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Case Note

Case Note: Purpuric Papules and Plaques on the Arms and Legs

History

  • A 40-year-old female is admitted to hospital for a wound infection following right-knee surgery indicated for an anterior and posterior cruciate ligament tear (performed 1 week ago)
  • Upon admission, the patient is noted to have symmetric papular and purpuric eruption on the arms and legs

Initial evaluation

  • Tired-appearing female presents with scattered purpuric macules and papules on bilateral arms and legs
    • Present for 2 days
    • No mucosal lesions noted
  • The surgical site on the right knee appears erythematous, warm, and with purulent drainage
  • Patient has no significant past medical history and she takes no medications, except for an opiate-based pain medication for her knee surgery
  • Review of systems is notable for recent low-grade fevers in association with the rash
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Enter your diagnosis

Diagnosis

  • A diagnosis of leukocytoclastic vasculitis (LCV) is rendered
    • Diagnosis of LCV favored over wound infection due to patient's medication (opiates)
    • Skin biopsy confirms diagnosis

Initial Treatment

  • Patient initiated on topical triamcinolone 0.1% ointment twice daily and hydroxyzine 25 mg at bedtime to help with skin pruritus
  • Concern for systemic involvement and/or underlying systemic disorder prompts stool guaiac testing, urinalysis with microscopy, and blood test for blood urea nitrogen and creatinine clearance, erythrocyte sedimentation rate, antistreptolysin O (ASO), and complete blood count with differential
    • All tests were normal except for a slight leukocytosis with neutrophilia and positive ASO titer, suggestive of streptococcal wound infection
      • Patient is started on systemic antibiotics
  • Patient's opiate-based pain medication is discontinued and she is switched to ibuprofen when her kidney function tests returned to normal
  • Follow up in 1 week

Follow-up evaluation strategy

1-week follow-up evaluation:

  • The rash is resolving and the patient reports no new lesions
  • It is recommended that the course of systemic antibiotics for the streptococcal infection is completed
  • Topical corticosteroids and antihistamines are continued for symptomatic relief
  • Further follow up at 4 weeks
    • Resolved

 

Further recommendations:

  • For skin pruritis, non-sedating antihistamines (e.g. loratadine, fexofenadine, cetirizine) may be used if first-generation agents are not tolerated
  • In addition to topical corticosteroids to treat cutaneous ulcers or bullae, emollients such as petrolatum may also be applied
    • Non-healing wounds benefit from semi-occlusive dressings with gentle compression

 

Subsequent treatment steps:

  • No treatment; wait 2-3 weeks for spontaneous resolution
    • This approach is acceptable if the lesions cause limited symptoms and there is no evidence of internal organ involvement
  • Treat with prednisone 1-2 mg/kg daily for 4-7 days
    • If the patient responds, taper prednisone over a 14-day period
  • Treat with colchicine 0.6 mg one or two tablets twice daily as tolerated
  • Treat with dapsone 50 mg twice daily and increase the dose up to 100 mg twice daily if required
  • Dapsone and colchicine may be combined for enhanced efficacy, to minimize toxicity from either medication (hemolysis and diarrhea, respectively)
  • Hydroxychloroquine 200 mg twice daily, azathioprine 50-100 mg daily, or methotrexate 10-25 mg weekly may be used as steroid-sparing agents
    • These agents are used for mild, persistent, but not rapidly progressive disease
  • If a steroid-sparing immunosuppressive agent is needed for aggressive disease, the two favored agents would be mycophenolate mofetil and, in severe cases, cyclophosphamide

General discussion

LCV represents inflammation of small vessels in the skin stemming from immune complex deposition. It is sometimes associated with skin pruritus or tenderness. Clinically, LCV presents with an eruption of palpable monomorphous purpuric papules, which may have central vesicles or pustules and may coalesce into larger plaques. The presentation is typically symmetric and affects dependent areas of the body (lower extremities in an ambulatory patient, sacrum and buttocks in a hospitalized patient). In approximately 50% of cases, cutaneous LCV is caused by medications, drugs (cocaine), infection (such as hepatitis, streptococcal infection), autoimmune disease, or malignancy. In rare cases, LCV will present in the setting of a syndrome such as Henoch-Schönlein purpura, typified by the triad of cutaneous LCV (immunoglobin A deposition), arthritis, and gastrointestinal involvement in which the skin lesions predominantly affect the lower extremities and buttocks.

 

The therapeutic strategy is to treat the underlying disorder. Most cases of cutaneous LCV will require no or only brief courses of immunosuppressive therapy so, if symptoms persist, the concern for a systemic vasculitis should be diagnostically evaluated. Cutaneous LCV may be associated with internal organ involvement, especially of small vessels of the brain, lungs, kidney, and gastrointestinal system, and the morphology and severity of the skin disease do not predict such involvement. Therefore, systemic small vessel vasculitis must be evaluated in every patient presenting with LCV. Treatment may be required for systemic disease even when skin lesions are mild.

Further reading

Podjasek JO, Wetter DA, Pittelkow MR, Wada DA. Cutaneous small-vessel vasculitis associated with solid organ malignancies: the Mayo Clinic experience, 1996 to 2009. JAAD 2012;66:e55-65.

Ting TV. Diagnosis and management of cutaneous vasculitis in children. Ped Clin North Amer 2014;61:321-6.

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