Helton J L, Maize J C
Department of Dermatology, Medical University of South Carolina, Charleston 29425, USA.
Am J Dermatopathol. 1996 Apr;18(2):111-7. doi: 10.1097/00000372-199604000-00002.
We review the clinical features, histopathology, and immunohistochemistry in three cases of eosinophilic histiocytosis, comparing lymphomatoid papulosis and eosinophilic histiocytosis. Each of the patients presented with self-healing recurrent papules and ulcerative nodules that were associated with pruritus. Disease duration was 5 months to 9 years. Histologically, the lesions demonstrated spongiosis and lymphocytic exocytosis, epidermal hyperplasia, papillary dermal edema, and a superficial and deep mixed perivascular inflammatory infiltrate. The infiltrate showed numerous eosinophils, histiocytoid cells, lymphocytes, and large mononuclear cells with atypical hyperchromatic nuclei. Most of the lymphocytes and large mononuclear cells with atypical nuclei marked with UCHL-1 (T-cell marker). The histiocytoid cells marked with S-100 and were dendritic both in the epidermis and the dermis. Eosinophilic histiocytosis appears to differ from classic lymphomatoid papulosis. It presents with recurrent papules and nodules associated with marked pruritus. Eosinophilic histiocytosis uniformly has more eosinophils and does not have the Reed-Sternberg cells often observed in lymphomatoid papulosis type A. Eosinophilic histiocytosis does not have cells that mark with Ki-1 and shows numerous S-100-positive histiocytoid cells that are most likely Langerhans cells, unlike lymphomatoid papulosis. However, eosinophilic histiocytosis may be an unusual Ki-1-negative variant of lymphomatoid papulosis with histopathologic changes not typical of type A or type B. In addition, eosinophilic histiocytosis lacks multinucleated histiocytes and the atypical histiocyte with a reniform nucleus, findings that are characteristic of histiocytosis X. Further studies are needed to define the pathophysiology and prognosis of this apparently distinct entity more accurately.
我们回顾了三例嗜酸性组织细胞增多症的临床特征、组织病理学和免疫组织化学表现，并与淋巴瘤样丘疹病进行比较。每位患者均表现为自愈性复发性丘疹和溃疡性结节，伴有瘙痒。病程为5个月至9年。组织学上，病变表现为海绵形成和淋巴细胞外渗、表皮增生、乳头真皮水肿以及浅层和深层混合性血管周围炎症浸润。浸润物显示有大量嗜酸性粒细胞、组织细胞样细胞、淋巴细胞和具有非典型深染核的大单核细胞。大多数淋巴细胞和具有非典型核的大单核细胞用UCHL-1（T细胞标志物）标记。组织细胞样细胞用S-100标记，在表皮和真皮中均为树突状。嗜酸性组织细胞增多症似乎与经典的淋巴瘤样丘疹病不同。它表现为伴有明显瘙痒的复发性丘疹和结节。嗜酸性组织细胞增多症始终有更多的嗜酸性粒细胞，且没有在A型淋巴瘤样丘疹病中常见的里德-斯腾伯格细胞。与淋巴瘤样丘疹病不同，嗜酸性组织细胞增多症没有用Ki-1标记的细胞，而是显示出许多S-100阳性的组织细胞样细胞，这些细胞很可能是朗格汉斯细胞。然而，嗜酸性组织细胞增多症可能是淋巴瘤样丘疹病的一种不寻常的Ki-1阴性变体，其组织病理学改变并非典型的A型或B型。此外，嗜酸性组织细胞增多症缺乏多核组织细胞和具有肾形核的非典型组织细胞，而这些是组织细胞增多症X的特征性表现。需要进一步研究以更准确地界定这一明显不同的实体的病理生理学和预后。
