Incontinentia pigmenti (IP) is an uncommon X-linked dominant genodermatosis characterized by four cutaneous stages and frequent association with dental (90 %), central nervous system (33%) and ocular (35 %) anomalies. The exact pathogenesis of this disorder remains unknown.Herein, we report a newborn girl with inflammatory vesiculobullous and warty skin lesions and a positive family history of IP, with blood leucocytosis (eosinophilia and also lymphocytosis), but no other extracutaneous findings.

Objective: Incontinentia pigmenti (IP) (Bloch_Sulzberg syndrome) is a rare neurocutaneous syndrome characterized by multisystemic involvement that is prenatally lethal in the majority of affected males and shows great clinical variability when expressed in women. The diagnosis of IP is performed based on clinical features and the family history with the support of histological findings.We report a 10-day-old female neonate with typical skin lesions and frequent seizure. Skin biopsy showed second stage IP.

Purpose: This case study reports on an infant presented with strabismus and poor red reflex, suspected of retinoblastoma, and finally diagnosed as a case of Incontinentia pigmenti (IP). Case presentation: A 7-month-old infant was referred due to poor fixation and abrupt red reflex of the left eye for further evaluation of retinoblastoma. In anterior segment examinations, we found a micro cornea, a relatively shallow anterior chamber, and hypotonia. Fundus examination revealed a total tractional retinal detachment with vitreous membranes. In her skin examination, we discovered diffuse cutaneous linear pigmentation (lines of Blaschko), consistent with Incontinentia pigmenti (IP). Discussion: A wide range of disorders involving the fundus, cause similar signs and symptoms to retinoblastomas, such as leukocoria and poor red reflex, categorized as pseudo retinoblastoma. IP is a dysplastic ectodermal disorder with dominant X-linked inheritance, affecting integumentary, ocular, nervous, and dental tissues, and is responsible for less than 1% of cases of pseudo retinoblastoma. Although rare, achieving early diagnosis for IP is of utmost importance, since not only would it enable clinicians to treat and manage retinal complications, but it would also help them detect potentially fatal neurological issues.

Multicystic dysplastic kidney is a congenital kidney malformation consisting of multiple cysts of various sizes without a normal kidney morphology. Incontinentia pigmenti is a rare X-linked dominant genodermatosis, which is usually lethal in males, that presents clinically in 4 stages. Here, we report a case of multicystic dysplastic kidney with ureterovesical junction obstruction and incontinentia pigmenti. Coexistence of these two rare diseases may be a coincidental phenomenon or an association between the two may exist.

Introduction: Incontinentia pigmenti (IP) is a rarely diagnosed x-linked dominant disease affecting tissues of ectodermal and mesodermal origin such as cutaneous tissues, teeth, eyes, hair, and the central nervous system. Dermatologic manifestations are often the first signs observed in patients diagnosed with IP and are present in nearly all the subjects, but they are less harmful and do not require treatment. Oral manifestations in patients diagnosed with IP might affect both the deciduous and permanent teeth, with tooth shape anomalies and hypodontia, delayed tooth eruption, cleft palate, and high arched palate. These oral abnormalities influence feeding, quality of life (QoL), and self-esteem of the patient but can be successfully corrected by oral rehabilitation. Case Presentation: Here, we report the case of a female patient, aged 11 years, presenting with dental manifestations such as hypodontia, conical teeth, delayed tooth eruption, narrow and atrophic dental ridge, and also some non-dental findings of IP. Her dental management included oral hygiene instructions, extraction of all unrestorable primary molars, and composite filling of all primary canines. Conclusions: A removable space maintainer was constructed for the patient, which resulted in favorable esthetic outcomes, proper re-establishment of mastication, and improved self-esteem.

Background: Incontinentia pigmenti (IP), also known as Bloch-Sulzberger syndrome, is a rare X-linked dominant genodermatosis that presents at the time of birth or soon after birth with cutaneous manifestation. This disorder may also affect the ectodermal tissues, such as the central nervous system, skeletal system, eyes, hair, nails, and teeth. The dermatological findings occur in four successive phases.Case report: Herein, we presented the case of a two day-old female newborn with inflammatory vesiculopustular lesions on the right forearm and lower limbs, who was in a good general condition. The patient had a history of similar disease in three other members of her family, who had dental abnormalities as the most common non-cutaneous manifestation. This case report highlighted the importance of a detailed diagnostic workup for the newborns with pustular skin disease.Conclusion: IP is a rare, x-linked dominant genodermatosis with multiple organs involvement. Dermatological abnormalities are the most prominent manifestation. The diagnosis is based on the clinical findings, the presence of positive family history of skin vesiculopustular lesions support the diagnosis. The skin lesions do not require specific treatment and prognosis depend to other organs involvement.

Incontinentia pigmenti is a rare inherited disorder involving abnormalities of the skin, hair, eyes, musculoskeletal system, central nervous system, and the teeth. Dental abnormalities are the most common manifestations of this disorder. The purpose of this case report was to present the clinical and radiological findings of a seven-year-old girl as well as the results of her five-year follow up. The patient showed faded linear pigmented macular lesions on the trunk and on upper and lower limbs. Dental examination was notable for conical and peg-shaped anterior teeth as well as delayed eruption of primary and permanent teeth. In addition to conservative treatments, prosthetic treatments such as interim removable partial dentures were indicated for the patient.