Journal of the Korean Pediatric Society 1991;34(2):281-286.
Published online February 28, 1991.
A case of hypomelanosis of Ito accompanying ureteral duplication and hypomelanotic scalp hair.
Dong Woo Son1, Beom Soo Park1, Heon Seok Han1, Hae Il Jung1, Yong Choi1, Hyung Ro Moon1, Seon Hoon Kim2, Hwang Choi3
1Department of Pediatrics, Seoul National University College of Medicine, Seoul, Korea
2Department of Pediatric Dermatology, Seoul National University College of Medicine, Seoul, Korea
3Department of Pediatric Urology, Seoul National University College of Medicine, Seoul, Korea
중복요관과 두발의 저색소증을 동반한 Ito 저멜라닌증 1례
손동우1, 박범수1, 한헌석1, 정해일1, 최용1, 문형로1, 김선훈2, 최황3
1서울대학교 의과대학 소아과학교실
2서울대학교 의과대학 피부과학교실
3서울대학교 의과대학 비뇨기과학교실
Received: 26 June 1990   • Accepted: 19 September 1990
Hypomelanosis of Ito (incontinentia pigmenti achromians) is a cutaneous abnormality consisting of bizarre, whorly, linear, or patchy hypopigmentation over variable portion of body surface. Multiple assocaited defects in other systems occur in three quarters of the affected individuals. Most common- ly, the central nervous system, eye, and musculoskeletal structures are involved. It is suggested that the cutaneous abnormality, which is often detectable at birth or during infancy, may forewarn pediatricians of the possible emergence of defects in other organ systems. We have experienced a case of hypomelanosis of Ito accompanying hypomelanosis of hair color and ureteral duplication. The involvement of hair in hypomelanosis of Ito as hypomelanotic hair is reported for the first time. The patient, a 2-year-5-month -old girl, was the product of non-related parents. At birth, linear or whorl like depigmented macules of bizarre irregularity on whole body surface were found. As she grew up, the depigmented macules on her lower extremities became less evident, but those on trunk and upper extremities became more vivid. Concomittantly, scalp hair became hypomelanotic result- ing a blond-like appearance. Extracutaneous manifestations were developmental delay, language retardation, and toe-out gait. Intravenous pyelogram and voiding cystoureterogram disclosed right ureteral duplication. The upper pole ureter formed ureterocele in urinary bladder. Ureteroceletomy and reimplantation of ureter was performed successfully.
Key Words: Hypomelanosis of Ito, Hypomelantic Scalp Hair, Ureteral duplication

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