Justine Kluk1, Anneke Kai1, Dimitra Koch2, Saleem M Taibjee2, Simon O'Connor3, Mojca Persic4, Stephen Morris1, Sean Whittaker1, Lorenzo Cerroni5, Werner Kempf6, Tony Petrella7, Alistair Robson8. 1. Skin Tumour Unit, St John's Institute of Dermatology, London, UK. 2. Department of Dermatology, Dorset County Hospital NHS Foundation Trust, Dorchester, UK. 3. Pathology Department, Nottingham University Hospitals NHS Trust, Nottingham, UK. 4. Department of Oncology, Derby Hospitals NHS Foundation Trust, Derby, UK. 5. Department of Dermatology, Medical University of Graz, Graz, Austria. 6. Department of Dermatology, University Hospital, Zurich, Switzerland. 7. Department of Pathology, University of Dijon, Dijon, France. 8. Dermatopathology Department, St John's Institute of Dermatology, Guy's & St Thomas' NHS Foundation Trust, London, UK.
Abstract
BACKGROUND: Primary cutaneous indolent CD8-positive lymphoid proliferation is an emerging entity characterized by slowly enlarging papules and nodules that are pathologically comprised of clonal nonepidermotropic medium-sized atypical CD8(+) T-cells. Although the majority of lesions are solitary and located on the ears, bilateral symmetrical presentations have been described and lesions may arise at other peripheral or 'acral' sites. Patients follow a benign clinical course and systemic involvement has not yet been observed. Despite this, some medical practitioners classify such lesions as peripheral T-cell lymphoma, NOS, a category implying aggressive disease. OBJECTIVES: We present three cases seen in our institutions and provide an update on a previously reported unique patient who continues to develop recurrent and multifocal skin lesions. RESULTS: Systemic disease progression has not been observed, even in the presence of recurrent and multifocal cutaneous disease. CONCLUSIONS: Indolent CD8-positive lymphoid proliferation of acral sites is a distinctive and readily identifiable entity and should be included in the next consensus revision of cutaneous lymphoma classification. Although cases described thus far have followed an indolent clinical course, dermatologists should remain guarded about the prognosis and full staging and longitudinal observation are recommended until this condition is better understood.
BACKGROUND: Primary cutaneous indolent CD8-positive lymphoid proliferation is an emerging entity characterized by slowly enlarging papules and nodules that are pathologically comprised of clonal nonepidermotropic medium-sized atypical CD8(+) T-cells. Although the majority of lesions are solitary and located on the ears, bilateral symmetrical presentations have been described and lesions may arise at other peripheral or 'acral' sites. Patients follow a benign clinical course and systemic involvement has not yet been observed. Despite this, some medical practitioners classify such lesions as peripheral T-cell lymphoma, NOS, a category implying aggressive disease. OBJECTIVES: We present three cases seen in our institutions and provide an update on a previously reported unique patient who continues to develop recurrent and multifocal skin lesions. RESULTS: Systemic disease progression has not been observed, even in the presence of recurrent and multifocal cutaneous disease. CONCLUSIONS: Indolent CD8-positive lymphoid proliferation of acral sites is a distinctive and readily identifiable entity and should be included in the next consensus revision of cutaneous lymphoma classification. Although cases described thus far have followed an indolent clinical course, dermatologists should remain guarded about the prognosis and full staging and longitudinal observation are recommended until this condition is better understood.