Primary cutaneous CD4+ small/medium T-cell lymphoproliferative disorders: demographic, clinical, therapeutic and prognostic aspects. A retrospective monocentric analysis.

Abstract

Primary cutaneous CD4+ small/medium T-cell lymphoproliferative disorders (PCSM-LPDs), represent a rare group of haematological diseases primarily affecting the skin. In this retrospective single-centre case series study, we aimed to investigate the demographic, clinical, therapeutic and prognostic aspects of PCSM-LPD. We collected data from cases diagnosed between 2010 and the present, employing histopathological and immunohistochemical methods following the World Health Organization criteria. We included 22 patients with a median age of 61.5 years and a median time between clinical onset and diagnosis of 3.0 months. Surgical excision with conservative margins was the primary choice, showing clinical remission in 17 cases, while nonsurgical treatments, including radiotherapy, high-potency steroids and ablative laser, achieved clinical remission in four cases. Clinical presentations varied, but the most common was a single violaceous nodule or papule on the upper body parts. In conclusion, our single-centre case series provides valuable insights into PCSM-LPD, highlighting the effectiveness of surgical treatments and the potential of nonsurgical ones. Even if controversial, the benign nature of PCSM-LPD emphasizes the importance of achieving tumour clearance with acceptable aesthetic outcomes.