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Type D Lymphomatoid Papulosis: An uncommon Variant. A case report and review of the literature

机译:D型淋巴瘤样丘疹:一种罕见的变异。病例报告及文献复习

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Lymphomatoid papulosis (LyP) is an indolent form of primary cutaneous T-cell lymphoma, currently classified together with primary cutaneous anaplastic large T-cell lymphoma within the spectrum of CD30-positive lymphoproliferative disorders. It is characterized by presenting as a clinically benign but histopathological malignant disease. Clinical features consist in recurrent waxing and waning red papules. Histopathologically, there are 4 variants recognized, Type A or Hystiocitic type, being the most frecuent of all, Type B or Mycosis fungoides-like, Type C or Anaplastic large-cell lymphoma-like and Type D, the most recently described and uncommon variant with features similar to Cutaneous Aggressive CD8-Positive Cytotoxic T-Cell Lymphoma. We present a case of a 22-year-old female with multiple papules and nodules in trunk and limbs that after histopathological and immunochemical examination was compatible with Type D LyP. It is important to report this case, as a perfect example of an uncommon variant of LyP, with emphasis in its typical clinical, histopathological and inmunohistochemical findings and review of the literature.
机译:淋巴瘤样丘疹病(LyP)是原发性皮肤T细胞淋巴瘤的一种惰性形式,目前与CD30阳性淋巴增生性疾病范围内的原发性皮肤间变性大T细胞淋巴瘤一起被分类。其特征是表现为临床上良性但组织病理学上为恶性的疾病。临床特征包括反复出现蜡状和减弱的红色丘疹。在组织病理学上,有4种变异,A型或Hystiocitic型是最常见的,B型或真菌病样,C型或间变性大细胞淋巴瘤样,D型是最近描述和罕见的变异。具有类似于皮肤侵袭性CD8阳性细胞毒性T细胞淋巴瘤的特征。我们提出了一例22岁的女性,在躯干和四肢中有多个丘疹和结节,经组织病理学和免疫化学检查后可与D型LyP相容。重要的是,报告此病例,作为LyP罕见变异的完美实例,重点是其典型的临床,组织病理学和免疫组化研究结果,并复习文献。

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