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Table of Contents 
Year : 2022  |  Volume : 67  |  Issue : 6  |  Page : 837
Primary cutaneous CD4+ small/medium T-cell lymphoproliferative disorder in an unusual location

1 Department of Dermatology, Hospital Universitario de Cabueñes, Gijón, Spain
2 Department of Dermatology, Hospital Universitario Río HOrtega, Valladolid, Spain
3 Department of Dermatology, Hospital Universitario Donostia, San Sebastián, Spain
4 Department of Dermatology, Hospital Clínico Universitario de Valladolid, Spain
5 Department of Pathology, Hospital Clínico Universitario de Valladolid, Spain

Date of Web Publication23-Feb-2023

Correspondence Address:
Daniel Ruiz-Sánchez
Department of Dermatology, Hospital Universitario de Cabueñes, Gijón
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijd.IJD_543_20

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How to cite this article:
Ruiz-Sánchez D, Valtueña J, Cantero MG, Barrutia L, Volo V, López PM, Martínez-García G. Primary cutaneous CD4+ small/medium T-cell lymphoproliferative disorder in an unusual location. Indian J Dermatol 2022;67:837

How to cite this URL:
Ruiz-Sánchez D, Valtueña J, Cantero MG, Barrutia L, Volo V, López PM, Martínez-García G. Primary cutaneous CD4+ small/medium T-cell lymphoproliferative disorder in an unusual location. Indian J Dermatol [serial online] 2022 [cited 2023 Mar 23];67:837. Available from:


Primary cutaneous CD4+ small/medium T-cell lymphoproliferative disorder (PCS-TCLPD) is an indolent T-cell lymphoproliferative disorder confined to the skin.

In the 2005 WHO-EORTC classification, primary cutaneous CD4+ small/medium T-cell lymphoma was included as a provisional type of cutaneous T-cell Lymphomas. The term “PCS-TCLPD” was finally included as an independent category in 2016, inside the WHO classification and the updated WHO-EORTC classification.[1]

We present the case of a 49-year-old man who came to the dermatology clinic with a 3-week course of a solitary nodule in the left inframammary region. He had a relevant background of eosinophilic esophagitis. The physical skin examination revealed a 5x11 mm pearly nodule, flexible in consistency, with overlying telangiectasia under the left breast [Figure 1]. The remainder of the skin examination was normal, and he did present neither lymphadenopathy nor visceromegaly.
Figure 1: (a) Picture of the lesion located in the left inframammary region (b) Image at higher magnification of the nodule

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Excision of the lesion was performed.

Histologic evaluation was consistent with a PCS-TCLPD. A small/medium lymphocytic infiltrate that occupies almost all the dermal thickness with no significant epidermotropism was observed. A small proportion (<30%) of large pleomorphic cells was presented mixed with small reactive CD8+ T cells, B cells, and plasma cells. The Ki-67 proliferation index was around 20% [Figure 2].
Figure 2: (a) (H and E: x4) Histological features of the lymphocytic infiltrate. (b) (H and E: x20) Histological features of the small/medium lymphocytic infiltrate with the accompanying cells. (c) (Ki-67 x40) Ki-67 index proliferation positive

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The atypical cells showed a characteristic immunophenotype of CD3+, CD4+, CD8-, CD30- [Figure 3]. PD1 was expressed with CD10 and the molecular EBER-FISH study, negatives.
Figure 3: (a) (CD4: x4) Immunohistochemical CD4 study was positive. (b) (CD8: x20) CD8 study was negative for the atypical cells. (c) (PD1 × 20) PD1 study was positive

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Monoclonal TCR gene rearrangements was detected, indicative of T lymphoid clonal proliferation. Polyclonal B-cell population was also observed.

No significant findings were observed in the imaging studies. After six months of follow-up, the patient remains asymptomatic.

The PCS-TCLPD can occur at any age, but most commonly affects individuals in the 5th to 7th decade of life and there is no gender predilection.

It commonly presents as a slow-growing erythematous or violaceous cutaneous lesion in the form of a papule, plaque, nodule, or tumor with ulceration rarely present.

The majority of cases involve the head and neck followed by the trunk and upper extremities. Lesions are typically asymptomatic, as it happened in our patient, although pain and pruritus have been reported.[2] The management if it does not resolve spontaneously after the skin biopsy, should be intralesional steroids, surgical excision, or, in rare instances, radiotherapy.[2]

Histological examination shows dense, nodular to diffuse dermal infiltrates that consist mainly of CD4+ small-/medium-sized pleomorphic T cells, whereas a small proportion (30%) of large pleomorphic cells may be present. These cells consistently express the follicular helper T-cell markers PD-1 (CD279), BCL6, and CXCL13.[3] The proliferation rate is low, varying between, 5% and at most 20%. In almost all cases, there is a considerable admixture with small reactive CD8+ T cells, B cells, and histiocytes, including multinucleated giant cells. In some cases, monotypic plasma cells may be present.[4]

PCS-TCLPD has a very favorable prognostic, with a five-year survival rates around 80-90%[5] and in typical cases, staging is not recommended.[1]

In conclusion, we present a new case of PCS-TCLPD, without extracutaneous involvement and with a complete remission after local surgical excision.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

   References Top

Willemze R, Cerroni L, Kempf W, Berti E, Facchetti F, Swerdlow SH, et al. The 2018 update of the WHO-EORTC classification for primary cutaneous lymphomas. Blood 2019;133:1703-14.  Back to cited text no. 1
Gru AA, Wick MR, Eid M. Primary cutaneous CD4+small/medium T-cell lymphoproliferative disorder-clinical and histopathologic features, differential diagnosis, and treatment. Semin Cutan Med Surg 2018;37:39–48.  Back to cited text no. 2
Cetinözman F, Jansen PM, Willemze R. Expression of programmed death-1 in primary cutaneous CD4-positive small/medium-sized pleomorphic T-cell lymphoma, cutaneous pseudo-T-cell lymphoma, and other types of cutaneous T-cell lymphoma. Am J Surg Pathol 2012;36:109-16.  Back to cited text no. 3
Balagué O, Martínez A, Colomo L, Roselló E, Garcia A, Martínez-Bernal M, et al. Epstein-Barr virus negative clonal plasma cell proliferations and lymphomas in peripheral T-cell lymphomas: A phenomenon with distinctive clinicopathologic features. Am J Surg Pathol 2007;31:1310-22.  Back to cited text no. 4
Beltraminelli H, Leinweber B, Keri H, Cerroni L. Primary cutaneous CD4+small/medium-sized pleomorphic T-cell lymphoma: A cutaneous nodular proliferation of plemorphic T lymphocites of undeterrmined significance? A study of 136 cases. Am J Dermatopathol 2009;31:317-22.  Back to cited text no. 5


  [Figure 1], [Figure 2], [Figure 3]


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