J Korean Soc Neonatol.  2001 Nov;8(2):276-280.

Incontinentia Pigmenti in a Mother and her Daughter

Affiliations
  • 1Department of Pediatrics, Gachon Medical School, Inchon, Korea.
  • 2Department of Dermatology, Gachon Medical School, Inchon, Korea.

Abstract

Incontinentia pigmenti (IP) is a rare multisystemic ectodermal disorder, which is characterized by vesicular, verrucous, and pigmented cutaneous lesions, and is frequently associated with various developmental defects of the eye, CNS, teeth, hair, and nail. It is regarded as an X-linked dominant genetic disorder. We recently experienced a case with IP, who presented with irregular, reticular, and slate-gray to brown colored pigmentation on the whole body at birth. Skin lesions were much improved by 6 month of age. The mother of this infant had the history of same cutaneous lesions in her neonatal period, suggesting that these lesions had familial tendency.

Keyword

Incontinentia pigmenti; Bloch-Siemens-Sulzberger syndrome; X-linked dominant inheritance

MeSH Terms

Ectoderm
Hair
Humans
Incontinentia Pigmenti*
Infant
Mothers*
Nuclear Family*
Parturition
Pigmentation
Skin
Tooth
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