Rubenstein Melissa A, Farnsworth Neil N, Pielop Josie A, Orengo Ida F, Curry Jonathan L, Drucker Carol R, Hsu Sylvia
Department of Dermatology, Baylor College of Medicine, USA.
Dermatol Online J. 2006 Jan 27;12(1):8.
Sinus histiocytosis with massive lymphadenopathy, or Rosai-Dorfman disease, is a benign idiopathic histiocytic proliferative disorder that commonly involves the lymph nodes but secondarily may involve the skin. However, purely cutaneous disease without lymphadenopathy or internal organ involvement rarely may occur. We present case reports of three patients who presented with asymptomatic nonspecific enlarging skin nodules without evidence of lymphadenopathy or internal disease. Histopathologic examination of skin lesions in all patients showed proliferation of large histiocytes with phagocytosed inflammatory cells characteristic of Rosai-Dorfman disease. However, the diagnoses of dermatofibroma, other spindle cell neoplasm, infectious granulomatous process, and other xanthohistiocytic proliferations were also considered due to the presence of storiform spindle cells and foamy cells in the first case. One patient experienced regression during a course of oral steroids, while another patient cleared spontaneously. In the absence of massive lymphadenopathy characteristic of Rosai-Dorfman disease, the diagnosis of purely cutaneous Rosai-Dorfman disease may be complicated by the rarity, non-specific clinical appearance of skin lesions, and broad histopathological differential diagnosis of this disorder. A high index of suspicion of the clinician and pathologist is often required.
窦性组织细胞增生伴巨大淋巴结病，即罗萨伊-多夫曼病，是一种良性特发性组织细胞增生性疾病，通常累及淋巴结，但其次也可能累及皮肤。然而，极少会出现无淋巴结病或内脏器官受累的单纯皮肤疾病。我们报告了3例患者的病例，这些患者表现为无症状的非特异性增大的皮肤结节，无淋巴结病或内科疾病证据。所有患者皮肤病变的组织病理学检查均显示大组织细胞增生，并吞噬有罗萨伊-多夫曼病特征性的炎性细胞。然而，由于第一例病例中存在漩涡状梭形细胞和泡沫细胞，也考虑过诊断为皮肤纤维瘤、其他梭形细胞瘤、感染性肉芽肿性病变以及其他黄色组织细胞增生性病变。1例患者在口服类固醇治疗过程中病情缓解，而另1例患者自行痊愈。由于罗萨伊-多夫曼病缺乏典型的巨大淋巴结病表现，单纯皮肤型罗萨伊-多夫曼病的诊断可能会因该病的罕见性、皮肤病变非特异性的临床表现以及广泛的组织病理学鉴别诊断而变得复杂。临床医生和病理医生通常需要保持高度的怀疑指数。
