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Case Reports
. 2025 Apr 13;17(4):e82211.
doi: 10.7759/cureus.82211. eCollection 2025 Apr.

Primary Cutaneous CD4+ Small/Medium T-cell Lymphoproliferative Disorder in a Young Japanese Male Patient

Yuta Norimatsu  1   2 , Mina Komuta  3 , Yuichiro Hayashi  4 , Kennosuke Karube  5 , Makoto Sugaya  2
Affiliations
Case Reports

Primary Cutaneous CD4+ Small/Medium T-cell Lymphoproliferative Disorder in a Young Japanese Male Patient

Yuta Norimatsu et al. Cureus. .

Abstract

A 34-year-old man became aware of an erythematous nodule on the left nasal wing. He was treated with topical steroids and oral antibacterial agents at his local doctor, but his condition did not improve, and he was referred to our hospital. A skin biopsy revealed diffuse cellular infiltration through the dermis. No epidermotropism was seen. The major infiltrate was small to medium-sized lymphoid cells. The number of CD3+ cells was almost the same as that of CD20+ cells, while CD4+ cells were dominant over CD8+ cells. Atypical lymphocytes were positive for BCL6 and PD-1. Polymerase chain reaction (PCR) analysis of immunoglobulin heavy chain and T-cell receptor gene rearrangements on paraffin-embedded tissue sections revealed a clonal expansion of T-cells. The patient was diagnosed as having primary cutaneous CD4+ small/medium T-cell lymphoproliferative disorder (PCSM-LPD) and treated with fludroxycortide tape. The red nodule completely disappeared after three months. Nuclear staining for nuclear factor of activated T-cells c1 (NFATc1), which had been suggested to be useful in distinguishing PCSM-LPD from pseudolymphoma, was negative in our case. Our case was considered to be typical of PCSM-LPD among existing reports of PCSM-LPD from Japan, except for the young age of the patient. Our case suggested that young cases with PCSM-LPD may have been misdiagnosed with cutaneous pseudolymphoma (CPL), which may be one of the reasons why this type of lymphoproliferative disorder has been reported to occur in elderly people.

Keywords: bcl6; cutaneous pseudolymphoma; nuclear staining for nuclear factor of activated t-cells c1 (nfatc1); primary cutaneous cd4+ small/medium t-cell lymphoproliferative disorder (pcsm-lpd); programmed death 1 (pd-1); t-cell receptor gene rearrangement.

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Conflict of interest statement

Human subjects: Consent for treatment and open access publication was obtained or waived by all participants in this study. Insitutional Review Board, International University of Health and Welfare, Japan issued approval 20-Nr-009. The authors obtained the necessary written in-force consent to report the case. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work.

Figures

Figure 1
Figure 1. Red nodule on the left wing of the nose at the first visit.
Figure 2
Figure 2. Hematoxylin and eosin stain shows significant cellular infiltration of the dermis (x40).
Figure 3
Figure 3. Cells infiltrating the dermis in hematoxylin and eosin stain are small to medium-sized lymphoid cells (x400).
Figure 4
Figure 4. Immunohistological staining for CD3.
Figure 5
Figure 5. Immunohistological staining for CD4.
Figure 6
Figure 6. Immunohistological staining for CD8.
Figure 7
Figure 7. Immunohistological staining for CD20.
Figure 8
Figure 8. Immunohistological staining for Ki-67.
Figure 9
Figure 9. Immunohistological staining for BCL6.
Figure 10
Figure 10. Immunohistological staining for PD-1.
Figure 11
Figure 11. Red nodule on left wing of nose completely disappeared after three months.
Figure 12
Figure 12. Nuclear staining for nuclear factor of activated T-cells c1 (NFATc1) in this case.
In a previous study [8], the cytoplasm was stained; however, in the present case (as shown in the image), the cytoplasm is not stained.
See this image and copyright information in PMC

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