• Journal of Yeungnam Medical Science
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• v.15 no.2
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• pp.381-390
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• 1998

Incontinentia pigmenti is characterized by irregular linear blisters with erythematous lesions which are developed from birth or later, verrucous papules on the extremities which are noted after a few weeks or months, and the appearance of streaks, spots and swirls of hyperpigmentation. This disorder is known as a systemic disorder caused by a defect at the developmental stage of organs originated from ectoderm or mesoderm. We experienced an incontinentia pigmenti in a male newborn infant who did not have any family history and had cutaneous lesions, which were in bullous and verucous stages, ophthalmic problems, and neurologic abnormalities.

• Journal of the korean academy of Pediatric Dentistry
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• v.36 no.4
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• pp.563-567
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• 2009

Incontinentia pigmenti(Bloch-Sulzberger syndrome) is a sex-linked hereditary disorder so girls are almost exclusively affected. The frequency rate is approximately 1:40,000 among girls. It is associated with skin, ocular, dental, skeletal and central nervous deformities. We reported 2 cases of medically diagnosed IP patients who were 4 and 5 years old girls. They had thin and sparse hair, and represented congenital missing of multiple primary and permanent teeth, accessory cusps and cone-shaped crowns. Therefore we report the dental manifestations and treatment progress.

• Journal of the korean academy of Pediatric Dentistry
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• v.32 no.4
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• pp.682-686
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• 2005

Incontinentia pigmenti(IP), so called Block-Sulzberger syndrome is a rare genodermatosis that occurs almost in female infant; usually lethal in males, X-linked dominantly inherited disorder. IP is characterized by abnormalities of mesodermal and ectodermal tissues including eye, tooth, skin, nail, breast and hair as well as neurological deficiencies. Dental problems are congenital missing of teeth, delayed eruption, abnormal crown shape and so on. Here is a case of 6 year-old female with IP. She had congenital missing of primary and permanent teeth, delayed eruption, maxillary deficiency and extra cusps, resulting in unstable occlusion. Systemically, she had a history of operating eyes due to problem of retina and hyperpigmented macules on her trunk and extremities as typical character of IP.

• Journal of the korean academy of Pediatric Dentistry
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• v.42 no.2
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• pp.180-187
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• 2015

Incontinentia pigmenti, also called Bloch-Sulzberger syndrome, is a rare X-linked inherited dominant disorder that affects females, but causes spontaneous abortion of prenatal males. Incontinentia pigmenti is a systemic disease with clinical features similar to ectodermal dysplasia, including congenitally missing teeth. The pathogenesis is related to gene mutations in NF-kappa-B essential modulator on chromosome Xq28. Incontinentia pigmenti is caused by a defect in the developmental stage of organs originating from the ectoderm or mesoderm and involves the skin, eyes, hair, teeth and central nervous system. This report discusses the management of three cases of 3 to 5 years old females with incontinentia pigmenti and accompanying multiple missing teeth. The cases had sparse hair, and showed oligodontia and anomalous crowns with supplementary cusps in the posterior teeth and conical anterior teeth. Removable space maintainers were applied, achieving improved esthetics, recovery of mastication and increased self-esteem in the patients.

• The Journal of Korea Assosiation for Disability and Oral Health
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• v.14 no.2
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• pp.88-91
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• 2018

Incontinentia pigmenti(IP), or Block-Sulzberger syndrome is a rare X-linked dominant genodermatosis that affects almost in female infant and is usually lethal for males in utero. IP is characterized by four cutaneous stages and is frequently associated with dental, ocular, central nervous system and structural anomalies. Dental problems are congenital missing of teeth, delayed eruption, abnormal crown shape. We reported a case of 5 year-old female with IP. She had congenital missing of multiple primary and permanent teeth, accessory cusp and cone-shaped crowns. Systemically, she had a problem of retina and hyperpigmented macules on her trunk and extremities as typical character of IP. As the syndrome involves many different medical problems and needs comprehensive consideration. Dental care should be performed in a multidisciplinary consultation system.

• Clinical and Experimental Pediatrics
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• v.45 no.4
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• pp.535-539
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• 2002

Incontinentia pigmenti is a rare neurocutaneous syndrome characterized by vesiculobullous skin disease in neonates and infants, a noninfectious disease that should be distinguished from infectious diseases with the neonatal seizure or encephalopathy. This disease is X-linked dominant with Xq28 region abnormalities and often associated with developmental defects of the ocular, skeletal, dental, and central nervous system. Central nervous system involvement in the neonatal period, or complicated by encephalopathy, may cause severe neurologic impairment, retardation or even death. We experienced a case of incontinentia pigmenti in a three-day-old female patient who had characteristic papulovesicular skin lesions and partial seizures with secondary generalization. Histopathological examination favored the diagnosis of incontinentia pigmenti and a brain MRI showed undifferentiated white matters with periventricular nodular lesions.