Primary Cutaneous CD4 + Small/Medium Pleomorphic T-Cell Lymphoproliferative Disorder: A Case Series.

Background: Primary cutaneous CD4 ⁺ small/medium T-cell lymphoproliferative disorder (CD4 ⁺ PCSM-LPD) is defined by a predominance of small- to medium-sized CD4 ⁺ pleomorphic T cells and a favorable clinical course.
Objective: We performed a retrospective analysis of 6 patients with CD4 ⁺ PCSM-LPD and reviewed the literature to address questions about its diagnosis, treatment, and prognosis.
Methods: Patients were 3 men and 3 women with a median age of 50 years. All patients presented with a single erythematous nodule, localised on the head in 4 patients and the upper trunk in 2 cases. No patients showed extracutaneous disease at any evaluation. Histopathologic features were characterised by nodular, diffuse, or, in 1 case, a superficial dense infiltrate of small/medium-sized pleomorphic CD4 ⁺ /PD1 ⁺ T lymphocytes. T-cell receptor clonality was demonstrated in 5 cases. Treatment was surgical excision in 5 cases and radiotherapy in 1 case.
Results: All patients achieved complete resolution without relapses, during a median follow-up of 3 years. A review of the literature confirmed that CD4 ⁺ PCSM-LPD presents predominantly with a solitary nodular lesion on the face, neck, or upper trunk in adult patients. Surgical excision is the preferred treatment. Spontaneous resolution after biopsy may occur.
Conclusions: CD4 ⁺ PCSM-LPD is a rare disorder with a favorable course.