DefinitionThis section has been translated automatically.
Polyorganic or also monoorganic vascular inflammation(vasculitis) of small vessels with deposits of immune complexes in the vessel walls and perivascularly with a characteristic hsitological substrate (leukocytoclastic vasculitis - see also leukocytoclasia).
ClassificationThis section has been translated automatically.
In the classification of leukocytoclastic vasculitis, a distinction is made between:
LcV, largely IgA-associated:
- LcV (IgA-associated/Purpura Schönlein-Henoch)
- Infantile, acute, hemorrhagic edema (age < 2 years)
LcV (non-IgA-associated)
- LcV (non-IgA-associated) - with/without systemic involvement.
- Urticarial vasculitis:
- Normocomplementemic urticarial vasculitis
- Hypocomplementemic urticarial vasculitis.
Serum sickness
Vasculitis in essential cryoglobulinemia
Other (special forms):
- Granuloma (eosinophilicum) faciei
- Erythema elevatum diutinum
- Septic vasculitis
- Purpura fulminans
- LcV with eosinophilia (entity questionable)
- LcV in collagenoses (SLE, scleroderma).
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DiagnosticsThis section has been translated automatically.
Detection of perivascular immune complexes using direct immunofluorescence (DIF). The analysis of these complexes provides an indication of the type of immune complexes involved (e.g. IgA vasculitis, IgG/IgM vasculitis, cryoglobulinemic vasculitis, lupus or rheumatoid vasculitis).
DiagnosisThis section has been translated automatically.
Detection of perivascular immune complexes using direct immunofluorescence (DIF). The analysis of these complexes provides an indication of the type of immune complexes involved (e.g. IgA vasculitis, IgG/IgM vasculitis, cryoglobulinemic vasculitis, lupus or rheumatoid vasculitis).
It is not uncommon for IC vasculitis that is otherwise confirmed to be negative. This is probably due to the age of the biopsied efflorescence. Sequential biopsies of patients, sometimes after provocation with histamine and kinetics, have shown that the positivity subsides after 48-72 hours and only C3 or fibrinogen is still detectable. A higher yield can be achieved with fresh, slightly raised efflorescences, sometimes with still compressible redness.
An older efflorescence should be used for histologic confirmation because it more reliably shows the characteristic features of leukocytoclastic vasculitis (perivascular neutrophilia, nuclear dust, fibrinoid necrosis of the postcapillary venules, erythrocyte extravasations).
In a larger study (n=56), in a group of patients with immune complex vasculitis, biopsies of early lesions revealed perivascular deposits of at least 1 immunoglobulin in 92.9% of DIF, 7.1% were negative.
Perivascular IgA was found in 85.7%, of these around 10% also had perivascular IGM, around 2% also had IgG and around 6% IgM and IgG.
Of the IgA-positive patients, around 20% showed evidence of systemic IgA vasculitis (postprandial abdominal pain, signs of IgA nephritis or arthritis). In 80% of this group, the vasculitis was limited to the skin.
Note(s)This section has been translated automatically.
The high number of patients with cutaneous (rather than systemic) IgA vasculitis indicates that this is a selective dermatological collective, whereas results from other collectives are primarily characterized by a non-dermatological organ component (e.g. kidney). In severe cases, this is associated with high levels of galactose-deficient IgA1. The perivascularly deposited complement factor C3 is the most frequently detected component in the DIF, but still has the same significance as the detection of immunoglobulins. It is formed during an amplification step of the complement cascade, whereby the number of C3 molecules formed can significantly exceed that of the immunoglobulins.
LiteratureThis section has been translated automatically.
Herda L et al. (2025) High detection rate for perivascular deposits of immunoglobulins in immune complex vasculitis from biopsies of early macular lesions. J Dtsch Dermatol Ges 23:479-485.
Kawasaki Y et al. (2003) Clinical and pathological features of children with Henoch-Schoenlein purpura nephritis: risk factors associated with poor prognosis. Clin Nephrol 60: 153-160
Linskey KR et al. (2012) Immunoglobulin-A--associated small-vessel vasculitis: a 10-year experience at the Massachusetts General Hospital. J Am Acad Dermatol. 2012 May;66(5):813-22
Mohamed MMB et al. (2021) De Novo Immunoglobulin A Vasculitis Following Exposure to SARS-CoV-2 Immunization. Ochsner J 21:395-401.
Schönlein JL (1832) General and specific pathology and therapy. Written down from his lectures by some of his listeners and published without authorization. Würzburg, Etlinger
Schönlein JL (1841) Peliosis rheumatica. In: General and special pathology and therapy. 5th edition, St. Gallen, 2 volumes, p. 41
Umemura H et al. (2018) Leukocytoclastic vasculitis associated with immunoglobulin A lambda monoclonal gammopathy of undetermined significance: A case report and review of previously reported cases. J Dermatol 45:1009-1012.