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Abstract
Primary cutaneous CD4+ small/medium T-cell lymphoproliferative disorder is indolent clinical behaviour and uncertain malignant potential. Histologically, these lesions show a predominance of small to medium-size CD4+ pleomorphic T-cell expressing follicular helper T-cell markers. We report the case of a 59-year-old female who presented with nodules on the left chest for 3 years. Dermatological examination showed four red nodules localized on the left chest with angiotelectasis without tenderness. The histopathological manifestation was consistent with the diagnosis of primary cutaneous CD4+ small/medium T-cell lymphoproliferative disorder. We focus on the clinical appearance, histopathological features, diagnosis, and differential diagnosis of primary cutaneous CD4+ small/medium T-cell lymphoproliferative disorder.
Keywords: Differential diagnosis, immunohistochemical characteristics, lymphoproliferative disorder, pathological, primary
Introduction 
Primary cutaneous CD4+ small/medium T-cell lymphoproliferative disorder (PCS-TCLPD) was previously considered lymphoma but was classified as lymphoproliferative disorder by WHO in 2016 according to its indolent clinical behaviour and uncertain malignant potential, accounting for about 2–3% of all cutaneous lymphomas.[1] Its rash is usually single, with slow-growing papules, plaques, nodules, tumours as the main manifestation, with a reddish surface, rarely ulcerated, and usually no systemic symptoms.[2] Histologically, these lesions show a predominance of small to medium-size CD4+ pleomorphic T-cell expressing follicular helper T-cell markers.[3] Some studies suggest PCS-TCLPD can be deduced by immunosuppressive agents and biological agents to treat rheumatoid arthritis, IL-2, and vemurafenib to treat metastatic melanoma, etc.[4]
Herein, we report a case of PCS-TCLPD in a middle-aged female and its differential diagnosis through pathological.
Case Report A 59-year-old female patient presented with nodules on the left chest for 3 years; the number and size of nodules increased, without eroding and rupturing. She has had hypertension for more than 10 years and coronary heart disease for more than 5 years. No family history of genetics and tumours. Physical examination showed a stable general appearance; there was no obvious abnormality in the heart, lung, and abdomen, and superficial lymph nodes were not palpated. Dermatological examination revealed four red nodules on the upper part of the left chest. They manifested as 1 × 1 cm to 2 × 3 cm smooth nodules with angiotelectasis, medial hardness, mobility, and clustered [Figure 1]. Auxiliary examination: hyperlipoidemia, blood routine, urine routine, stool routine, liver and kidney function, blood glucose, myocardial enzymes, and tumour screening indicators were not abnormal. There were not tumour changes in the abdominal and superficial lymph nodes through ultrasonography and X-ray of the lung. Dermal pathology showed no infiltrating zone under the epidermis, and dense lymphocytes with nodular infiltration were present at the whole dermis, partially segmented by collagen fibres. Lymphocytes were small to medium size, with mild atypia, and a few histiocytes and plasma cells were observed [Figure 2]. Immunostaining showed tumour cells were positive for CD3, CD4, CD5, Bcl-6, CD8, CD20, negative for CD10, CD21, CD23, CD30, CD56, PAX-5, Cyclin-D1, and TCR; 10% positive for Ki-67 [Figure 3].
Figure 1: Skin lesions on the left chest, 1 × 1 cm to 2 × 3 cm smooth nodules with angiotelectasis, medial hardness, mobility, and clustered
Figure 2: Hematoxylin-eosin staining, (i) magnification is 40, (ii) magnification is 100, (iii) magnification is 200, (iv) magnification is 400
Figure 3: Streptavidin-perosidase staining, (i) magnification is 200, (ii, iii, iv, v, vi and vii) magnification are 400According to this case's clinical, pathological, and immunohistochemical characteristics, the patient was diagnosed with PCS-TCLPD.
Discussion In this case, the skin lesions presented as nodules on the left chest growing slowly, and the following diseases should be considered: dermatofibrosarcoma protuberans presents as a slowly progressive, painless cutaneous lesion that may begin as a violet or pink plaque,[5],[6] commonly seen in young and middle-aged men; lymphoma showed painless lymphadenopathy with other systemic symptoms; sarcoidosis, clinical manifestation is chronic granuloma, often involving the lung, skin, and eyes;[7] other soft tissue tumours or metastatic carcinomas.
The cells were expressed in PCS-TCLPD, especially Bcl-6, CXCL-13, PD-1, while CD-10 was usually negative.[1] For diagnosis, it must be differentiated from other lymphomas expressing PD-1 and CXCL-13.
Cutaneous pseudolymphoma: nodular PSL is characterized by a dense nodular infiltrate in the reticular dermis; there is an admixture of dendritic cells and macrophages.[8]
Mycosis fungoides: mature, indolent T-cell lymphomas characterized by infiltrated plaques that usually persist over a long time.[9] Histological findings show small to medium gyrus T-cell infiltration in the superficial dermis, and lymphocytes infiltrate the epidermis to form a Pautrier microabscess.[10]
Lymphomatoid papulosis: belongs to the CD30 positive cutaneous lymphoproliferative disorder.[11]
Peripheral T-cell lymphoma, not otherwise specified, is a group of T-cell lymphomas that shows more than 30% pleomorphic medium to large tumour cells, a high Ki-67 labelling index.[12]
Primary cutaneous marginal zone lymphoma and primary cutaneous follicle centre lymphomas are low-grade lymphoma with indolent clinical.[13] Primary cutaneous marginal zone lymphoma shows nodular proliferation of dense lymphoid cells mainly in the dermis.[14]
PCS-TCLPD is a clinically indolent process with a good overall prognosis. In this case, the patient was lost to follow-up. Current treatments include surgical resection, injection of steroid hormone into the skin lesion, topical corticosteroid ointment, local radiotherapy, etc.[15] A review of the few similar reported cases indicates that the 5-year survival rate is almost 100%. Some cases have reported spontaneous remission after biopsy in some patients.[1]
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.
Financial support and sponsorship
This work was supported by the Natural Science Foundation of Hunan Province, China (grant No. 2019JJ40498).
Conflicts of interest
There are no conflicts of interest.
References 
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