We herein report a two-month old girl. She had rashes and blisters on trunk and limbs at birth. Dermatological examination revealed reticulate pigmented spots scattered on trunk and extremities. Some dark red papules and blisters in different size were located on both interfemus and wrists. The bilsters had thick walls and the Nikolsky's sign was negative. Most of the lesions were distributed in a splash manner. His-topathologically, the hyperkeratesis, acanthosis and epidermal vesicles filled with eosinophils were found in epidermis, and a small number of lymphocytes and eosinophils infiltration in the superficial dermis capillary were reported. Based on the clinical and histological findings, the final diagnosis was neonatal Bloch-Sie-mens syndrome.%患儿,女,2个月。因躯干及四肢皮疹2个月就诊。皮肤科检查：躯干及四肢皮肤弥漫分布网状色素沉着斑。双股内侧、手腕部见暗红色大小不一丘疹、水疱,水疱壁厚,尼氏征阴性,部分水疱结痂。皮疹呈泼溅状分布。组织病理示：表皮角化过度,棘层增厚,表皮内见多房性水疱,疱液中可见大量嗜酸性粒细胞。真皮浅层毛细血管周围可见少量淋巴细胞及嗜酸性粒细胞浸润。诊断：新生儿色素失禁症。
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