Leukocytoclastic vasculitis

Last Updated: 2022-03-11

Author(s): Anzengruber F., Navarini A.

ICD11: 4A44.BZ

  • Gougerot 1932.
  • Ruiter and Brandsma 1948.

Anaphylactoid purpura, Arteritis allergica cutis, Arteriolitis allergica cutis, Arteriolitis hyperergica cutis, Cutaneous leukocytoclastic vasculitis, Cutaneous small vessel vasculitis, Gougerot-symptom, hyperergic vasculitis, hyperergic vasculitis, hypersensitivity angiitis, immune complex vasculitis, cutaneous immune complex vasculitis, cutaneous necrotising vasculitis, leukocytoclastic purpura, leukocytoclastic vasculitis, necrotising vasculitis, purpura rheumatica
purpura rheumatica, vasculitis allergica, vasculitis hyperergic, vasculitis necrotising, vasculitis allergica

Collective term for immune complex vasculitis, which is characterised by petechial exanthema.

  • Most common manifestation of necrotising vasculitis
  • Non age-specific.
  • Incidence: 20/100,000/year.
  • F: M = 1:1.

  • Mostly after infections, immune complex formation occurs. These immune complexes accumulate on the vessel walls (and perivascularly), resulting in complement activation and thus the migration of granulocytes and lysozymal enzymes.
  • Trigger factors:
    • Drugs (allopurinol, ASA, β-blockers, contraceptives, streptomycin, penicillin, etc.).
    • Infections.
    • Bacterial (mostly strep, tonsillitis, erysipelas, GI infections, CMV, etc.).
    • Viruses (hepatitis A, B, C virus, etc.).
    • Chronic inflammations (inflammatory bowel disease, etc.).
    • Neoplasms (CLL, mycosis fungoides, colon, breast, lung, prostate CA, multiple myeloma, etc.).
    • Collagenoses (Sjörgren's syndrome, SLE, etc.).
    • Rheumatic diseases (rheumatoid arthritis, etc.).

Localisation:

  • especially lower leg.
  • Arms and trunk may also be affected.
  • ↓ general condition with fever, fatigue, cephalgias, arthralgias.
  • Petechial petechiae that cannot be pushed away diascopically, mostly on the lower legs.

Hemorrhagic type:

  • Small, pinhead- to coin-sized, slightly raised petechiae, first red, then blue and brownish as they progress.

Hemorrhagic-necrotic type:

  • Partly necrotising and ulcerating haemorrhages.

Papulonecrotic type:

  • Macules, papules with central ulcerative necrosis, nodules and wheals.

Special form:

Purpura Schoenlein-Henoch:

  • IgA immune complex vasculitis
  • Mostly occurring in the cold season
  • Children:
    • Frequently following streptococcal infection.
    • Triad: Palpable purpura, abdominal colic and arthritis
    • More mild course.
  • Adults:
    • Severe course
      • CAVE: Renal involvement (IgA glomerulonephritis)
      • Therapy: Immunosuppressive: e.g.: Prednisolone 1 mg/kg bw/d, tapering off in the course.

  • Anamnesis (infections (diarrhoea, flu-like infection, burning during urination, productive cough, toothache, sinusitis, etc.)? Unusual/new medication use? Neoplasia? Underlying diseases? Collagenoses?)
  • Clinical appearance
  • Biopsy (take as far cranial as possible), as well as direct immunofluorescence (IgA, IgM) --> biopsy the freshest lesion it has for this.
  • Body temperature measurement.
  • Laboratory:
    • Blood count (leukocytosis), ↑ sedimentation rate, ↑ CRP, coagulation, liver and kidney values, anti DNase B, antistreptolysin titre, circulating immune complexes, cryoglobulins, hepatitis B and hepatitis C serologies, HIV serology, ANA, ds DNA, Rh factor, ANCA, quantiferon.
  • Urine status (proteinuria, microhaematuria)
  • Hemoccult, stool examination for pathogenic germs
  • ECG
  • Rapid urease breath test, e.g. for Helicobacter pylori infection
  • Focus search (Lymph node palpation, X-ray thorax, upper abdominal sonography)
  • Consil: ENT, dental consil, in women: gynaecological consil.

Legs, especially lower legs. Upper body may also be affected in more severe cases.

  • Fibrinoid vessel wall necrosis, perivascular oedema, leucocyte diapedesis and perivascular leucocytoclasia, erythrocyte extravasates, immune complexes, erythrocyte extravasates, siderophages.

Direct immunofluorescence

  • IgG, IgA deposits in the vessel walls.

  • Healing can be expected within a few weeks in most cases.
  • However, chronic courses are also possible.

  • Hospital admission
  • Bed rest.
  • Elevation of the legs.
  • Compression bandage according to Pütter.
  • Elimination of the trigger or therapy of the underlying disease

     

    Topical therapy

  • Mometasone fuorate cream / solution / ointment
  • Clobetasol cream 1-2 daily (for 1-3 days)
  • Polyvidone-iodine wound ointment/ solution / ointment gauze
  • Compression bandages

     

    Systemic therapy

  • Prednisolone p.o. 1 mg/kg bw daily (taper off over approx. 2 weeks)
    • Additive in case of therapy resistance or systemic involvement
  • Cyclosporine p.o. 3-5 mg/kg bw daily
    • Take independently of meals
      • Initially, simultaneous administration with prednisolone is advisable, as the onset of action is delayed.
  • Alternatively:
  • Intravenous immunoglobulins i.v. 1g/ kg bw daily divided over 2 days, every month
    • Initial dose: 0.4-0.8 g/kg bw
    • In the course: 0.2 g/kg bw every 3 to 4 weeks
    • Determination of the IgG serum level always immediately before the next infusion!
    • An IgG valley level of at least 5 to 6 g/l should be achieved before re-infusion.

 

A discontinuation or extension of the interval may still be attempted after several months of successful therapy.

 

If the vasculitis takes a chronic course, permanent immunosuppressive maintenance therapy is indicated (as little as possible, as much as necessary).

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