Jacyk Witold K, Grayson Wayne, Dinkel Jurgen Erich, Requena Luis
Department of Dermatology, University of Pretoria, Pretoria, South Africa.
J Cutan Pathol. 2007 Aug;34(8):644-7. doi: 10.1111/j.1600-0560.2006.00698.x.
Pagetoid reticulosis (PR) is a low-grade primary cutaneous T-cell lymphoma that usually presents as a solitary, slowly enlarging erythematous or hyperkeratotic plaque on the distal areas of the extremities. Histopathologically, it is characterized by a dense, band-like infiltrate of atypical lymphocytes with prominent epidermotropism within a hyperplastic epidermis, and immunophenotypic studies show in most cases, a CD4-positive T-helper phenotype for the neoplastic lymphocytes. We describe an African man with a more than 20-year history of an acral lesion of PR, which was histopathologically characterized by lymphocyte immunophenotype consisting of CD8- and CD30-positive cells. We discuss the differential diagnosis with other primary cutaneous lymphoproliferative disorders showing similar immunophenotype. This case shows that CD30-positive PR should be included as a rare variant within the spectrum of CD30-positive primary cutaneous lymphoproliferative disorders. As in other primary cutaneous CD30-positive lymphoproliferative processes, lesions of CD30-positive PR show an indolent course and a benign biological behavior.
帕哲样网状细胞增生症(PR)是一种低度恶性的原发性皮肤T细胞淋巴瘤,通常表现为四肢远端孤立的、逐渐增大的红斑或角化过度斑块。组织病理学上,其特征为在增生的表皮内有密集的、带状的非典型淋巴细胞浸润,伴有明显的亲表皮现象,免疫表型研究显示,在大多数情况下,肿瘤淋巴细胞呈CD4阳性辅助性T细胞表型。我们描述了一名患有PR手部病变20多年的非洲男性,其组织病理学特征为淋巴细胞免疫表型由CD8和CD30阳性细胞组成。我们讨论了与其他表现出相似免疫表型的原发性皮肤淋巴增生性疾病的鉴别诊断。该病例表明,CD30阳性PR应作为CD30阳性原发性皮肤淋巴增生性疾病谱中的一种罕见变异类型。与其他原发性皮肤CD30阳性淋巴增生性病变一样,CD30阳性PR的病变呈惰性病程和良性生物学行为。