De Dulanto Campos C, Camacho-Martínez F
Ann Dermatol Venereol. 1979 Apr;106(4):337-43.
We describe 7 observations of incontinentia pigmenti (0.0026 p. 100 of the patients visited in the Department of Medical-Surgical Dermatology, Faculty of Medicine, University of Granada). Two cases with congenital pigmentation and one of these with secondary inflammatory lesions. The electron microscopy shows relation between the classic stages of the process. In two patients there were defects of the Central Nervous System: brain paralysis and microcephaly. Only two patients with blood eosinophilia (13 p. 100-21 p. 100). Evolution benign, except in the case with brain paralysis. There were familiar antecedents in three women and these were not found in two women and two men with normal karyotype. It suggests a mutation. This study has shown a high rate men/women: 2/5; in comparison to other series. We think that it is possible for a mutation and that this disease is more frequent than it seems. It is necessary to think in this disease in all pigmentary abnormalities in children.
我们描述了7例色素失禁症病例（占格拉纳达大学医学院外科皮肤科就诊患者的0.0026%）。2例有先天性色素沉着，其中1例伴有继发性炎症性病变。电子显微镜显示了该过程经典阶段之间的关联。2例患者存在中枢神经系统缺陷：脑瘫和小头畸形。仅有2例患者出现血液嗜酸性粒细胞增多（占100%-21%）。除脑瘫患者外，病情进展良性。3名女性有家族病史，而2名女性和2名核型正常的男性未发现家族病史。这提示存在突变。本研究显示男性/女性比例较高：2/5；与其他系列研究相比。我们认为可能存在突变，且这种疾病比看起来更为常见。对于儿童的所有色素异常情况，都有必要考虑到这种疾病。
