Familial partial deficiency of the third component of complement (C3) and the hypocomplementemic cutaneous vasculitis syndrome

McLean, Robert H.; Chapitis, Jane; Weinstein, Arthur; Lowenstein, Mitchell B.; Rothfield, Naomi F.

doi:10.1016/0002-9343(80)90305-8

Cited by 40 publications

(11 citation statements)

References 36 publications

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“…The term 'urticarial vasculitis' refers to patients with recurrent urticaria and the histological features of necrotising vasculitis. Several papers have described the clinical and immunopathological features (McDuffie et al, 1973;Soter, Austen & Gigli, I974;Agnello et al, I975;Agnello, Gabriel & Tai, 1976;Feig et ai, 1976;Gammon & Wheeler, i979;Ludivico, Myers & Maver, 1979;McLean et al, 1980;Millns et ai, 1980;Phanuphak et ai, 1980;Zeiss et ai, t98o;Van der Horst & Bronsveld, 1981;Callen & Kalbfleisch, 1982), but the group of sixteen patients studied by Soter (1977) is the largest series in the literature. The syndrome affects predominantly females, and it can be distinguished from ordinary urticaria since the individual lesions last longer and often fade to leave purpuric staining or other evidence of red cell extravasation.…”

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confidence: 99%

Urticaria and vasculitis: a continuum of histological and immunopathological changes

et al. 1983

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Histopathological criteria were used to classify twenty-four patients with chronic urticaria into three groups, which were then studied to establish whether circulating immune complexes (CICs), complement activation and deposition of immunoreactants are confined to patients with urticarial vasculitis. Group I (three patients) had classical urticarial vasculitis, and two of these patients showed hypocomplementaemia with evidence of C3 conversion and deposition of immunoreactants in lesional and uninvolved skin. Ten patients (group 2) with a dense perivascular mixed-cellular infiltrate had normal or raised complement levels and infrequent evidence of C3 conversion. Immunoreactants were detected only in their lesional skin. Eleven patients (group 3) had only a sparse perivascular infiltrate. In this group, complement was normal and immunofluorescence was essentially negative. Cryoglobulins were detected in group I patients only. Monoclonal rheumatoid factor and C1q binding were positive in all group I patients, half the group 2 patients and none of the group 3 patients. This study suggests that urticaria and urticarial vasculitis form a disease continuum, and identifies a group of patients with features intermediate between urticarial vasculitis and ordinary urticaria.

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confidence: 99%

Urticaria and vasculitis: a continuum of histological and immunopathological changes

et al. 1983

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“…Two families are summarized in Table 1 in which partial FH deficiency occurs with IgA nephropathy. 51 - 52 There is one instance of IgA nephropathy in a patient with homozygous C3 deficiency. 42 No disease has been associated with heterozygous Fl deficiency.…”

Section: Discussionmentioning

confidence: 99%

Hereditary complement factor I deficiency

Vyse

Davies³

et al. 1994

QJM: An International Journal of Medicine

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QJM SummaryWe describe four cases (from three families) of hereditary factor I deficiency, bringing the total number of cases now reported to 23. In one family there are two affected siblings: one has suffered recurrent pyogenic infections; the other is asymptomatic. In the second family, the patient had recurrent pyogenic infections and a self-limiting vasculitic illness; in the third family, the patient suffered recurrent pyogenic and neisserial infections. All four patients had markedly reduced concentrations of C3 in the serum (family 1 propositus: 28%; family 1 asymptomatic sibling: 15%; family 2: 31%; and family 3: 31 % normal human serum) which was in the form of C3b. Low lgG 2 levels may occur in primary C3 deficiency, and a reduction in lgG 2 concentration to 1.14 g/l (normal: 1.30-5.90 g/l) was found in the patient from family 2. Using radioligand binding assays, we demonstrated increased binding of C3b to erythrocytes in a patient with factor I deficiency. This C3b could not be cleaved by autologous serum but could be cleaved by normal serum or purified factor I. We review and compare the published cases of C3, factor H and factor I deficiency.

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“…C1 inhibitor levels are normal. A patient with familial partial deficiency of C3 has been reported (82). Two patients with C3 nephritic factor activity developed urticarial vasculitis (83,84).…”

Section: Laboratory Findingsmentioning

confidence: 99%

Urticarial venulitis

Soter

2000

Dermatologic Therapy

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Episodes of recurrent urticaria and angioedema may be a clinical manifestation of cutaneous necrotizing venulitis. Known as urticarial vasculitis, this edematous form of necrotizing venulitis occurs in patients with serum sickness, connective tissue disorders, hematologic malignant conditions, an IgMKM component, infections, or physical urticarias after the administration of therapeutic agents, and as an idiopathic disorder. The skin lesions appear as erythematous, occasionally indurated wheals which may contain foci of purpura. Although the individual urticarial lesions may last for less than 24 hours, they often persist up to 3–5 days. The episodes of urticaria are chronic; approximately 70% of the afflicted individuals are women. General features include fever, malaise, and myalgia. Specific organ involvement includes enlargement of the lymph nodes, liver, and spleen and involvement of the synovia, kidneys, gastrointestinal tract, respiratory tract, eyes, and central nervous system. In the treatment of urticarial vasculitis there are no double‐blind, placebo‐controlled trials. Reports exist of the treatment of patients with H1 antihistamines, nonsteroidal anti‐inflammatory agents, colchicine, hydroxychloroquine, dapsone, prednisone, azathioprine, methotrexate, cyclophosphamide, and intramuscular gold therapy.

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Familial partial deficiency of the third component of complement (C3) and the hypocomplementemic cutaneous vasculitis syndrome

Cited by 40 publications

References 36 publications

Urticaria and vasculitis: a continuum of histological and immunopathological changes

Urticaria and vasculitis: a continuum of histological and immunopathological changes

Hereditary complement factor I deficiency

Urticarial venulitis

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