All issues > Volume 34(2); 1991

Original Article

J Korean Pediatr Soc. 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

Received: June 26, 1990;  Accepted: September 19, 1990.

Abstract

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.

Keywords :Hypomelanosis of Ito;Hypomelantic Scalp Hair;Ureteral duplication