Background Psoriasis is a chronic immune-mediated disorder that primarily affects the skin in both adults and children but can also have systemic involvement, particularly with arthritis and kidney injury. IgA nephropathy is the most frequent kidney disorder associated with psoriasis. Approximately one third of all cases of psoriasis begin in childhood, but association between psoriasis and renal disorders has scarcely been reported in pediatric patients. Henoch-Schönlein purpura (HSP) is a systemic vasculitis characterized by IgA deposits in the vessel walls of affected organs and in the mesangium of the kidney. HSP nephritis histopathology is identical to IgA nephropathy. Case report A 6-year-old boy with recent onset of psoriasis developed HSP with kidney involvement, clinically manifested by nephrotic-range proteinuria and hematuria. Kidney biopsy revealed fibrocellular glomerular crescents and mesangial IgA deposits compatible with IgA nephropathy. Treatment with systemic corticosteroids led to the control of hematuria, but as nephrotic-range proteinuria persisted, cyclophosphamide was added, leading to a gradual decrease in proteinuria. Conclusions We propose an underlying common mechanism in the pathogenesis of both HSP and psoriasis, involving a dysregulation of the IgA-mediated immune response, which could predispose to both entities as well as to kidney damage and IgA nephropathy in these patients.
The authors present a clinical report of a previously healthy 4-yearold girl, admitted to a pediatric intensive care unit (PICU) presenting with a fluid-refractory shock. She was initially admitted in the pediatric emergency department with a history of fever and petechial rash and an initial diagnosis of invasive meningococcal disease was placed. During the PICU stay, acute myocarditis with episodes of supraventricular tachycardia and lateral and inferior wall myocardial ischemia, ileitis and serositis were noted. At 14th day of stay, she developed peeling on the hands and feet and, aneurysmatic ectasia of the coronary arteries were visualized in transthoracic echocardiography, allowing the diagnosis of Kawasaki disease shock syndrome (KDSS). The child improved after empirical treatment with antibiotics, intravenous immunoglobulin and corticosteroid therapy. She was started on antiplatelet therapy due to changes in the coronary arteries. Early recognition of KDSS can be challenging; however, a delayed diagnosis increases the risk of coronary changes and a fatal outcome.
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