Incontinentia Pigmenti in a Mother and her Daughter.

Seol,  Kye Hwan; Lee,  Gwang Hoon; Kim,  Gil Hyun; Lee,  Hak Soo; Lee,  Jeong Deuk

Original Article

Journal of the Korean Society of Neonatology 2001;8(2):276-280.
Published online November 1, 2001.

Incontinentia Pigmenti in a Mother and her Daughter.

Kye Hwan Seol, Gwang Hoon Lee, Gil Hyun Kim, Hak Soo Lee, Jeong Deuk Lee

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

모녀에서 발생한 색소실조증 1례

^, ^, ^, ^,

^¹
^²

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.

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