title: Incontinentia pigmenti.
abstract: 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.
authors.affiliation: 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.
authors.forename: Cláudia Schermann
authors.initials: CS
authors.lastname: Poziomczyk
authors.affiliation: Porto Alegre Health Sciences Federal University, Porto AlegreRS, Brazil, Medical Student at Porto Alegre Health Sciences Federal University (UFCSPA) - Porto Alegre (RS), Brazil.
authors.forename: Júlia Kanaan
authors.initials: JK
authors.lastname: Recuero
authors.affiliation: Porto Alegre Health Sciences Federal University, Porto AlegreRS, Brazil, Medical Student at Porto Alegre Health Sciences Federal University (UFCSPA) - Porto Alegre (RS), Brazil.
authors.forename: Luana
authors.initials: L
authors.lastname: Bringhenti
authors.affiliation: Porto Alegre Health Sciences Federal University, Pathology Post-graduation Program, Porto AlegreRS, Brazil, DDS - MSc (in course) at the Pathology Post-graduation Program at Porto Alegre Health Sciences Federal University (UFCSPA) - Porto Alegre (RS), Brazil.
authors.forename: Fernanda Diffini Santa
authors.initials: FD
authors.lastname: Maria
authors.affiliation: Santa Casa de Misericórdia de Porto Alegre, Ophthalmology Program, Porto AlegreRS, Brazil, MD - Resident in the Ophthalmology Program at Santa Casa de Misericórdia de Porto Alegre - Porto Alegre (RS), Brazil.
authors.forename: Carolina Wiltgen
authors.initials: CW
authors.lastname: Campos
authors.affiliation: Santa Casa de Misericórdia de Porto Alegre, Porto AlegreRS, Brazil, MD - Ophthalmologist with specialization in Pediatric Ophthalmology and Strabismus Licensed physician at Santa Casa de Misericórdia de Porto Alegre- Porto Alegre (RS), Brazil.
authors.forename: Giovanni Marcos
authors.initials: GM
authors.lastname: Travi
authors.affiliation: Santa Casa de Misericórdia de Porto Alegre Ophthalmology Service, Ophthalmology Service, Retina Sector, Porto AlegreRS, Brazil, MD - Ophthalmologist - Chief of the Retina Sector at Santa Casa de Misericórdia de Porto Alegre Ophthalmology Service - Porto Alegre (RS), Brazil.
authors.forename: André Moraes
authors.initials: AM
authors.lastname: Freitas
authors.affiliation: Rio Grande do Sul Federal University, Porto AlegreRS, Brazil, DDS - Specialist in Orthodontics and Facial Orthopedics at Rio Grande do Sul Federal University (UFRGS). PhD in Dentistry at Rio Grande do Sul Pontifical Catholic University (PUCRS) - Substitute Professor of the Discipline of Orthodontics Applied to Speech Therapy at Porto Alegre Health Sciences Federal University (UFCSPA) - Porto Alegre (RS), Brazil.
authors.forename: Marcia Angelica Peter
authors.initials: MA
authors.lastname: Maahs
authors.affiliation: Porto Alegre Health Sciences Federal University, Pathology Post-graduation Program, Porto AlegreRS, Brazil, MD, PhD at the Pathology Post-graduation Program at Porto Alegre Health Sciences Federal University (UFCSPA) - Adjunct Professor of Genetics at Porto Alegre Health Sciences Federal University (UFCSPA) - Porto Alegre (RS), Brazil.
authors.forename: Paulo Ricardo Gazzola
authors.initials: PR
authors.lastname: Zen
authors.affiliation: Porto Alegre Health Sciences Federal University, Basic Health Sciences Department, Porto AlegreRS, Brazil, Pharm. D. - PhD in Genetics and Molecular Biology at Rio Grande do Sul Federal University (UFRGS) - Adjunct Professor at the Basic Health Sciences Department at Porto Alegre Health Sciences Federal University (UFCSPA) - Porto Alegre (RS), Brazil.
authors.forename: Marilu
authors.initials: M
authors.lastname: Fiegenbaum
authors.affiliation: Porto Alegre Health Sciences Federal University, Speech Therapy Department, Porto AlegreRS, Brazil, Speech-Language Pathologist (SLPs) - Specialist in Orofacial Motricity and Dysphagia. MSc in Health Sciences - Assistant Professor at the Speech Therapy Department at Porto Alegre Health Sciences Federal University (UFCSPA) - Porto Alegre (RS), Brazil.
authors.forename: Sheila Tamanini de
authors.initials: ST
authors.lastname: Almeida
authors.affiliation: Porto Alegre Health Sciences Federal University, Pathology Postgraduation Program, Porto AlegreRS, Brazil, MD - PhD in Health Sciences at Rio Grande do Sul Federal University (UFRGS) - Head Professor of Dermatology and Professor of the Pathology Postgraduation Program at Porto Alegre Health Sciences Federal University (UFCSPA) - Porto Alegre (RS), Brazil.
authors.forename: Renan Rangel
authors.initials: RR
authors.lastname: Bonamigo
authors.affiliation: Porto Alegre Health Sciences Federal University, Porto AlegreRS, Brazil, MD - PhD in Pathology at Porto Alegre Health Sciences Federal University (UFCSPA)- Adjunct Professor of Dermatology at Porto Alegre Health Sciences Federal University (UFCSPA). Pediatric Dermatologist at Santo Antonio Children's Hospital and at Santa Casa de Misericórdia de Porto Alegre - Porto Alegre (RS), Brazil.
authors.forename: Ana Elisa Kiszewski
authors.initials: AE
authors.lastname: Bau
mesh_terms: D000505:Alopecia / Q000209:etiology / Q000473:pathology; D002386:Cataract / Q000209:etiology / Q000473:pathology; D002493:Central Nervous System Diseases / Q000473:pathology; D006801:Humans; D007184:Incontinentia Pigmenti / Q000150:complications / Q000473:pathology*; D008297:Male; D009154:Mutation; D012867:Skin / Q000473:pathology*; D014071:Tooth Abnormalities / Q000209:etiology / Q000473:pathology