Illustrations
Figure 1
A , B ) Clinical appearance of the skin lesions at the first visit. A ) A rectangular patch measuring 20 cm×12 cm with a poorly defined border on the inner side of the right thigh. The rectangular patch consisted of a cluster of light violet-red macules, less than 0.5 cm in diameter, or papules. On the cranial side of the rectangular patch, there was a violet-red plaque, measuring 6.5 × 3.5 × 0.5 cm. Diffuse erythema was present on both cheeks. B ) Erythematous papules were scattered on the erythema. C -G ) Histopathological findings of the nodule on the right thigh. C ) Two kinds of region (*, **) were observed in the dermis (haematoxylin and eosin; original magnification: ×40). D ) The region indicated by * shows a dense infiltrate of large macrophages (haematoxylin and eosin; original magnification: ×400). E ) The region indicated by ** shows a dense infiltrate of lymphocytes and plasma cells (haematoxylin and eosin; original magnification: ×400). F ) Macrophages in (D ) were positive for S100 protein (original magnification: ×400). G ) Some macrophages showed emperipolesis (arrows) (original magnification: ×1,000). H , I ) Histopathological findings of a papule on the right cheek. H ) A nodular infiltration of macrophages, intermingled with diffuse infiltration of lymphocytes and neutrophils, was present in the superficial to middle dermis (haematoxylin and eosin; original magnification: ×40). I ) Macrophages in (H ) were positive for S100 protein (original magnification: ×400). J , K ) Slit-lamp findings of the left eye at the first examination. Bulbar ciliary injection (J ) and mutton-fat keratic precipitates (arrow heads) (K ) due to anterior granulomatous uveitis were noted.
Figure 1
Auteurs
1 Division of Dermatology, Department of Internal Related, Kobe University Graduate School of Medicine, 7-5-1 Kusunoki-cho, Chuo-ku, Kobe 650-0017,
2 Department of Infection and Host Defense, Graduate School of Medicine, Chiba University, 1-8-1 Inohara, Chuo-ku, Chiba 260-8670,
3 Department of Dermatology, Kobe Accident Hospital, 4-1-23 Kagoike-dori, Chuo-ku, Kobe 651-0053,
4 Division of Ophthalmology, Department of Surgery, Kobe University Graduate School of Medicine, 7-5-1 Kusunoki-cho, Chuo-ku, Kobe 650-0017, Japan
Rosai-Dorfman disease (RDD) was originally described as a rare pseudolymphomatous disorder, characterised by persistent massive lymphadenopathy and caused by increased numbers of macrophages within lymph node sinuses [1, 2]. These macrophages are often large and exhibit abnormal behaviour, taking intact cells, such as lymphocytes, erythrocytes, and plasma cells, into the cytoplasm, as a phenomenon known as emperipolesis. Population sites of these abnormal macrophages are not confined to lymph node [...]