Nevo sebáceo de Jadassohn. Informe de un caso

Introduction. Jadassohn's nevus sebaceous or epidermal nevus syndrome is a disease of unknown etiology. It is usually present at birth and characterized by hyperpigmentation and hyperkeratosis lesions, most commonly observed in the midfacial area, from the forehead down into the nasal area, tending to be linear in distribution. These lesions may also affect trunk and limbs. Other abnormalities are moderate to severe mental deficiency and seizures, cranial asymmetry, spotty alopecia, coarctation of aorta, cryptorchism, asymmetric overgrowth, scoliosis, and ocular abnormalities including palpebral coloboma, dermoids and corneal leucomas. Case report. This study reports the case of a 5 year-old girl who presented since birth hyperpigmentation from the chin down into the thorax and limbs with linear distribution. She also had corneal leucomas, dermolipomas, left lid coloboma and areas of alopecia. Conclusion. The histopathologic studies of an alopecic area of the scalp confirmed the diagnosis of Jadassohn's nevus sebaceous characterized by immature hair follicles and absence of sebaceous glands.