Vasculitis leukocytoclastic (non-iga-associated) D69.0; M31.0

Authors: Prof. Dr. med. Peter Altmeyer, Konrad Heisterkamp

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Last updated on: 29.10.2020

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Synonym(s)

allergic arteriolitis cutis; anaphylactoids purpura; arteriolitis hyperergica cutis; Arteritis allergica cutis; Cutaneous leukocytoclastic vasculitis; Cutaneous small vessel vasculitis; Gougerot symptom; Hyperergic vasculitis; IgA negative immune complex vasculitis; IgA negative vasculitis; IgG-/IgM-positive immune complex vasculitis; Immunocomplex vasculitis; leukocytoclastic vasculitis; necrotizing vasculitis; Non-IgA Vasculitis; purpura rheumatica; vasculitis allergica; Vasculitis hyperergic; Vasculitis necrotizing

History
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Heberden, 1802; Schönlein, 1832; Henoch, 1868

Definition
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Inflammation of small vessels (small vessel vasculitis), which is caused by deposition of circulating immune complexes or bacterial endotoxins in vessel walls with subsequent complement activation and is histologically presented as leukocytic vasculitis of post-capillary venules with fibrinoid swelling of the vessel wall and/or thrombi in the lumina (the synonyms usually describe only partial aspects). In contrast to the purpura Schönlein-Henoch, primary IgG/IgM vasculitis does not detect IgA immune complexes in the vessel walls.

Classification
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It seems to become apparent that a classification of IgA-negative leukocytoclastic vasculitis into:

  • LcV with systemic involvementgg
  • LcV without systemic involvement (cutaneous LcV).

Both forms have an analogous etiology.

Occurrence/Epidemiology
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The classical cutaneous immune complex vasculitis (including the "IgA-associated vasculitides e.g. the Purpura Schönlein) is a disease frequently occurring in large dermatological collectives. Its incidence is estimated at 10-20/100,000 per year.

Etiopathogenesis
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Circulating immune complexes can be detected in the majority of patients. They are of great etiopathological importance.

Manifestation
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Occurrence both in adulthood (average 39.-49. LJ) and in childhood. Women are 2-3 times more frequently affected than men.

Localization
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Due to stasis phenomena, the lower legs are preferentially affected; in the case of higher acuity, also on the thighs or on the trunk at fixed clothing contact points. The probability of systemic involvement increases as soon as the skin symptoms appear above the belt line.

Clinical features
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The clinical picture corresponds to an acute chronic inflammatory systemic disease with varying organ involvement. The clinical and dermatological leading symptom of "small vessel vasculitis of the leukocytoclastic type" is a " purpura". The clinical picture depends, among other things, on the stage of development, the acuteity of the disease and the extent of organ manifestation (monorganic or polyorganic in about 50% of patients).

At the beginning there are often vague general symptoms with fever, uncharacteristic rheumatic complaints with (poly)arthralgia, arthritis or myalgia, more rarely myositis. The characteristic clinical-dermatological picture of leukocytoclastic vasculitis is the purpura with lesions between 0.1 cm and several centimetres. Depending on the acuteity and the stage of development of the vasculitis, the purpura impresses by haemorrhagic spots or papules (palpable purpura), accompanied by itching, pain or burning. In further stages of development, vesicles or blisters, hemorrhagic plaques and secondary pustules, erosions or ulcers may develop.

Laboratory
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Acute-phase reactions (high BSG, CRP, leuko- and thrombocytosis) go parallel with the acuteity of vasculitis. Circulating immune complexes (lowered complement: CH50,C3, Ced,C4), possibly pathological urine sediment (the urine status should be done at least 3 times in a row), blood in stool (the haemocult test should be done at least 3 times in a row) . Activity parameters are the soluble sIl-2 and factor VIII-associated antigen (extent of endothelial damage). In addition: smear test of the pharynx and tonsils. There is no diagnostic evidence of any parameter in the laboratory. In addition, immunological and molecular biological techniques should be used to exclude hepatitis C/B. Possible autoantibodies such as ANCA and ANA are both activity and diagnosis associated.

Histology
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In early stages edema of the papillary dermis. Sparse, superficial, intramural and sleeve-shaped perivascularly oriented inflammatory infiltrate of lymphocytes, histiocytes and apoptotically decomposing neutrophil leukocytes (leukocytoclasia, nuclear dust) and, to varying degrees, eosinophilic leukocytes. Endothelial cells appear epitheloid swollen and protruding into the lumen. Fibrin is found in the vessel wall of post-capillary venules. At the same time there are perivascular accentuated erythrocyte extravasations of varying density. The exudation can lead to subepidermal blistering or even pustular formation. Vascular occlusion clinically results in a grey shade in the centre of the lesion and, depending on its extent, can lead to tissue necrosis, recognisable by spongiosis and fading keratinocytes. There seems to be a correlation between tissue eosinophilia and drug-induced vasculitis.

The following algorithm can be schematized:

Histopathology of leukocytoclastic vasculitis (lowest common denominator:italics, leading symptoms:bold) varies according to Ratzinger et al. 2105
Accentuated around post-capillary venules
Capillaries recessed
perivascular leukocytoclasia
Damage to endothelial cells
Fibrin in/around vessel walls
Perivascular extravasation of erythrocytes
Edema in the papillary dermis
Collagen degeneration
Variable number of eosinophils
No plasma cells or fibrosclerosis

Direct Immunofluorescence
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Perivascular IgG and/or IgM deposits (in contrast to the Purpura Schönlein-Henoch in which perivascular IgA deposits occur)

Diagnosis
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Organ diagnostics: Exclusion of kidney involvement (careful analysis of the urine status); sonogram with determination of the organ size; attention to neurological symptoms (if necessary cranial magnetic resonance tomogram); ENT consultation and ophthalmological consultation.

Therapy
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For all severe leukocytoclastic vasculitis that do not show a self-limiting course:

  • Predominant or exclusive affection of the skin: glucocorticoids ( prednisone, 60-100 mg/day) as monotherapy, in case of resistance to therapy in combination with azathioprine (100-150 mg/day), reduction after clinical treatment to the lowest possible maintenance dose.
  • Alternatively, if resistant to therapy, high-dose IVIG therapy(e.g. Intratect) 0.5-1.0 g/kg bw every 28 days i.v.
  • System involvement: glucocorticoids (prednisone 60-150 mg/day), possibly in combination with cyclophosphamide (100 mg/day), possibly cyclophosphamide as shock therapy (1 g as short infusion + 3 l liquid/day + mesna (uromitexane) 200 mg i.v. or orally. A total of 3 cycles in 2-4 weeks intervals).
  • In severe cases, plasmapheresis may be added.

External therapy
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Symptomatic.

Progression/forecast
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I.A.'s prognosis is favourable. It depends on the acuteity and extent of internal organ involvement. In larger statistics, deaths have been reported in 2-3% of patients. Chronic recurrent course may indicate cryoglobulinemia or a persistent infection (e.g. hepatitis C).

Note(s)
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To distinguish it from the purpura Schönlein-Henoch (PSH):
  • Absence of IgA deposits
  • Age > 20 years
  • Occurrence of eosinophilic leukocytes in the infiltrate
  • Lower involvement of the intestinal tract.

Literature
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  1. Albrecht J et al (1999) Henoch-Schonlein purpura: successful treatment with Dapsone. dermatologist 50: 809-811
  2. Egan CA et al (2000) Relapsing Henoch-Schonlein purpura associated with Pseudomonas aeruginosa pyelonephritis. J Am Acad Dermatol 42: 381-383
  3. Fervenza FC (2003) Enoch-Schonlein purpura nephritis. Int J Dermatol 42: 170-177
  4. Fritsch PO (1991) Necrotizing vasculitis. dermatologist 42: 599-604; 661-670; 729-738
  5. Galaria NA et al (2002) Henoch-Schonlein purpura secondary to subacute bacterial endocarditis. Cutis 69: 269-273
  6. Heberden W (1802) De purpureis maculis. In: Commentaries on the History and Cure of Diseases. chapter 78, T. Payne (ed.), London
  7. Hengge UR et al (2002) Purpura fulminans. A fatal consequence of a widely used medication? dermatologist 53: 483-487
  8. Enoch E (1868) On the connection between purpura and intestinal disorders. Berlin clinical weekly 5: 517-519
  9. Enoch E (1874) On a peculiar form of purpura. Berlin clinical weekly 11: 641
  10. 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
  11. Loricera J et al (2015) Single-organ cutaneous small-vessel vasculitis according to the 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides: a study of 60 patients from a series of 766 cutaneous vasculitis cases. Rheumatology (Oxford) 54: 77-82.
  12. Ratzinger G et al (2015) The Vasculitis Wheel-an algorithmic approach to cutaneous vasculitides. JDDG 1092-1118
  13. Ronkainen J et al (2002) The adult kidney 24 years after childhood Henoch-Schonlein purpura: a retrospective cohort study. Lancet 360: 666-670
  14. Sam's World Cup (1980) Necrotizing Vasculitis. J Am Acad Dermatol 3: 1-13
  15. Schönlein JL (1832) General and special pathology and therapy. Written down after his lectures by some of his listeners and published without authorization. Etlinger, Würzburg
  16. Singh H et al (2016) Vasculitis as a Presenting Manifestation of Chronic Hepatitis B Virus Infection: A Case Report. J Clin Diagn Res 10: OD25-26.

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Last updated on: 29.10.2020