Incontinentia pigmenti: case report

Incontinent pigment is a rare inherited disorder, X chromosome-linked dominant The condition affects the newborn affecting the skin, hair and central nervous system, has a higher incidence in females, being lethal in most cases, when it affects males. The disease is characterized by cutaneous and extra skin, the former being more prevalent. The skin lesions are progressive evolving in four stages according to each phase of the disease diagnosis based on clinical and histological presentation. The good response to treatment in clinical report of a case of pigmentary incontinence motivated the authors to discuss the etiological aspects, diagnosis and treatment of disease.