Dermatologist - specialist in Hansenology by the Brazilian Society of Hansenology, Piracicaba, SP, Brazil.
Department of Pathology, Universidade Estadual de Campinas, Campinas, SP, Brazil.
An Bras Dermatol. 2022 Nov-Dec;97(6):778-782. doi: 10.1016/j.abd.2022.01.005. Epub 2022 Sep 21.
Congenital and self-healing Hashimoto-Pritzker reticulohistiocytosis is the benign variant of the Langerhans cell histiocytosis (LCH) group. It is characterized by multiple skin lesions (congenital or appearing during the first days after birth), without systemic manifestations and spontaneous resolution in days to months. The authors report the case of a boy with a single congenital leg skin lesion, a rare disease variant. Through histopathology, a dense skin infiltration of S100 protein-, CD1a-, CD207-immunomarked cells was found. KI67 index was high (62%). A complete spontaneous resolution occurred 07 days after the biopsy (25 days after birth). Monolesional disease, distal limb lesion, absence of lesions in the mucous membrane or seborrheic area, and less than 25 percent of LCs with Birbeck granules were said to be possible clues for a favorable prognosis in LCs histiocytosis. But, as a precautionary measure, the child will be followed up until at least 2 years of age.
先天性自愈性桥本-普里茨克网状组织细胞增生症是朗格汉斯细胞组织细胞增生症（LCH）组的良性变异。其特征为多发性皮肤损伤（先天性或出生后最初几天出现），无全身表现，数天至数月内自发消退。作者报告了一例男孩患有单个先天性腿部皮肤损伤，为罕见疾病变异。通过组织病理学，发现 S100 蛋白、CD1a、CD207 免疫标记细胞密集皮肤浸润。Ki67 指数高（62%）。活检后 07 天（出生后 25 天）完全自发消退。单病灶病变、肢体远端病变、粘膜或脂溢部位无病变、不到 25%的朗格汉斯细胞具有伯贝克颗粒被认为是朗格汉斯细胞组织细胞增生症预后良好的可能线索。但是，作为预防措施，将对患儿进行随访，直至至少 2 岁。
