title: Primary cutaneous aggressive epidermotropic cytotoxic T-cell lymphomas: reappraisal of a provisional entity in the 2016 WHO classification of cutaneous lymphomas.
abstract: Primary cutaneous CD8-positive aggressive epidermotropic T-cell lymphoma is a rare and poorly characterized variant of cutaneous lymphoma still considered a provisional entity in the latest 2016 World Health Organization Classification of Cutaneous lymphomas. We sought to better characterize and provide diagnostic and therapeutic guidance of this rare cutaneous lymphoma. Thirty-four patients with a median age of 77 years (range 19-89 years) presented primarily with extensive annular necrotic plaques or tumor lesions with frequent mucous membrane involvement. The 5-year survival was 32% with a median survival of 12 months. A subset of 17 patients had a prodrome of chronic patches prior to the development of aggressive ulcerative lesions. We identified cases with lack of CD8 or αβ T-cell receptor expression yet with similar clinical and pathological presentation. Allogeneic stem cell transplantation provided partial or complete remissions in 5/6 patients. We recommend the term primary cutaneous aggressive epidermotropic cytotoxic T-cell lymphoma as this more broad designation better describes this clinical-pathologic presentation, which allows the inclusion of cases with CD8 negative and/or αβ/γδ T-cell receptor chain double-positive or double-negative expression. We have identified early skin signs of chronic patch/plaque lesions that are often misdiagnosed as eczema, psoriasis, or mycosis fungoides. Our experience confirms the poor prognosis of this entity and highlights the inefficacy of our standard therapies with the exception of allogeneic stem cell transplantation in selected cases.
authors.affiliation: Department of Dermatology and Pathology, Northwestern University Feinberg Medical School, Chicago, IL, USA.
authors.forename: Joan
authors.initials: J
authors.lastname: Guitart
authors.affiliation: Department of Dermatology and Pathology, Northwestern University Feinberg Medical School, Chicago, IL, USA.
authors.forename: M Estela
authors.initials: ME
authors.lastname: Martinez-Escala
authors.affiliation: Department of Dermatology and Pathology, Yale University, New Haven, CT, USA.
authors.forename: Antonio
authors.initials: A
authors.lastname: Subtil
authors.affiliation: Departments of Dermatology and Pathology, MD Anderson, Houston, TX, USA.
authors.forename: Madeleine
authors.initials: M
authors.lastname: Duvic
authors.affiliation: Department of Dermatology and Pathology, Sloan Kettering Cancer Center, New York, NY, USA.
authors.forename: Melissa P
authors.initials: MP
authors.lastname: Pulitzer
authors.affiliation: Department of Dermatology and Pathology, Duke University, Durham,NC, USA.
authors.forename: Elise A
authors.initials: EA
authors.lastname: Olsen
authors.affiliation: Department of Dermatology and Pathology, University of Pennsylvania, Philadelphia, PA, USA.
authors.forename: Ellen
authors.initials: E
authors.lastname: Kim
authors.affiliation: Department of Dermatology and Pathology, University of Pennsylvania, Philadelphia, PA, USA.
authors.forename: Alain H
authors.initials: AH
authors.lastname: Rook
authors.affiliation: Department of Dermatology and Pathology, University of Pennsylvania, Philadelphia, PA, USA.
authors.forename: Sara S
authors.initials: SS
authors.lastname: Samimi
authors.affiliation: Department of Dermatology and Pathology, University of Wisconsin VAMC, Madison, WI, USA.
authors.forename: Gary S
authors.initials: GS
authors.lastname: Wood
authors.affiliation: Department of Dermatology and Pathology, Yale University, New Haven, CT, USA.
authors.forename: Michael
authors.initials: M
authors.lastname: Girardi
authors.affiliation: Department of Dermatology and Pathology, Ackerman Academy, New York, NY, USA.
authors.forename: Jacqueline
authors.initials: J
authors.lastname: Junkins-Hopkins
authors.affiliation: Departments of Dermatology and Pathology, MD Anderson, Houston, TX, USA.
authors.forename: Doina S
authors.initials: DS
authors.lastname: Ivan
authors.affiliation: Department of Dermatology and Pathology, Duke University, Durham,NC, USA.
authors.forename: M Angelica
authors.initials: MA
authors.lastname: Selim
authors.affiliation: Department of Dermatology and Pathology, Northwestern University Feinberg Medical School, Chicago, IL, USA.
authors.forename: Kimberly A
authors.initials: KA
authors.lastname: Sable
authors.affiliation: Department of Dermatology and Pathology, Duke University, Durham,NC, USA.
authors.forename: Pooja
authors.initials: P
authors.lastname: Virmani
authors.affiliation: Department of Dermatology and Pathology, University of California in San Francisco, San Francisco, CA, USA.
authors.forename: Laura B
authors.initials: LB
authors.lastname: Pincus
authors.affiliation: Departments of Dermatology and Pathology, MD Anderson, Houston, TX, USA.
authors.forename: Michael T
authors.initials: MT
authors.lastname: Tetzlaff
authors.affiliation: Department of Dermatology and Pathology, Stanford University, Stanford, CA, USA.
authors.forename: Jinah
authors.initials: J
authors.lastname: Kim
authors.affiliation: Department of Dermatology and Pathology, Stanford University, Stanford, CA, USA.
authors.forename: Youn H
authors.initials: YH
authors.lastname: Kim
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D005260:Female; D006801:Humans; D016410:Lymphoma, T-Cell, Cutaneous; D008297:Male; D008875:Middle Aged; D012878:Skin Neoplasms; D013602:T-Lymphocytes, Cytotoxic / Q000473:pathology*; D014944:World Health Organization; D055815:Young Adult