Poziomczyk Cláudia Schermann, Recuero Júlia Kanaan, Bringhenti Luana, Maria Fernanda Diffini Santa, Campos Carolina Wiltgen, Travi Giovanni Marcos, Freitas André Moraes, Maahs Marcia Angelica Peter, Zen Paulo Ricardo Gazzola, Fiegenbaum Marilu, Almeida Sheila Tamanini de, Bonamigo Renan Rangel, Bau Ana Elisa Kiszewski
Porto Alegre Health Sciences Federal University, Pathology Post-graduation Program, Porto AlegreRS, Brazil, MD, Dermatologist - MSc (in course) at the Pathology Post-graduation Program at Porto Alegre Health Sciences Federal University (UFCSPA) - Porto Alegre (RS), Brazil.
Porto Alegre Health Sciences Federal University, Porto AlegreRS, Brazil, Medical Student at Porto Alegre Health Sciences Federal University (UFCSPA) - Porto Alegre (RS), Brazil.
An Bras Dermatol. 2014 Jan-Feb;89(1):26-36. doi: 10.1590/abd1806-4841.20142584.
Incontinentia pigmenti is a rare genodermatosis in which the skin involvement occurs in all patients. Additionally, other ectodermal tissues may be affected, such as the central nervous system, eyes, hair, nails and teeth. The disease has a X-linked dominant inheritance pattern and is usually lethal to male fetuses. The dermatological findings occur in four successive phases, following the lines of Blaschko: First phase - vesicles on an erythematous base; second phase - verrucous hyperkeratotic lesions; third phase - hyperchromic spots and fourth phase - hypochromic atrophic lesions.
色素失禁症是一种罕见的遗传性皮肤病，所有患者均会出现皮肤受累。此外，其他外胚层组织也可能受到影响，如中枢神经系统、眼睛、毛发、指甲和牙齿。该疾病具有X连锁显性遗传模式，通常对男性胎儿具有致死性。皮肤表现按照Blaschko线依次出现四个阶段：第一阶段——红斑基底上的水疱；第二阶段——疣状角化过度性病变；第三阶段——色素沉着斑；第四阶段——色素减退性萎缩性病变。
