North Texas Research Forum 2025
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Division
North Texas
Hospital
Medical City Fort Worth
Specialty
Dermatology
Document Type
Presentation
Publication Date
2025
Keywords
lymphoproliferative disorders, cutaneous disorders
Disciplines
Dermatology | Hemic and Lymphatic Diseases | Immune System Diseases | Medicine and Health Sciences
Abstract
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.
Original Publisher
HCA Healthcare Graduate Medical Education
Recommended Citation
do Valle, Rafael; Ayoub, Reem; Hall, Marshall; Lim, Henry; Scheufele, Christian; Furth, Garrett; Carletti, Michael; and Weis, Stepehen E., "Atypical Presentation of CD4+ Small/Medium T-cell Lymphoproliferative Disorder" (2025). North Texas Research Forum 2025. 11.
https://scholarlycommons.hcahealthcare.com/northtexas2025/11
Included in
Dermatology Commons, Hemic and Lymphatic Diseases Commons, Immune System Diseases Commons