Rafael do Valle Reem Ayoub Marshall Hall Henry Lim Christian Scheufele Garrett Furth Michael Carletti Stepehen E. Weis
UNTHSC HCA Healthcare
01-01-2025
Introduction: Primary cutaneous CD4+ small/medium T-cell lymphoproliferative disorder (PCSM-LPD) is a rare cutaneous disorder that until 2016, was classified as a primary cutaneous lymphoma. PCSM-LPD..
Introduction: Primary cutaneous CD4+ small/medium T-cell lymphoproliferative disorder (PCSM-LPD) is a rare cutaneous disorder that until 2016, was classified as a primary cutaneous lymphoma. PCSM-LPD histopathologically consists of a localized proliferation of small to medium-sized T lymphocytes. Clinically there is minimal to no systemic involvement. It typically presents as an asymptomatic, solitary red-purple nodule, papule, or plaque on the face. PCSM-LPD has an indolent course and an excellent prognosis. It is important to recognize PCSM-LPD as a distinct entity from primary cutaneous lymphoma to avoid treatments with side effects. Case Presentation: A 68-year-old female with a history of insulin-dependent type 2 diabetes mellitus complicated by diabetic neuropathy, major depressive disorder, gastroesophageal reflux, and essential hypertension presented with a two-month history of progressively increasing, small, itchy bumps on the right cheek. On examination, there were scattered, non-tender, pink, non-scaly papules limited to her right cheek. A punch biopsy revealed a nodular infiltrate of small regular appearing CD4+/CD3+ lymphocytes with a T-cell receptor gamma gene arrangement, most consistent with PCSM-LPD. At follow-up, it was noted that her lesions had improved around the biopsy site. She was subsequently treated with intralesional triamcinolone injections and is currently pending follow-up. Learning Points: PCSM-LPD is a rare cutaneous disease with an undetermined malignant potential. Despite its uncertain malignant potential, PCSM-LPD typically follows a benign course with an excellent prognosis. PCSM-LPD most commonly presents as a solitary asymptomatic lesion. However, in some cases, multiple lesions may appear. In other instances, they can be pruritic. Due to its routinely benign course aggressive treatment or extensive diagnostic work-up is not recommended. PCSM-LPD is an indolent disease with no long-term risk of secondary lymphomas. For this reason, imaging modalities and bone marrow evaluations are low-yield. Conservative management including local treatment modalities and clinical observation can be used with a high degree of success and should be considered before invasive or systemic treatments.