10.5826/dpc.1304a221
text/xml
2160-9381
3202
en
Cüyan  Demirkesen
https://creativecommons.org/licenses/by-nc/4.0
Dermatology Practical & Conceptual
An Atypical Presentation of Primary Cutaneous CD4+ Small/Medium Pleomorphic T-Cell Lymphoproliferative Disorder
Text.Serial.Journal
https://dpcj.org/index.php/dpc
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2023-10-31
2023-01-31
2023-10-31
2024-01-15
e2023221
Dermatol Pract Concept
Cüyan  Demirkesen
2023/10/31
10.5826/dpc.1304a221
e2023221
Department of Pathology, Acıbadem University, Istanbul, Turkey
2160-9381
en
e2023221
Swerdlow SH, Campo E, Pileri SA, et al. The 2016 revision of the World Health Organization classification of lymphoid neoplasms. Blood. 2016;127(20):2375-2390.
An Atypical Presentation of Primary Cutaneous CD4+ Small/Medium Pleomorphic T-Cell Lymphoproliferative Disorder
https://dpcj.org/index.php/dpc/article/download/3202/2372
      An Atypical Presentation of Primary Cutaneous
         CD4+ Small/Medium Pleomorphic T-Cell
              Lymphoproliferative Disorder
         Didem Kazan1, Dilek Bayramgürler1, Ahmet Tuğrul Eruyar2, Cüyan Demirkesen3


1 Department of Dermatology and Venerology, Kocaeli University, Kocaeli, Turkey
2 Department of Pathology, Kocaeli University, Kocaeli, Turkey
3 Department of Pathology, Acıbadem University, Istanbul, Turkey



Citation: Kazan D, Bayramgürler D, Eruyar AT, Demirkesen C. An Atypical Presentation of Primary Cutaneous CD4+ Small/Medium
Pleomorphic T-Cell Lymphoproliferative Disorder. Dermatol Pract Concept. 2023;13(4):e2023221. DOI: https://doi.org/10.5826/
dpc.1304a221
Accepted: April 17, 2023; Published: October 2023
Copyright: ©2023 Kazan et al. This is an open-access article distributed under the terms of the Creative Commons
Attribution-NonCommercial License (BY-NC-4.0), https://creativecommons.org/licenses/by-nc/4.0/, which permits unrestricted
noncommercial use, distribution, and reproduction in any medium, provided the original authors and source are credited.
Funding: None.
Competing Interests: None.
Authorship: All authors have contributed significantly to this publication.
Corresponding Author: Didem Kazan, Department of Dermatology and Venerology, Kocaeli University. Business tel : +902623037575
E-mail: didem.senses_343@hotmail.com




Case Presentation                                                        cutaneous CD4+ small/medium pleomorphic T-cell lymph-
                                                                         oproliferative disorder (CD4+ PCSM-LPD). Almost com-
A 64-year-old man presented to our out-patient clinic with               plete clinical response was achieved after nine sessions of
erythematous patches and papules on the left medial thigh                radiotherapy treatment (Figure-1B).
persisted for two years. Dermatological examination showed
erythematous patches and papules tended to merge on the
                                                                         Teaching Point
inner surface of the left thigh (Figure 1A). Other body areas
were normal. Histological examination of multiple punch                  CD4+ PCSM-LPD usually presents with a solitary slow-
biopsies showed diffuse infiltration of small-medium-sized               growing papule or nodule on the face, neck, or upper limbs.
atypical lymphocytes throughout the dermis. Immunohisto-                 It can occur in both sexes and at any age but especially in the
chemical studies found the atypical lymphocytes were pos-                6th and 7th decades of life [1]. Histopathological examina-
itive for CD2, CD3, CD4 in 95%, CD8 in 5%, and CD20                      tion and immunohistochemistry tests are the gold standard
in 2% but negative for CD7 and CD30. BCL-6 and PD-1                      diagnostic tests. It is classified as a provisional entity in the
expression was positive in 10% and 1-2% of the atypical                  5th edition of the World Health Organization (WHO) clas-
lymphocytic infiltration respectively (Figure 1, C-H). Blood             sification, due to its limited clinical risk, and indolent course
tests and lymph node ultrasonography were normal. Based                  to reflect its uncertain malignant potential [2]. Because of
on these findings, the patient was diagnosed with primary                localized disease, staging procedures are usually unnecessary.




Image Letter | Dermatol Pract Concept. 2023;13(4):e2023221                                                                              1
                       Figure 1. (A) Erythematous patches and papules that tended to merge on the inner surface
                       of the left thigh, (B) After nine sessions of radiotherapy treatment. (C-G) Atypical lym-
                       phocytes were positive for CD2 (C), CD3 (D), CD4 in 95% (E), BCL-6 in 10% (F), and
                       negative for CD-30 (G). (H) CD20 positivity detected in atypical lymphocytes 1%-2%.




However, in our patient we have a suspicion of follicu-              References
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