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J Korean Acad Pediatr Dent. 2005;32(4): 682-686.
INCONTINENTIA PIGMENTI (BLOCH-SULZBERGER SYNDROME) WITH DENTAL PROBLEMS : CASE REPORT
이상엽, 김대업, 이광희
원광대학교 치과대학 소아치과학교실 원광치의학연구소
Corresponding Author: 이광희 ,
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ABSTRACT
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.
Keywords: Incontinetia pigmenti | Block-Sulzberger syndrome | Congenital missing | Ectodermal | Hyperpigmented macules
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