Imaoka Kaoru, Furumura Minao, Maruyama Riruke, Nagasako Ren, Morita Eishin
Department of Dermatology, Shimane University Faculty of Medicine, Izumo.
Case Rep Dermatol. 2011 Sep;3(3):244-50. doi: 10.1159/000334830. Epub 2011 Nov 30.
A 70-year-old man was admitted to our hospital for evaluation of a rapidly progressive erythrodermia. He had superficial lymph node swelling and gluteal/inguinal nodosum-like lesions. A skin biopsy of the erythrodermia showed dense mixed infiltrates distributed throughout the whole dermis, predominantly consisting of small lymphocytes and histiocytes with multinucleated giant cells presenting with a granulomatous appearance. The dense infiltrates showed a characteristic angiocentric pattern surrounding the upward vasculature interconnecting the subcutaneous/subpapillary plexus in the dermis. Some infiltrating lymphocytes showed mild atypia with somewhat irregularly shaped nuclei. Their immunologic staining profiles supported the diagnosis of lymphomatoid granulomatosis. Despite the dense angiocentric infiltration in the dermis, typical angiodestructive infiltration with necrotic changes was not seen on pathological examination. In this case, in situ hybridization yielded negative findings for Epstein-Barr virus-encoded RNAs. Three months after the onset of erythrodermia, the patient developed pulmonary lymphomatoid granulomatosis. Corticosteroid pulse therapy was effective for the treatment of severe pulmonary infiltrations and erythrodermia. However, there had been mild recurrence of the condition or hypereosinophilia during the 4 years of follow-up. Low maintenance doses of cyclophosphamide and corticosteroid provided the patient symptomatic relief to date.
一名70岁男性因快速进展的红皮病入院接受评估。他有浅表淋巴结肿大及臀部/腹股沟结节样病变。红皮病的皮肤活检显示密集的混合性浸润分布于整个真皮层，主要由小淋巴细胞、组织细胞及多核巨细胞组成，呈现肉芽肿样外观。密集浸润围绕真皮内连接皮下/乳头下血管丛的向上走行血管呈现特征性的血管中心模式。部分浸润淋巴细胞显示轻度异型性，核形状略不规则。其免疫染色结果支持淋巴瘤样肉芽肿病的诊断。尽管真皮层有密集的血管中心浸润，但病理检查未见典型的伴有坏死改变的血管破坏性浸润。在该病例中，原位杂交检测爱泼斯坦-巴尔病毒编码RNA结果为阴性。红皮病发病3个月后，患者出现肺部淋巴瘤样肉芽肿病。糖皮质激素冲击疗法对治疗严重的肺部浸润和红皮病有效。然而，在4年的随访期间病情有轻度复发或嗜酸性粒细胞增多。低维持剂量的环磷酰胺和糖皮质激素至今为患者提供了症状缓解。
