Primary cutaneous CD4+ small/medium T-cell lymphoproliferative disorder (PCS-TCLPD) is a rare disease with no clear diagnostic and treatment guidelines [1]. According to the WHO classification of hematopoietic neoplasms, this is an indolent T-cell lymphoproliferative disorder confined to the skin, with a characteristic population of T cells with a follicular T-helper phenotype [2]. So far, this poorly defined disease has an undetermined malignant potential [3].
We present a case report of a 46-years-old Caucasian male who presented with a flat circular erythematous skin lesion on his forehead (fig. 1). The lesion was excised and histopathology revealed a skin covered with epithelium without atypia, massive lymphocytic infiltration extending into the subcutaneous tissue. There was perivascular infiltration and infiltration of skin appendages; CD3+ T cells predominate the lesion; CD4+ significantly predominate over CD8–/+, CD30–. The image most closely matched PCS-TCLPD.
PCS-TCLPD has no long-term risk of secondary lymphomas and an excellent prognosis. It has an indolent clinical behavior with a 5-year survival rate of 100% [1, 2]. Imaging modalities and bone marrow evaluations are of a relatively low diagnostic value and are not mandatory [1]. Local surgical treatment can be used with a high degree of success and should be considered before other options [1]. In summary, PCS-TCLPD is a rare disease, usually presenting as a plaque or nodule in the head and neck region and can be treated successfully by simple surgical excision with clear margins [3].
