Recurrent lymphomatoid papulosis associated with nephrotic syndrome. An occurrence of uncertain origin

Abstract

We report on a 10-year-old child with nephrotic syndrome who developed recurrent lymphomatoid papulosis (LYP) 60 months after the first episode of proteinuria. LYP appeared when the child was taking low-dose cyclosporin and disappeared after the drug was replaced by prednisone at doses utilized for nephrotic syndrome (2 mg/kg). During the tapering of steroids, when the child was treated with low-dose prednisone (0.2 mg/kg), both LYP and nephrotic syndrome started again and required the reintroduction of prednisone to restore a normal clinical situation. This is the first case of LYP occurring in concomitance and synchronous with nephrotic syndrome. LYP was unrelated to cyclosporin (second episode after its withdrawn) but preceded the recurrence of proteinuria, suggesting a relationship with the disease activity. Even though the etiology of LYP is, in this case, uncertain, it should be considered as a clinical association of nephrotic syndrome in children and also included among potential triggers of the disease.

Clinical, histopathologic, and immunophenotypic features of lymphomatoid papulosis with CD8 predominance in 14 pediatric patients

“…Previous studies of childhood LyP reported a younger mean age of onset (%9 years), a similar presentation with cyclic papulonodular eruptions, and variable response to treatment. 2,4,5,[7][8][9][10][11] The histopathologic features showed a predominance of type A LyP with a polymorphous infiltrate, with or without eosinophils and large atypical CD30 1 lymphocytes, similar to features in adults. 5,6 Previous reports of TCRGR studies showed positive results in two cases without association with lymphoproliferative disorders.…”

“…Only a few previous reports have detailed the clinical and laboratory features of LyP in pediatric patients. [4][5][6][7][8][9][10] The most common histopathologic subtype described for adult-onset and pediatric-onset LyP is type A, which is characterized by large, atypical, CD30 1 lymphocytes, resembling Reed-Sternberg cells of Hodgkin lymphoma, present in a wedge-shaped distribution throughout the dermis and admixed with various numbers of inflammatory cells. Fewer cases are classified as type B, which is characterized by small, CD30 e lymphocytic cells with cerebriform nuclei in a bandlike pattern, along with epidermotropism.…”

Minimal Change Nephrotic Syndrome Associated With Non-Hodgkin Lymphoid Disorders

“…29 A small number of case reports have suggested that MCNS may be associated with several subtypes of NHL; however, this association has not been studied in depth. 7 , 10 , 13 , 17 , 20 , 21 , 31 These case reports highlight a close relationship between the progression of NHL and MCNS, suggesting that MCNS may be considered as a paraneoplastic glomerulonephritis in the context of NHL. 8 , 19 , 22 , 28 …”