Silvestre J F, Aliaga A
Department of Dermatology, Hospital General Universitario de Alicante, Spain.
Pediatr Dermatol. 2000 Sep-Oct;17(5):377-80. doi: 10.1046/j.1525-1470.2000.017005377.x.
Sinus histiocytosis with massive lymphadenitis or Rosai-Dorfman disease (RDD) is a rare, benign, proliferative histiocytic disease of unknown origin. It predominately affects the lymph nodes The skin is the extranodal organ most frequently involved, although a few patients present with only lesions of the skin and soft tissues, without involvement of the lymph nodes (purely cutaneous RDD). Ocular involvement is rare, and generally localized to the eyelid. We report an unusual case of cutaneous sinus histiocytosis without nodal involvement and with bilateral uveitis in a girl. As far as we know, this is the first case reported in which cutaneous and ocular RDD occurred without nodal involvement.
伴巨大淋巴结炎的窦性组织细胞增生症或罗萨伊-多夫曼病(RDD)是一种罕见的、良性的、起源不明的增生性组织细胞疾病。它主要累及淋巴结。皮肤是最常受累的结外器官,尽管少数患者仅表现为皮肤和软组织病变,而无淋巴结受累(纯皮肤型RDD)。眼部受累罕见,且通常局限于眼睑。我们报告了一例女童患无淋巴结受累且伴有双侧葡萄膜炎的皮肤窦性组织细胞增生症的不寻常病例。据我们所知,这是首例报道的无淋巴结受累的皮肤和眼部RDD病例。