Primary cutaneous CD4⁺ small/medium T‐cell lymphoproliferative disorder in children: A case report and review of the literature

Abstract

Primary cutaneous CD4 + small/medium T-cell lymphoproliferative disorder (PCSM-TCLPD) is a rare benign lymphoproliferative disorder, recently redefined by the 2016 World Health Organization classification of lymphoid neoplasms. In adults, PCSM-TCLPD responds well to monotherapy with surgical excision or local radiation, with or without topical/injected corticosteroids; in contrast, PCSM-TCLPD has only rarely been reported in children, in whom treatments favored in adults may be non-optimal.We present a 14-year-old male with PCSM-TCLPD on the forehead, who achieved complete remission following biopsy, topical corticosteroids, and surgical excision. We also review all literature-reported cases of pediatric PCSM-TCLPD, emphasizing the disorder's benign nature and treatment responsiveness in children.

Piddling and Moderate-Primary Cutaneous CD4+ Small/Medium T Cell Lymphoproliferative Disorder

“…Staging of characteristic lesions of primary cutaneous CD4+ small/medium T cell lymphoproliferative disorder delineating an excellent prognosis is not recommended (Besch-Stokes JG, Costello CM et al, 2022;Plumptre IR, Said JT et al, 2022).…”

“…Primary cutaneous CD4+ small/medium T cell lymphoproliferative disorder commonly appears as a solitary plaque, tumour or nodule situated upon face, neck, upper trunk or thoracic region. Multifocal lesions or tumefaction confined to lower extremities are exceptionally exemplified (Besch-Stokes JG, Costello CM et al, 2022;Plumptre IR, Said JT et al, 2022). Primary cutaneous CD4+ small/medium T cell lymphoproliferative disorder is engendered from cutaneous homing CD4+ T cells demonstrating follicular T helper phenotype.…”

“…Monotypic plasma cells can be enunciated. Occasionally, multinucleated giant cells are observed (Besch-Stokes JG, Costello CM et al, 2022;Plumptre IR, Said JT et al, 2022).…”

“…Frequently, small / medium pleomorphic CD4+ T cells configure the neoplastic infiltrate. Enlarged pleomorphic cells articulate < 30% of neoplastic cellular infiltrate (Besch-Stokes JG,Costello CM et al, 2022;Plumptre IR, Said JT et al, 2022). Tumour cells are variably intermixed with polymorphic cellular component such as miniature CD8+ T cells, B cells, plasma cells and histiocytes.…”

“…Multinucleated giant cells may be discerned. Ki67 proliferation index is minimal and varies from 5% to 20%(Besch-Stokes JG, Costello CM et al, 2022;Plumptre IR, Said JT et al, 2022).…”