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Indolent CD8-positive lymphoid proliferation of acral sites: three further cases of a rare entity and an update on a unique patient


Kluk, Justine; Kai, Anneke; Koch, Dimitra; Taibjee, Saleem M; O'Connor, Simon; Persic, Mojca; Morris, Stephen; Whittaker, Sean; Cerroni, Lorenzo; Kempf, Werner; Petrella, Tony; Robson, Alistair (2016). Indolent CD8-positive lymphoid proliferation of acral sites: three further cases of a rare entity and an update on a unique patient. Journal of Cutaneous Pathology, 43(2):125-136.

Abstract

BACKGROUND: Primary cutaneous indolent CD8-positive lymphoid proliferation is an emerging entity characterised by slowly enlarging papules and nodules that are pathologically comprised of clonal non-epidermotropic 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.

Abstract

BACKGROUND: Primary cutaneous indolent CD8-positive lymphoid proliferation is an emerging entity characterised by slowly enlarging papules and nodules that are pathologically comprised of clonal non-epidermotropic 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.

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Additional indexing

Item Type:Journal Article, refereed, original work
Communities & Collections:04 Faculty of Medicine > University Hospital Zurich > Dermatology Clinic
Dewey Decimal Classification:610 Medicine & health
Language:English
Date:2016
Deposited On:16 Dec 2015 08:45
Last Modified:05 Apr 2016 19:38
Publisher:Wiley-Blackwell Publishing, Inc.
ISSN:0303-6987
Publisher DOI:https://doi.org/10.1111/cup.12633
PubMed ID:26423705

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