| A case of hypomelanosis of Ito accompanying ureteral duplication and hypomelanotic scalp hair. |
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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 |
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손동우1, 박범수1, 한헌석1, 정해일1, 최용1, 문형로1, 김선훈2, 최황3 |
1서울대학교 의과대학 소아과학교실
2서울대학교 의과대학 피부과학교실
3서울대학교 의과대학 비뇨기과학교실 |
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Received: 26 June 1990 • Accepted: 19 September 1990 |
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| Abstract |
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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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