Hypomelanosis of Itō associated with benign tumors and chromosomal abnormalities: A neurocutaneous syndrome

Pigmentary dysplasias and chromosomal mosaicism: Report of 9 cases

“…We are aware of 62 individuals with pigmentary dysplasias chromosomally analyzed since 1985, including the 9 individuals in the present series [Flannery et al, 1985;Fujimoto el al., 1985;Ishikawa et al, 1985;Miller and Parker, 1985;Rott et al, 1986;Turleau et al, 1986;Wertelecki et al, 1986;Happle, 1987;Glover et al, 1989;Cantu et al, 1989;Thomas et al, 1989;Sybert et al, 1990;Ritter et al, 1990;Chitayat et al, 1990;Muran0 et al, 1991;Wulfsberg et al, 19911. They included 37 chromosomally abnormal and 25 chromosomally normal individuals.…”

“…The hypo-or hyperpigmented lines often follow Blaschko lines on the back and the limbs. It is now clear that the disorder represents mosaicism of 2 kinds of melanocytes with different pigmentary potentials, due to genetic or chromosomal mosaicism or chimerism [Findlay and Moores, 1980;Hall, 1988;Thomas et al, 1989;Flannery, 19901. Interest in chromosomal analysis of the individuals with pigmentary dysplasia was aroused in 1985, when its association with chromosomal mosaicism was confirmed [Flannery et al, 1985;Fujimoto et al, 1985;Ishikawa et al, 1985;Miller and Parker, 19851. We are aware of at least 53 individuals with the disorder chromosomally analyzed since then.…”

Hypomelanosis of lto associated with neuroblastoma

“…Except for a limited number of cases associated with benign tumor, such as a mature mediastinal teratoma and diploic epidermoid [2], there has been only one report of hypomelanosis of Ito associated with malignancy. Tateno et al reported one case with acute lymphatic leukemia [3], but the association of hypomelanosis of Ito and solid malignant neoplasm has not been reported previously.…”

“…The consistent feature of this disease is a characteristic cutaneous hypopigmentation area following the lines of Blascko, but the clinical manifestations are varied [1]. Only a limited number of patients with neoplasm have been reported previously [2,3]. This is the first case of hypomelanosis of Ito associated with neuroblastoma.…”

Hypomelanosis of Ito with Growth Hormone Deficiency

“…Some neurocutaneous syndromes (neurofibromatosis of von Recklinghausen, tuberous sclerosis of Bouneville-Pringle) are known to be sometimes complicated by tumors. Ishikawa reported a case of HI with benign tumors (7). HGH therapy in HI should be given under close observation and needs careful follow-up.…”