Autoimmune markers and vascular immune deposits in Finkelstein-Seidlmayer vasculitis: Systematic literature review. Issue 136 (April 2023)

• Record Type: Journal Article
• Authors: Bronz, Gabriel; Gianini, Jvan; Passi, Alberto G.; Rizzi, Mattia; Bergmann, Marcel M.; Milani, Gregorio P.; Lava, Sebastiano A.G.; Bianchetti, Mario G.; Terziroli Beretta-Piccoli, Benedetta; Vanoni, Federica
• Is Part Of: Journal of autoimmunity. Issue 136(2023)
• Publication Date: 2023-04
• Subjects: Finkelstein-seidlmayer vasculitis -- Acute hemorrhagic edema BIbliographic database -- Rheumatoid factor -- Anti-nuclear antibodies -- Immunoglobulin A; Autoimmunity -- Periodicals; Autoimmune diseases -- Periodicals; Autoantibodies -- Periodicals; Autoimmune Diseases -- Periodicals; Auto-immunité -- Périodiques; Maladies auto-immunes -- Périodiques; Electronic journals; 616.978005
• DOI: 10.1016/j.jaut.2023.103002
• Languages: English
• ISSNs: 0896-8411

Abstract

Abstract: Finkelstein-Seidlmayer vasculitis, also called acute hemorrhagic edema of young children or infantile immunoglobulin A vasculitis, is habitually a benign skin-limited small vessel leukocytoclastic vasculitis that mainly affects infants 24 months or less of age. Since this disease is commonly triggered by an infection, an immune-mediated origin has been postulated. To better appreciate the possible underlying immune mechanism of this vasculitis, we addressed circulating autoimmune markers and vascular immune deposits in patients contained in the Acute Hemorrhagic Edema BIbliographic Database, which incorporates all original reports on Finkelstein-Seidlmayer vasculitis. A test for at least one circulating autoimmune marker or a vascular immune deposit was performed in 243 cases. Subunits of complement system C4 resulted pathologically reduced in 4.7% and C3 in 1.4%, rheumatoid factor was detected in 6.1%, and antinuclear antibodies in 1.9% of cases. Antineutrophil cytoplasmic antibodies were never demonstrated. Immunofluorescence studies were performed on 125 skin biopsy specimens and resulted positive for complement subunits in 46%, fibrinogen in 45%, immunoglobulin A in 25%, immunoglobulin M in 24%, immunoglobulin G in 13%, and immunoglobulin E in 4.2% of cases. Infants testing positive for vascular immunoglobulin A deposits did not present a higher prevalence of systemic involvement or recurrences, nor a longer disease duration. In conclusion, we detected a very low prevalence of circulating autoimmune marker positivity in Finkelstein-Seidlmayer patients. Available immunofluorescence data support the notion that immune factors play a relevant role in this vasculitis. Furthermore, vascular immunoglobulin A deposits seem not to play a crucial role in this disease. Highlights: Finkelstein-Seidlmayer vasculitis usually appears after an infection or vaccination. The Acute Hemorrhagic Edema BIbliographic Database includes all 586 reported cases. Subunits of complement system C4 is reduced in 4.7%, and of C3 in 1.4% of the cases. Rheumatoid factor positive in 6.1%; antinuclear antibodies in 1.9% of cases. Immunofluorescence of 125 skin biopsies: positive for immunoglobulin A in 25%.