title: Indolent CD8-positive lymphoid proliferation of acral sites: three further cases of a rare entity and an update on a unique patient.
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.
  
  
  OBJECTIVE
  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.
authors.affiliation: Skin Tumour Unit, St John's Institute of Dermatology, London, UK.
authors.forename: Justine
authors.initials: J
authors.lastname: Kluk
authors.affiliation: Skin Tumour Unit, St John's Institute of Dermatology, London, UK.
authors.forename: Anneke
authors.initials: A
authors.lastname: Kai
authors.affiliation: Department of Dermatology, Dorset County Hospital NHS Foundation Trust, Dorchester, UK.
authors.forename: Dimitra
authors.initials: D
authors.lastname: Koch
authors.affiliation: Department of Dermatology, Dorset County Hospital NHS Foundation Trust, Dorchester, UK.
authors.forename: Saleem M
authors.initials: SM
authors.lastname: Taibjee
authors.affiliation: Pathology Department, Nottingham University Hospitals NHS Trust, Nottingham, UK.
authors.forename: Simon
authors.initials: S
authors.lastname: O'Connor
authors.affiliation: Department of Oncology, Derby Hospitals NHS Foundation Trust, Derby, UK.
authors.forename: Mojca
authors.initials: M
authors.lastname: Persic
authors.affiliation: Skin Tumour Unit, St John's Institute of Dermatology, London, UK.
authors.forename: Stephen
authors.initials: S
authors.lastname: Morris
authors.affiliation: Skin Tumour Unit, St John's Institute of Dermatology, London, UK.
authors.forename: Sean
authors.initials: S
authors.lastname: Whittaker
authors.affiliation: Department of Dermatology, Medical University of Graz, Graz, Austria.
authors.forename: Lorenzo
authors.initials: L
authors.lastname: Cerroni
authors.affiliation: Department of Dermatology, University Hospital, Zurich, Switzerland.
authors.forename: Werner
authors.initials: W
authors.lastname: Kempf
authors.affiliation: Department of Pathology, University of Dijon, Dijon, France.
authors.forename: Tony
authors.initials: T
authors.lastname: Petrella
authors.affiliation: Dermatopathology Department, St John's Institute of Dermatology, Guy's & St Thomas' NHS Foundation Trust, London, UK.
authors.forename: Alistair
authors.initials: A
authors.lastname: Robson
mesh_terms: D000328:Adult; D018414:CD8-Positive T-Lymphocytes / Q000378:metabolism / Q000473:pathology; D049109:Cell Proliferation; D005260:Female; D006801:Humans; D016410:Lymphoma, T-Cell, Cutaneous / Q000378:metabolism / Q000473:pathology; D016411:Lymphoma, T-Cell, Peripheral / Q000378:metabolism / Q000473:pathology; D008297:Male; D008875:Middle Aged; D012878:Skin Neoplasms / Q000378:metabolism / Q000473:pathology
keywords: CD8; acral; indolent; lymphoid proliferation; lymphoma