Department of Dermatology, Inje University Sanggye Paik Hospital, Seoul, Republic of Korea; and.
Gaekhyeon-ri, Jeokseong-myeon, Paju-si, Gyeonggi-do, Republic of Korea.
Am J Dermatopathol. 2022 Feb 1;44(2):121-125. doi: 10.1097/DAD.0000000000002011.
Lennert lymphoma is a lymphoepithelioid variant of peripheral T-cell lymphoma (not otherwise specified) with characteristics that do not fit into other peripheral T-cell lymphoma categories. Lennert lymphoma is primarily a nodal disease, and skin involvement may be exhibited. Cutaneous manifestations in Lennert lymphoma are nonspecific and include erythematous papules, nodules, and small plaques. Histological examination of cutaneous involvement characteristically presents epithelioid histiocytes and atypical small lymphocytes around vessels or appendages. A lymph node (LN) biopsy is essential for Lennert lymphoma diagnosis. In Lennert lymphoma, immunohistochemistry of both LNs and the involved skin reveals T-cell marker positivity. Although most Lennert lymphoma cases present with a single-positive CD4/CD8 immunophenotype, few cases present with a double-positive CD4/CD8 immunophenotype. We report a case of a 54-year-old woman presenting with fever, chills, general weakness, and a skin rash of erythematous patches on the trunk, extremities, and buttocks. A skin biopsy of the buttocks revealed atypical lymphocytes around the dermal vessels. In immunohistochemistry, these atypical lymphocytes stained positive for CD3, CD4, CD8, and CD68 but negative for CD20, CD30, and granzyme B. Similarly, a biopsy of the axillary LN revealed numerous epithelioid cells with atypical lymphocytes, exhibiting positivity for CD3, CD4, CD8, and CD68 but negativity for CD20, CD30, and S-100. Ki-67 was overexpressed in both the skin and LN. The final diagnosis of the patient was Lennert lymphoma with cutaneous involvement and a rare double-positive CD4/CD8 immunophenotype. The patient was transferred to another hospital for chemotherapy as per her request.
林内特淋巴瘤是一种外周 T 细胞淋巴瘤(未特指)的淋巴上皮样变异型,其特征不符合其他外周 T 细胞淋巴瘤类别。林内特淋巴瘤主要是一种结内疾病,可能会出现皮肤受累。林内特淋巴瘤的皮肤表现是非特异性的,包括红斑丘疹、结节和小斑块。皮肤受累的组织学检查特征是血管周围或附属物周围有上皮样组织细胞和非典型小淋巴细胞。林内特淋巴瘤的诊断需要进行淋巴结(LN)活检。在林内特淋巴瘤中,LN 和受累皮肤的免疫组织化学均显示 T 细胞标志物阳性。尽管大多数林内特淋巴瘤病例表现为单一阳性的 CD4/CD8 免疫表型,但少数病例表现为双阳性的 CD4/CD8 免疫表型。我们报告了一例 54 岁女性,表现为发热、寒战、全身无力和躯干、四肢和臀部出现红斑斑块样皮疹。臀部皮肤活检显示真皮血管周围有异型淋巴细胞。在免疫组织化学中,这些异型淋巴细胞 CD3、CD4、CD8 和 CD68 染色阳性,而 CD20、CD30 和颗粒酶 B 染色阴性。同样,腋窝 LN 活检显示大量上皮样细胞伴异型淋巴细胞,CD3、CD4、CD8 和 CD68 染色阳性,CD20、CD30 和 S-100 染色阴性。Ki-67 在皮肤和 LN 中均过度表达。患者最终诊断为伴有皮肤受累和罕见双阳性 CD4/CD8 免疫表型的林内特淋巴瘤。根据患者要求,将其转至另一家医院进行化疗。