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酷似间质性T细胞淋巴瘤的泛发性早期炎症性硬斑病:诊断陷阱。

Generalized early inflammatory morphea mimicking interstitial T-cell lymphoma: A diagnostic pitfall.

作者信息

Go Janelle, Wu Yu-Hung

机构信息

Department of Dermatology, Region 1 Medical Center, Dagupan, Philippines.

Department of Dermatology, MacKay Memorial Hospital, Taipei, Taiwan.

出版信息

J Cutan Pathol. 2024 Jan;51(1):34-39. doi: 10.1111/cup.14516. Epub 2023 Aug 19.

Abstract

Early generalized morphea can clinically mimic mycosis fungoides. The microscopic features of early inflammatory morphea may show variable degrees of infiltration and do not have the characteristic dermal collagen sclerosis. We report the case of a 63-year-old female patient who presented with a 2-month history of an asymptomatic skin rash. Physical examination revealed multiple erythematous to dusky patches on the trunk and thighs, resembling the patch stage of mycosis fungoides. Two skin biopsies were performed, both of which showed prominent interstitial lymphoid infiltration in the reticular dermis without dermal sclerosis. Small lymphocyte exocytosis and lining along the dermal-epidermal junction were observed focally in the epidermis. Small clusters of plasma cells and eosinophils were observed in perivascular areas. Although no predominant clonality was found for CD4 and CD8 stains, 50% loss of CD5 antigen and 90% loss of CD7 antigen expression were apparent in immunohistochemical studies. Subsequent blood tests showed a normal blood cell count and positive human T-lymphotropic virus Type 1 antibodies. The overall findings suggested interstitial mycosis fungoides or early adult T-cell lymphoma-leukemia. The patient refused aggressive treatment, and 3 months later, she presented with indurated plaques from the previous rash. A repeat biopsy revealed the typical features of morphea. This report discussed the pitfalls in the clinical and histopathological diagnosis of early generalized inflammatory morphea that both clinicians and pathologists should consider.

摘要

早期泛发性硬斑病在临床上可模仿蕈样肉芽肿。早期炎症性硬斑病的微观特征可能显示不同程度的浸润,且不具有特征性的真皮胶原硬化。我们报告一例63岁女性患者,有2个月无症状皮疹病史。体格检查发现躯干和大腿有多处红斑至暗斑,类似蕈样肉芽肿的斑片期。进行了两次皮肤活检,均显示网状真皮有明显的间质淋巴细胞浸润,无真皮硬化。表皮局灶性观察到小淋巴细胞外渗并沿真皮 - 表皮交界处排列。血管周围区域观察到小簇浆细胞和嗜酸性粒细胞。尽管CD4和CD8染色未发现明显的克隆性,但免疫组化研究显示CD5抗原表达丧失50%,CD7抗原表达丧失90%。随后的血液检查显示血细胞计数正常,人类嗜T淋巴细胞病毒1型抗体阳性。总体结果提示间质型蕈样肉芽肿或早期成人T细胞淋巴瘤 - 白血病。患者拒绝积极治疗,3个月后,她出现了先前皮疹部位的硬结性斑块。再次活检显示为硬斑病的典型特征。本报告讨论了临床医生和病理学家在早期泛发性炎症性硬斑病临床和组织病理学诊断中应考虑的陷阱。

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