Primary Cutaneous Aggressive Epidermotropism CD8+ T-Cell Lymphoma: A Challenging Case
DOI:
https://doi.org/10.33820/mdvi.v50i2.409Keywords:
agresif, CD8 cytotoxic t-cell, cutaneous T-cell lymphoma, epidermotropismeAbstract
Introduction: Primary cutaneous aggressive epidermotropism CD8+ cytotoxic t-cell lymphoma (PCAE-CD8+ CTCL) is an aggressive variant of cutaneous T-cell lymphoma which has poor prognosis. Because of its rarity, its relatively variable clinical dan histopathological presentation, and no diagnostic criteria for PCAE-CD8+ CTCL, establishing diagnosis can be challenging. Case: A case of PCAE-CD8+ CTCL in a 67-year-old man whose early histopathological features resembling parapsoriasis was reported. Physical examination showed multiple lesions in form of erythematous plaques with ulcers and crusts in almost part of the body. Histopathological features showed epidermotropism and infiltrates of atypical also pleomorphic lymphocytes. Immunohistochemistry result was positive for CD8, CD4, Granzyme, CD3, and Ki-67 supported PCAE-CD8+ CTCL. Discussion: Clinical manifestation of PCAE-CD8+ CTCL varies among patients. Histopathological and immunohistochemistry examination are needed to establish diagnosis. Conclusion: The understanding of clinical manifestation of PCAE-CD8+ CTCL is needed. Because of its poor prognosis and high proliferative activity, early diagnosis may be helpful in improving the likelihood of patient survival.
Downloads
References
1. Bagot M SR. Fitzpatrick’s dermatology. Kang S AM, Bruckner AL, Enk AH, Margolis DJ, McMichael AJ, editor. New York: McGraw-Hill; 2019. 2072-107 p.
2. Gormley RH HS, Anand D, Junkins-Hopkins J, Rook AH, Kim EJ. Primary cutaneous aggressive epidermotropic CD8+ T-cell lymphoma. J Am Acad Dermatol. 2010;62:300-7.
3. Robson A AC, Bagot M, Burg G, Calonje E, Castillo C. Aggressive epidermotropic CD8+ lymphoma: a cutaneous lymphoma with distinct clinical and pathological features. Report of an EORTC cutaneous lymphoma task force workshop. Histopathol. 2015;67:425-41.
4. Willemze R CL, Kempf W, Berti E, Facchetti F, Swerdlow H. The 2018 update of the WHO-EORTC classification for primary cutaneous lymphomas. Am Soc Hematol. 2019;133:1703-14.
5. Onsun N DD, Emiroğlu N, Yildiz P, Akaslan TÇ, Tosuner Z, Çetin G, Demirkesen C. Challenges in early diagnosis of primary cutaneous CD8+ aggressive epidermotropic cytotoxic T-cell lymphoma: a case series of four patients. Eur J Dermatol. 2020;30:358-61.
6. Nofal A A-MM, Assaf M, Salah E. Primary cutaneous aggressive epidermotropic CD8+ T-cell lymphoma: proposed diagnostic criteria and therapeutic evaluation. J Am Acad Dermatol. 2012;67:748-59.
7. Griffiths CEM BJ, Bleiker T, Chalmers R, Creamer D. Rook’s textbook of dermatology. Whittaker SJ CF, editor. West Sussex: Black Willey; 2019.
8. Patterson JW HG, Prenshaw KL. Weedon’s skin pathology. Patterson JW HG, Prenshaw KL, editor. Virginia: Elsevier; 2021.
9. Tomasini C NM, Fanoni D, Berti EF. Erythema multiformeâ€like lesions in primary cutaneous aggressive cytotoxic epidermotropic CD8+ Tâ€cell lymphoma: A diagnostic and therapeutic challenge. J Cutan Pathol. 2017;44:867-73.
10. Beggs SM FB, Kornreich D, Okon L, Alpdogan O, Shi W, Sahu J. Primary cutaneous CD8+ T-cell lymphoma, an indolent and locally aggressive form mimicking paronychia. Am J Dermatol. 2018;56:1448-50.
11. Cyrenne BM SA, Girardi M, Foss F. Primary cutaneous aggressive epidermotropic cytotoxic CD8+ T-cell lymphoma: long-term remission after brentuximab vedotin. Int J Dermatol. 2017;56:1448-50.
12. Terui H YK, Yoshida S, Aiba S. Primary cutaneous aggressive epidermotropic CD8+ T-cell lymphoma with brain involvement and mimicking dermatomyositis. Acta Derm Venereol. 2018;98:128-30.
13. Deenen NJ KL, Jaspars EH, Vermeer MH, Rie MA, Bekkenk MW. Pitfalls in diagnosing primary cutaneous aggressive epidermotropic CD8+ T-cell lymphoma. Br J Dermatol. 2018;180:1-7.