This case report represents a rare case of 14-year-old female who diagnosed with hypocompementemic urticarial vasculitis syndrome that presents with glomerulonephritis, diffuse alveolar hemorrhage, and acute disseminated encephalomyelitis. The progression of the symptoms explained in the text below in which the final diagnosis was reached after a challenging approach. Patient was managed properly and followed up after treating with rituximab, although she represents no sign of the disease after a total of two cycles.
Hoang MP, Park J. Vasculitis. In: Hoang MP, Selim MA, editors. Hospital-based dermatopathology: An illustrated diagnostic guide. Springer International Publishing; 2020. https://doi.org/10.1007/978-3-... PMCid:PMC7120156.
Ueki K, Tsuchimoto A, Matsukuma Y, et al. Hypocomplementemic urticarial vasculitis syndrome with gastrointestinal vasculitis and crescentic membranoproliferative glomerulonephritis without immune complex deposits. CEN Case Rep. 2020;9(1):30-5. https://doi.org/10.1007/s13730... PMid:31586313 PMCid:PMC6990357.
Jachiet M, Flageul B, Bouaziz JD, Bagot M, Terrier B, Groupe Français d’Étude des Vascularites (GFEV). Les vascularites urticariennes hypocomplémentémiques [Hypocomplementemic urticarial vasculitis]. Rev Med Interne. 2018;39(2):90-8. https://doi.org/10.1016/j.revm... PMid:28457680.
Buck A, Christensen J, McCarty M. Hypocomplementemic urticarial vasculitis syndrome: A case report and literature review. J Clin Aesthet Dermatol. 2012;5(1):36-46.
Sjöwall C, Skattum L, Olsson M, Nilsson A, Mohammad AJ. [Hypocomplementemic urticarial vasculitis syndrome: A rare but not always benign condition]. Lakartidningen. 2019;116:FR49.
Simmons SC, Smith ML, Chang-Miller A, KeddisMT. Antinuclear antibody-negative lupus nephritis with full house nephropathy: A case report and review of the literature. Am J Nephrol. 2015;42(6):451-9. https://doi.org/10.1159/000443... PMid:26812129.
Dagel T, Meram E, Onal E, Erbil D, Bulbul M, Uysal S. Rare case: 70 year old patient with seronegative lupus nephritis. Medeni Medical J. 2018;33(1):54-6. https://doi.org/10.5222/MMJ.20....
Mirouse A, Parrot A, Audigier V, et al. Severe diffuse alveolar hemorrhage related to autoimmune disease: A multicenter study. Crit Care. 2020;24:231. https://doi.org/10.1186/s13054... PMid:32423434 PMCid:PMC7236262.
Di Pietro GM, Castellazzi ML, Mastrangelo A, Montini G, Marchisio P, Tagliabue C. Henoch-Schönlein Purpura in children: Not only kidney but also lung. Pediatr Rheumatol Online J. 2019;17(1):75. https://doi.org/10.1186/s12969... PMid:31752918 PMCid:PMC6873759.
Sun Y, Zhou C, Zhao J, et al. Systemic lupus erythematosus-associated diffuse alveolar hemorrhage: A single-center, matched case-control study in China. Lupus. 2020;29(7):795-803. https://doi.org/10.1177/096120... PMid:32321345.
Bermejo PE, Ruiz A, BeisteguiM, Zabala JA, Escamilla C. Hemorrhagic acute disseminated encephalomyelitis as first manifestation of systemic lupus erythematosus. J Neurol. 2008;255(8):1256-8. https://doi.org/10.1007/s00415... PMid:18425695.
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