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Rapidly Evolving Necrotic Plaques and Nodules in a Middle-aged Woman

Educational Objective
Based on this clinical scenario and the accompanying image, understand how to arrive at a correct diagnosis.
1 Credit CME

A woman in her 50s presented to a tertiary dermatology referral center with scaly erythematous patches and plaques covering 10% of her total body surface area without clinically appreciable lymphadenopathy. Histopathological testing demonstrated an epidermotropic infiltrate of hyperchromatic, convoluted, atypical CD3+, CD4, and CD8+ lymphocytes with haloed nuclei with basilar tagging and papillary dermal fibrosis. Studies of T-cell gene rearrangements identified a clonal population. Blood-flow cytometry did not reveal aberrant T-cell immunophenotypes. She was treated initially with topical and intralesional corticosteroids and narrow band UV B phototherapy. Oral bexarotene was subsequently added for recalcitrant disease. Within 2 months, she developed isolated, painful tumors. A second biopsy was performed, which was notable for an epidermotropic infiltrate of CD3+, CD4, and CD8 weak lymphocytes, which were positive for TIA-1, granzymes, and B factor (Bf) 1 and negative for CD30 and CD56. The results of flow cytometry and imaging were unremarkable. After an initial favorable response with chlorambucil, she developed expanding ulcerated tumors complicated by a polymicrobial superinfection. Chlorambucil was discontinued in favor of antimicrobial treatment. She then received liposomal doxorubicin, although skin involvement continued to progress rapidly, with ulcerating tumors making up as much as 80% of her total body surface area. She was hospitalized for worsening disease and pain (Figure, A). Repeated histopathologic analysis was done (Figure, B, C, and D).

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C. Primary cutaneous aggressive epidermotropic cytotoxic CD8+ T-cell lymphoma

The repeated histopathologic analysis revealed atypical, strikingly epidermotropic lymphocytes positive for CD3, CD8, TIA1, granzymes, and Bf1 and negative for CD4, CD30, CD56, and Epstein-Barr virus–encoded RNA, with enlarged, misfolded nuclei throughout the spinous layer (Figure, C and D). Histological testing and clinical presentation supported a diagnosis of primary cutaneous aggressive epidermotropic cytotoxic CD8+ T-cell lymphoma (PCECTCL).

This condition is recognized as a distinct diagnosis in current World Health Organization and European Organization for Research and Treatment of Cancer guidelines and represents less than 1% of all cutaneous T-cell lymphomas.13 It is characterized by the abrupt onset of widespread, ulcerating papules, plaques, or tumors with or without mucosal and/or visceral involvement.1,36 While this disease entity is generally described as strikingly and rapidly progressive, in several case reports, patients presented initially with patch/plaque-stage CD8+ mycosis fungoides (MF), which slowly progressed to recalcitrant, ulcerating tumors over months to years after the initial diagnosis.6 On histopathologic examination, marked epidermotropism in a pagetoid reticulosis–like pattern may be appreciated, generally in the absence of significant Pautrier microabscesses.1,6 Angiocentricity and destruction are not uncommon and likely contribute to the ulceration perceived on clinical examination.1,6 A characteristic cytotoxic phenotype is present with lymphocytes expressing Bf1, TIA1, and granzyme B to a marked degree.1,3,6 Expression of CD2 and CD5 is typically not present, and lymphocytes rarely express CD30 or CD56.1,3,6 In addition, high proliferative fractions may be present, as evidenced by high KI-67 fractions.1

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Article Information

Corresponding Author: Laurel L. Wessman, MD, Department of Dermatology, University of Minnesota, 516 Delaware St SE, Mail Code 98, Phillips-Wangensteen Building, Ste 4-240, Minneapolis, MN 55455 (wessm018@umn.edu).

Published Online: February 19, 2020. doi:10.1001/jamadermatol.2019.4992

Conflict of Interest Disclosures: None reported.

Disclaimer: Dr Gaddis is associate editor of JAMA Dermatology, but he was not involved in any of the decisions regarding review of the manuscript or its acceptance.

Additional Contributions: We thank the patient’s daughter for granting permission to publish this information.

References
1.
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2.
Kempf  W , Zimmermann  AK , Mitteldorf  C .  Cutaneous lymphomas—an update 2019.   Hematol Oncol. 2019;37(suppl 1):43-47. doi:10.1002/hon.2584PubMedGoogle ScholarCrossref
3.
Geller  S , Myskowski  PL , Pulitzer  M , Horwitz  SM , Moskowitz  AJ .  Cutaneous T-cell lymphoma (CTCL), rare subtypes.   Chin Clin Oncol. 2019;8(1):5. doi:10.21037/cco.2018.11.01 PubMedGoogle ScholarCrossref
4.
Al Aoun  SM , Iqbal  S , AlHalouli  TM , Zaidi  SZ , Motabi  IH .  Durable remission of a patient with primary cutaneous CD8+ aggressive epidermotropic cytotoxic T-cell lymphoma.   Hematol Oncol Stem Cell Ther. 2018;S1658-3876(18)30096-7. doi:10.1016/j.hemonc.2018.09.004 PubMedGoogle Scholar
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Tomasini  C , Novelli  M , Fanoni  D , Berti  EF .  Erythema multiforme-like lesions in primary cutaneous aggressive cytotoxic epidermotropic CD8+ T-cell lymphoma.   J Cutan Pathol. 2017;44(10):867-873. doi:10.1111/cup.12995 PubMedGoogle ScholarCrossref
6.
Guitart  J , Martinez-Escala  ME , Subtil  A ,  et al.  Primary cutaneous aggressive epidermotropic cytotoxic T-cell lymphomas.   Mod Pathol. 2017;30(5):761-772. doi:10.1038/modpathol.2016.240 PubMedGoogle ScholarCrossref
7.
Cyrenne  BM , Subtil  A , Girardi  M , Foss  F .  Primary cutaneous aggressive epidermotropic cytotoxic CD8+ T-cell lymphoma.   Int J Dermatol. 2017;56(12):1448-1450. doi:10.1111/ijd.13792 PubMedGoogle ScholarCrossref
8.
Berti  E , Tomasini  D , Vermeer  MH , Meijer  CJ , Alessi  E , Willemze  R .  Primary cutaneous CD8-positive epidermotropic cytotoxic T cell lymphomas.   Am J Pathol. 1999;155(2):483-492. doi:10.1016/S0002-9440(10)65144-9 PubMedGoogle ScholarCrossref
9.
Pan  ST , Chang  WS , Murphy  M , Martinez  A , Chuang  SS .  Cutaneous peripheral T-cell lymphoma of cytotoxic phenotype mimicking extranodal NK/T-cell lymphoma.   Am J Dermatopathol. 2011;33(2):e17-e20. doi:10.1097/DAD.0b013e3181ea6571 PubMedGoogle ScholarCrossref
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