Primary cutaneous CD4+ small/medium T-cell lymphoproliferative disorder: Diagnosis and management

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

“…PCSM‐TCLPD, while rare and challenging to diagnose, has an excellent clinical prognosis and response to locally directed therapies, including to topical steroids and surgical excision, as demonstrated in this case 2–4 . To date, PCSM‐TCLPD has been almost entirely reported in adults in the literature 2–4 .…”

“…PCSM-TCLPD, while rare and challenging to diagnose, has an excellent clinical prognosis and response to locally directed therapies, including to topical steroids and surgical excision, as demonstrated in this case. [2][3][4] To date, PCSM-TCLPD has been almost entirely reported in F I G U R E 1 Body map showing locations and sizes of lesions in pediatric patients with primary cutaneous CD4 + small/medium T-cell lymphoproliferative disorder (PCSM-TCLPD) from literature review. See Table 1 for individual-level details of patients.…”

“…The single patient with multifocal lesions is presented in blue adults in the literature. [2][3][4] Among adult cases, systemic involvement is extremely rare, and may indicate an alternative diagnosis given this disorder's typical benign nature; local management with surgical excision or radiation therapy is generally sufficient for cases that do not resolve with biopsy alone. [2][3][4] Rarely pediatric cases have been reported; however, it is not clear if the presentation and prognosis in children is the same as in adult populations, or if optimal treatment strategies should differ for pediatric cases.…”

“…1 PCSM-TCLPD typically responds with a complete remission to locally directed therapy alone, and is most commonly treated by monotherapy with surgical excision or local radiation, with or without topical/injected corticosteroids. [2][3][4] While this rare benign lymphoproliferative disorder has been nearly entirely reported in adults, PCSM-TCLPD has been rarely reported in children, in whom optimal treatment modalities may require different considerations. We report a case of PCSM-TCLPD on the forehead of Abbreviations: CD3, cluster of differentiation 3; CD4, cluster of differentiation 4; MNF116, anti-cytokeratin antibody; PCSM-TCLPD, primary cutaneous CD4 + small/medium T-cell lymphoproliferative disorder.…”

“…Primary cutaneous CD4 + small/medium T‐cell lymphoproliferative disorder (PCSM‐TCLPD) is a rare and heterogenous clinical entity that was downgraded from cutaneous lymphoma by the World Health Organization in 2016, given its excellent clinical prognosis and extremely low rate of progression to extracutaneous disease 1 . PCSM‐TCLPD typically responds with a complete remission to locally directed therapy alone, and is most commonly treated by monotherapy with surgical excision or local radiation, with or without topical/injected corticosteroids 2–4 . While this rare benign lymphoproliferative disorder has been nearly entirely reported in adults, PCSM‐TCLPD has been rarely reported in children, in whom optimal treatment modalities may require different considerations.…”