Urticaria vasculitis M31.8

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

C1q vasculitis; HUVS; Hypocomplementary urticarial vasculitis syndrome; Hypocomplementary vasculitis; Hypocomplementemic urticarial vasculitis syndrome; urticarial vasculitis; Urticarial vasculitis; urticaria vasculitis; Urticaria vasculitis; Vasculitis hypocomplementämische

History
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McDuffie, 1973

Definition
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Chronic inflammatory disease with recurrent course, clinically characterized by urticarial papules or plaques, sometimes also by deep-reaching itchy or differently painful nodules and angioedema. A distinction is made between 2 forms:

  • Urticarial vasculitis with hypocomplementary vasculitis (HUVS)
  • Urticarial vasculitis with normocomplementary (C1q) vasculitis.

Urticarial vasculitis with hypocomplementaemia (HUVS) is associated up to 50% with lupus erythematosus.

Note: Urticarial vasculitis (UV) is wrongly named because the primary efflorescence is not wheals but a urticarial papule or plaque. The urticarial papule, in contrast to the wheal (persistence of hours), persists for several days!

Occurrence/Epidemiology
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Incidence and prevalence are unknown. Approximately 2-20% of inpatient cases admitted for urticaria suffer from urticarial vasculitis, of which again 20% suffer from HUV. In Germany about 20,000- 50,000 patients are affected (source Charité).

Etiopathogenesis
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It is suspected that autoantibodies against C1q;

Associations are described among others with:

Manifestation
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Women are affected twice as often as men. Illness peak is the 5th decade of life. Hypocomplementaemic urticarial vasculitis is found almost exclusively in women.

Clinical features
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Exanthema accompanied by fever attacks, small spots, maculo-papular, itchy or painful, reminiscent of urticaria. A purple component is usually detectable. Arthralgias and arthritides are frequent, large and small joints are affected. The course is intermittent, possibly with abdominal pain and membranoproliferative glomerulonephritis. Often swelling of the lymph nodes. Patients with hypocomplementary urticarial vasculitis are more prone to systemic complaints (involvement of joints 70%, kidneys 50%, GIT 30%, lungs 20%, eyes 10%). ANA and signs of systemic lupus erythematosus are more frequent in these patients.

Laboratory
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There are no pioneering laboratory constellations. Increase in acute-phase proteins. Hypocomplementation (C3 andC4 significantly reduced) is detectable in about 50% of patients. Often the detection of anti C1q-Ak is successful. The detection of ANA is not uncommon (usually no ENA or antiphospholipid-Ak).

Histology
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  • Inconspicuous surface epithelium. Interstitial edema in the upper and middle dermis. Some patients show signs of leukocytoclastic vasculitis.
  • In the majority of patients superficial and profound interstitial infiltrates of neutrophil granulocytes are predominantly found, mostly (but not obligatory) with clear leukocytoclasia. Not infrequently, the vascularity that is the main feature of classical leukocytoclastic vasculitis is lost. The neutrophil infiltrate is mixed with round cell infiltrates and eosinophilic granulocytes of varying density.

Direct Immunofluorescence
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Granular immunoglobulin and complement deposits in the dermal vessel walls.

Differential diagnosis
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Clinical differential diagnosis:

  • Acute urticaria (efflorescences persist for max. 12 hours; no evidence of leukocytoclastic vasculitis)
  • Sweet syndrome (painful, succulent, papular or plaque skin changes; leukocytosis with neutrophilia)
  • Drug exanthema (anamnesis; otherwise a urticarial exanthema is also possible there)
  • viral exanthema (usually signs of infection are detectable; often general symptoms)

Histological differential diagnoses:

  • Urticaria (no evidence of leukocytoclastic vasculitis)
  • Sweet syndrome (diffuse, neutrophilic, non-vasculitic dermatitis)
  • eosinophilic cellulite (diffuse dermal infiltrate eosinophilic granulocytes; flame figures)
  • Arthropod sting reaction (epidermal puncture site with focal spongiosis; wedge-shaped mixed cell infiltrate with eosinophilic granulocytes of varying density)
  • Erythema anulare centrifugum (focal or absent spongiosis; strictly perivascular infiltrate sheaths with varying admixture of eosinophil granulocytes).

General therapy
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Initially symptomatic therapy. Antihistamines are not very effective. Combinations of H1-blockers with H2-blockers can be tried.

External therapy
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Itch-killing, cooling lotio alba, possibly with addition of 2-5% polidocanol R200.

Alternatively: 1% menthol solution.

Alternative: Gels containing antihistamines (e.g. Soventol, Fenistil, Tavegil; Remark: only moderate success) or mild lotions containing glucocorticoids R123.

Internal therapy
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Glucocorticoids have a beneficial effect in the hypocomplementary form of urticarial vasculitis. Start therapy in medium to high dosages such as prednisolone (e.g. Solu Decortin H) 60-100 mg/day. Reduction according to clinic, maintenance dose preferably below the Cushing's threshold. If resistant to therapy, attempt to reduce glucocorticoid levels by combining with azathioprine (e.g. Imurek) 50-100 mg/day p.o. or mucophenolate mofetil (2.0 g/day p.o.). Cave! Regular laboratory checks are required.

Alternatively: Chloroquine (e.g. Resochin) initial 1-2 Tbl./day, dose reduction according to clinic.

Alternative: DADPS (e.g. Dapson Fatol) as monotherapy 50-100 mg/day or in combination with Pentoxifyllin (e.g. Trental) 2-3 times/day 400 mg or Colchicin (e.g. Colchicum-Dispert Drg.) 0,5-1,5 mg/day can be tried.

Alternative: Successes are also described with systemic interferon alpha therapy(e.g. Roferon) 3 times/week 3 million IU s.c.

Further alternatives:

  • Methotrexate (e.g. MTX) or cyclophosphamide (e.g. endoxane), if necessary in combination with glucocorticoids (see above) or the use of plasmapheresis for complicated systemic manifestations.
  • IVIG (2g/kgKG).
  • In the case of concomitant arthralgia, non-steroidal anti-inflammatory drugs such as indomethacin (e.g. Amuno Kps.) have proven to be effective 2-3 times/day 25 mg in individual doses. No influence on skin symptoms!

Progression/forecast
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Very persistent, chronic recurrent course.

Less frequent are life-threatening courses with signs of systemic vasculitis (kidney, heart, lung, stomach and intestinal involvement and neurological symptoms with pseudotumour cerebri, aseptic meningitis and paresis of central or peripheral nerves).

Note(s)
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The following differently accentuated syndromes are regarded as "special forms" of hypocomplementary urticarial vasculitis:

  • AHA syndrome (arthritis, urticarial vasculitis, angioedema)
  • Schnitzler syndrome
  • Cogan syndrome (urticarial vasculitis, interstitial keratitis, possibly also scleritis, uveitis and hypacusis, tinnitus, dizziness, neurological symptoms such as polyneuropathy, mononeuritis multiplex with encephalitis episodes and myelopathies, CNS vasculitis)
  • Muckle-Wells Syndrome

Literature
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  1. Agnello V, Koffler D, Eisenberg JW, Winchester RJ, Kundel HG (1971) C1g precipitins in the sera of patients with systemic lupus erythematosus and other hypocomplementemic states: characterization of high and low molecular weight types. J Exp Med 134 (Suppl): 228
  2. Cherrez-Ojeda I et al (2019) Autoimmune thyroid disease and urticarial vasculitis: is there a significantassociation
    ? Allergy Asthma Clin Immunol 15:25.

  3. Duschet P et al. (1984) Urticarial vasculitis. Z Hautkr 59: 1427-1434

  4. Fortson J S et al (1986) Hypocomplementemic urticarial vasculitis syndrome responsive to dapsone. J Am Acad Dermatol 15: 1137-1142
  5. Lubach D (1983) The so-called urticaria vasculitis. Allergology 6: 300-303
  6. Mc Duffie FC, Sam's WM Jr, Maldonaldo JE et al (1973) Hypocomplementemia with cutaneous vasculitis and arthritis. Possible immune complex syndrome. Mayo Clin Proc 48: 340-348
  7. Sproßmann A et al (1994) Urticaria vasculitis syndrome in metastatic malignant testicular teratoma. dermatologist 45: 871-874
  8. Wang CC et al (2003) Urticarial vasculitis and dermatomyositis in a patient with nasopharyngeal carcinoma. Cutis 72: 399-402

Disclaimer

Please ask your physician for a reliable diagnosis. This website is only meant as a reference.

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Last updated on: 29.10.2020