PMC8402951_01
Representative images of skin manifestations and pathological examination of skin biopsy from the patient. (A) Keratoderma and fissures on her hands. (B and C). Nail thickening with color change on her hands (B) and feet (C). (D) Exfoliation on her back. (E-I) The skin biopsy shows non-specific dermatitis ((E) for original magnification x 40, (F) for original magnification x 400, (G-I) for original magnification x 200). (E and F) perivascular lymphocytes infiltration in the superficial dermis. (G) Positive staining for CD3. (H) Positive staining for CD4. (I) Positive staining for CD7.
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A 57-year-old female presented with longstanding skin symptoms and lesions, including a 6-year history of recurrent pruritus, a 4-year history of papules and painless inguinal lymphadenopathy, and a 2-year history of erythematous skin lesions and exfoliation that began on her legs, back, arms and eventually spread to erythroderma of the whole body, accompanied by keratoderma and fissures on her feet and hands and nail thickening with color change (Figure 1). Skin biopsy revealed non-specific dermatitis (Figure 1) without dermal lymphocytic atypia and epidermotropism. The lung computed tomography scan was normal (Supplementary Figure 1), and no hepatosplenomegaly was shown by abdominal ultrasound.
Pathological examination of the lymph node biopsy. (A and B). The lymph node biopsy shows extensive involvement of Langerhans cells in the sinus and paracortical regions, which present pale staining compared with residual follicles (A for original magnification x 25, (B) for original magnification x 200). (C-I) Immunohistochemical staining results ((D) for original magnification x 25; (C), (E-I) for original magnification x 40). (C) Positive staining for CD3. (D) Positive staining for CD20. (E) Positive staining for CD68. (F) Positive staining for CD1a. (G) Positive staining for S-100. (H) Negative staining for cyclinD1. (I) Positive staining for CD207.
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An inguinal LN biopsy showed extensive involvement of S-100, CD1a and CD207 positive and cyclinD1 negative Langerhans cells in the sinus and paracortical regions, presenting pale staining compared with residual follicles (Figure 2). T cells in paracortex were observed (Figure 2). The patient denied polyuria, pulmonary symptoms, bone pain and fever. TCRalphabeta; partially expressed CD2; and was negative for CD45RA, TCRgammadelta, CD25, CD161, CD38, CD16, CD57, HLA-DR, CD56, CD8, CD94, CD10, cKi67, CD34, CD117, or CD33 (Figure 3B). These abnormal mature T lymphocytes had infiltrated into the bone marrow (BM), as detected by FCI, accounting for 4.54% of all nucleated cells (Figure 3C). The CD26 expression was detected during the follow-up, the data showed CD4+CD26- cells accounting for 46.2% of total lymphocytes (Supplementary Figure 2).
Representative images of morphology and flow cytometric immunophenotyping. (A) Representative images of Sezary cells in peripheral blood (original magnificationx1000). (B) Representative scatter plots for peripheral blood; abnormal mature T lymphocytes were monoclonal (CD3dim+, CD5+, CD7+, CD4+, CD8-, CD45RO+, CD45RA-, TCRalphabeta+, TCRgammadelta-, CD10-, and cKi67-). (C) Representative scatter plots for bone marrow; abnormal mature T lymphocytes were monoclonal (CD3dim+, CD2+, CD7+, CD4+, CD8-, CD45RO+, CD45RA-, CD5+, TCRalphabeta+, TCRgammadelta-, and CD56-).
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TCRalphabeta; partially expressed CD2; and was negative for CD45RA, TCRgammadelta, CD25, CD161, CD38, CD16, CD57, HLA-DR, CD56, CD8, CD94, CD10, cKi67, CD34, CD117, or CD33 (Figure 3B). These abnormal mature T lymphocytes had infiltrated into the bone marrow (BM), as detected by FCI, accounting for 4.54% of all nucleated cells (Figure 3C). The CD26 expression was detected during the follow-up, the data showed CD4+CD26- cells accounting for 46.2% of total lymphocytes (Supplementary Figure 2). Moreover, T-cell receptor (TCR) gene rearrangement was found in PB, skin, LN and BM cells, whereas the BRAF V600E gene mutation was negative in any of these samples (Table 1, Supplementary Figure 3).