Tsang W Y, Chan J K, Loo K T, Wong K F, Lee A W
Department of Pathology, Queen Elizabeth Hospital, Hong Kong.
Histopathology. 1994 Jul;25(1):49-55. doi: 10.1111/j.1365-2559.1994.tb00597.x.
Granulomatous slack skin is an extremely uncommon form of cutaneous T-cell lymphoma. We report a case occurring in a 29-year-old man, who had generalized, progressive skin lesions evolving to nodular swellings and folds in the flexural regions, and peripheral blood and marrow involvement. The biopsies were initially misinterpreted as xanthogranuloma or granulomatous inflammation. Histologically, the entire dermis and subcutis was infiltrated by non-necrotizing granulomas comprising mononuclear histiocytes, multinucleated giant cells and small lymphoid cells with irregularly folded nuclei, associated with loss of elastic fibres. The small lymphoid cells showed focal epidermotropism. Immunohistochemical studies showed that they were of T-lineage (CD3+, CD43+, CD45RO+). The multinucleated giant cells, which showed reactivity with the histiocytic markers CD68 and Mac387, were highlighted by intense, thick membrane staining with CD45, CD43 and CD45RO. Ultrastructurally, they exhibited features of macrophages with numerous surface villous processes and lysosomes. Greater awareness of this entity may facilitate more prompt and accurate diagnosis, obviating a futile search for a non-existent infective aetiology.
肉芽肿性皮肤松弛症是一种极为罕见的皮肤T细胞淋巴瘤形式。我们报告了一例发生在一名29岁男性身上的病例，该患者有全身性、进行性皮肤病变，发展为结节性肿胀以及屈侧部位的褶皱，同时伴有外周血和骨髓受累。活检最初被误诊为黄色肉芽肿或肉芽肿性炎症。组织学上，整个真皮和皮下组织被非坏死性肉芽肿浸润，这些肉芽肿由单核组织细胞、多核巨细胞和核呈不规则折叠的小淋巴细胞组成，并伴有弹性纤维丧失。小淋巴细胞表现出灶性亲表皮性。免疫组化研究显示它们属于T细胞谱系（CD3 +、CD43 +、CD45RO +）。多核巨细胞与组织细胞标志物CD68和Mac387反应，通过CD45、CD43和CD45RO的强烈厚膜染色得以凸显。超微结构上，它们表现出巨噬细胞的特征，有许多表面绒毛状突起和溶酶体。对该疾病有更高的认识可能有助于更迅速准确的诊断，避免徒劳地寻找不存在的感染病因。
