Caputo R, Grimalt R, Ermacora E, Cavicchini S, Portaleone D
First Department of Dermatology and Pediatric Dermatology, University of Milan, Italy.
J Am Acad Dermatol. 1994 Feb;30(2 Pt 2):367-70. doi: 10.1016/s0190-9622(94)70042-7.
A 28-month-old boy had firm, red nodules for 4 months that were mostly localized to the face. The eruption was preceded by conjunctivitis, eyelid edema, and swelling of the root of the nose. He also had dyspnea, anisocoria with areflexia, swelling of the parotid glands, and hepatosplenomegaly. A bone marrow aspirate showed 25% eosinophils. A skin biopsy specimen revealed a lymphohistiocytic infiltrate that involved the entire dermis. Most of the cells expressed antimacrophage markers; S-100 was negative. Electron microscopy showed poorly differentiated histiocytes without any specific marker. Steroid therapy induced complete clearing. Two years later, after several cutaneous recurrences, the patient is free of disease. We believe that this case represents a nodular, benign non-Langerhans cell histiocytosis with cutaneous lesions that differ from previously described histiocytopathies.
一名28个月大的男孩出现坚实的红色结节4个月，主要局限于面部。皮疹出现前有结膜炎、眼睑水肿和鼻根部肿胀。他还伴有呼吸困难、瞳孔不等大伴反射消失、腮腺肿大和肝脾肿大。骨髓穿刺显示嗜酸性粒细胞占25%。皮肤活检标本显示淋巴细胞组织细胞浸润累及整个真皮层。大多数细胞表达抗巨噬细胞标志物；S-100阴性。电子显微镜显示低分化组织细胞，无任何特异性标志物。类固醇治疗后皮疹完全消退。两年后，虽有几次皮肤复发，但患者目前无病。我们认为该病例代表一种结节性、良性非朗格汉斯细胞组织细胞增多症，其皮肤病变与先前描述的组织细胞病不同。
