A Rare Case of Primary Cutaneous CD4+ Small/Medium Size T-Cell Lymphoproliferative Disorder Responding to Rituximab.

Introduction: Primary cutaneous CD4+ small/medium T-cell lymphoproliferative disorder is a rare lymphoproliferative disorder (PCS-TCLPD) characterized by a solitary erythematous plaque or nodule on the face, neck, or upper trunk with a favorable outcome. Very rarely, multiple plaques or masses may be seen in which precise evaluation should be performed to exclude other primary cutaneous lymphoma.

Case presentation: Here we report a rare case of recalcitrant primary cutaneous small-/medium-sized CD4+ lymphoproliferative disorder in a 55-year-old male patient who responded well to rituximab injection but had recurrence after 1 year from the injections.

Conclusion: Aggressive treatment is avoided in solitary lesions of PCS-TCLPD, but multiple lesions with no response to the conventional modalities may need more sophisticated management, including anti-CD20 rituximab.