Primary cutaneous CD4+ small or medium T-cell lymphoproliferative disorder: a case report and review of treatment...

Primary cutaneous CD4+ small or medium T-cell lymphoproliferative disorder (PCSMLPD) is a rare indolent cutaneous lymphoid proliferation accounting for approximately 2% of all cutaneous lymphomas. It typically presents as a solitary nodule on the face, neck, or upper trunk and is associated with an excellent prognosis. This review evaluates current treatment options for PCSMLPD and discusses outcomes reported in the literature. Management strategies include watchful waiting, surgical excision, low-dose radiotherapy, topical or intralesional steroids, and, in selected cases, systemic therapies such as methotrexate or doxycycline. Spontaneous regression following diagnostic biopsy has also been documented. Analysis of the largest published series demonstrates that complete surgical excision provides the highest rate of complete remission and the lowest risk of recurrence, making it the preferred first-line treatment for localized disease. Low-dose radiotherapy is highly effective for patients that are unsuitable for surgery, for recurrent lesions, or for lesions on the face, whereas topical or intralesional steroids may achieve remission in selected cases but are associated with a higher likelihood of requiring additional treatment. Relapses are uncommon and generally occur within the first 2 years after diagnosis, supporting regular clinical follow-up during this period. Staging examinations are no longer recommended in patients with a typical clinicopathological presentation. Overall, PCSMLPD has an excellent prognosis, and conservative skin-directed therapies are usually sufficient to achieve durable disease control, with surgical excision remaining the treatment of choice.