cutaneous limited disease, hypocomplementemic levels are seen with arthritis and gastrointestinal and pulmonary involvement.
  • Microscopic polyangiitis – High incidence of renal involvement with associated weight loss, fever, and other systemic symptoms. Typically ANCA positive (about 10% are ANCA negative).
  • Erythema elevatum diutinum – Violaceous, erythematous, or brown papules, nodules, and/or plaques, predominantly over the extensor surfaces of the body. Chronic course with spontaneous resolution after 5-10 years.
Other small vessel vasculitis that also affects medium-sized vessels (typically with systemic organ involvement):
Infection:
Other:

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Management Pearls

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Therapy

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Drug Reaction Data

Below is a list of drugs with literature evidence indicating an adverse association with this diagnosis. The list is continually updated through ongoing research and new medication approvals. Click on Citations to sort by number of citations or click on Medication to sort the medications alphabetically.

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References

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Last Reviewed: 11/17/2016
Last Updated: 06/15/2018
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Leukocytoclastic vasculitis in Child
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Leukocytoclastic vasculitis : Palpable purpura, Symmetric extremities, Arthralgia
Clinical image of Leukocytoclastic vasculitis
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