Paquet P, Hermanns-Lê T, Piérard G E
Service de dermatopathologie, CHU du Sart Tilman, Liège, Belgique.
Arch Pediatr. 1994 Jun;1(6):578-81.
Congenital histiocytosis is divided into four entities differing in their clinical and histological features and prognosis. Early, accurate diagnosis is essential for treatment.
Seven cutaneous nodules were seen in a male neonate. One of these nodules was biopsied on the 7th day of life; it showed the typical findings of Hashimoto-Pritzker reticulohistiocytosis by optical and electron microscopy using immunological markers. The nodules spontaneously disappeared after a few months.
Congenital forms of histiocytosis must be accurately characterized. Hashimoto-Pritzker reticulohistiocytosis is a benign and self-healing disease restricted to the skin, while the Letterer-Siwe disease has a completely different course and treatment.
先天性组织细胞增多症分为四种类型，它们在临床、组织学特征及预后方面存在差异。早期准确诊断对治疗至关重要。
一名男新生儿身上出现七个皮肤结节。其中一个结节在出生后第7天进行了活检；通过光学显微镜、电子显微镜及免疫标记显示出典型的桥本 - 普利茨克网状组织细胞增多症表现。这些结节在数月后自行消失。
先天性组织细胞增多症的各种形式必须得到准确界定。桥本 - 普利茨克网状组织细胞增多症是一种局限于皮肤的良性自愈性疾病，而勒 - 雪病的病程和治疗则完全不同。
