Indian Journal of Dermatology
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Year : 2006  |  Volume : 51  |  Issue : 1  |  Page : 65-66
Hypomelanosis of ITO

Department of Dermatology, Venereology and Leprosy, Father Muller Medical College, Mangalore - 575 002, Karnataka, India

Correspondence Address:
D Sukumar
Department of Dermatology, Venereology and Leprosy, Father Muller Medical College, Mangalore - 575 002, Karnataka
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0019-5154.25211

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How to cite this article:
Chethan C J, Ashique K T, Sukumar D, Nanda Kishore B. Hypomelanosis of ITO. Indian J Dermatol 2006;51:65-6

How to cite this URL:
Chethan C J, Ashique K T, Sukumar D, Nanda Kishore B. Hypomelanosis of ITO. Indian J Dermatol [serial online] 2006 [cited 2023 Oct 2];51:65-6. Available from:

Hypomelanosis of Ito, also known as Incontinentia Pigmenti Achromians (IPA), is a rare neurocutaneous syndrome clinically characterized by macular hypopigmented worls, patches and streaks resembling fountain spray splatters.[1] These skin lesions become apparent at birth or during childhood and normally are present on the trunk, occasionally on the extremities and rarely on the face.[2] Most patients have multisystem involvement and may show chromosomal mosaicism, the most common being neurological, muscular, skeletal and ocular.[3],[4]

A 14 year old boy from Kerala presented with asymptomatic whitish patches over the body since birth. On cutaneous examination he had multiple, bizarre, whirled, well defined, asymmetric, hypopigmented macules distributed bilaterally along the  Lines of Blaschko More Details over the back, arms and legs. General physical exmination revealed that the patient was mentally retarded since birth and had marked kyphoscoliosis, arm and leg length discrepancy, divergent squint, language retardation, splenomegaly. A 4 mm punch biopsy taken from a hypopigmented macule showed a marked decrease in melanocytes. Ultrasound abdomen revealed feature suggestive of multiple space occupying lesions in both kidney's with grade II parenchymal changes and clacification suggestive of neoplasm. Systolic murmur was present in the mitral and pulmonary areas. His complete haemogram was normal except for eosinophilia (70%) and nonmocytic hypochromic to microcytic hypochromic anemia with mild degree of anisopoikilocytosis.

Hypomelanosis of Ito is a neurocutaneous disorder characterized by a bizarre, bilateral, irregularly shaped leukoderma afecting the trunk and extremities and often associated with neurologic and musculoskeletal abnormalities.[5] Though originally described as a purely cutaneous disease subsequent reports have included 33% to 94% association with multiple extracutaneous manifestations mostly of the central nervous and musculoskeletal systems leading to frequent characterization as a neurocutaneous disorder.[6] The hypomelanotic macules of Hypomelanosis of Ito are usually present at birth but may appear in early infancy or childhood.[5] Clinically the lesions appear as a negative image of incontinentia pigmenti, distributed with a libateral, asymmetric, whirled or streaked "marble cake" pattern often in parallel arrway and along Blaschko'' lines.[5] The most severe complications concerned with the central nervous system are mental retardiation and epilepsy and both of these are present in the more than 50% of cases.

Moreover other complications can be observed in some patients which consists of ocular, musculoskeletal and oral alterations, hypotonia, macrocephalia, microcephalia, congenital cardiac malformations, urological and genital malformations and other rarer disorders.[7] Of 115 reported cases, abnormal chromosomal patterns have been found in 60.[8] The most common alterations include mosaic trisomy 18, diploidy/triploidy, mosacism for sex chromosome aneuploidey and tetrasomy 12 p.8 According to earlier studies on this pathological condition a marked reduction of melanocytes is detected in the hypopigmented areas.[9]

  References Top

1.Sybert VI. Hypomelanosis of Ito. Pediatr Dermatol 1990; 7:74-76.  Back to cited text no. 1    
2.Griebel V, Krageloh-mann I, Michaelis R. Hypomelanosis of Ito: report of four cases and survey of the literature. Neuropediatrics 1989; 20:234-37.  Back to cited text no. 2  [PUBMED]  
3.Sybert VP, Pagon RA, Donian M, et al . Pigmentary abnormalities and mosaicism for chromosomal aberration: Association with clinical features similar to hypomelanosis of Ito. J. Pediatr 1990; 116:581-686.  Back to cited text no. 3    
4.Cellini A, Morroni M, Simonettio, et al. Hypomelanosis of Ito: a case report with clinical and ultrastructural data. J. Acad Dermatol Venereol 1998 Jan; 10(1):73-6.  Back to cited text no. 4    
5.Mosher DB, Fitzpatrick TB, Ortonne JP et al. Hypomelanoses a Hypermelanoses. In: Freedberg Im; Eisen AZ, Wolff K, et al. eds. Fitzpatrick's Dermatolgoy in general medicine. 5th ed. United States of America; McGraw-Hill, 1999: 945-1017.  Back to cited text no. 5    
6.Ruggieri M, Pavone L. Hypomelanosis of Ito: Clinical syndrome or just Phenotypez. J. Child Neurol, 2000 Oct; 15(10):635-44.  Back to cited text no. 6    
7.Pascual-castrovieio I. Hypomelanosis of Ito. Neurologia. 1997 Aug-Sep; 12(7):300-5.  Back to cited text no. 7    
8.Sybert VP. Hypomelanosis of Ito: A description, not a diagnosis. J Invest Dermatol, 1994 Nov; 103(5 suppl):1415-35.  Back to cited text no. 8    
9.Cavallari V, Ussia AF, Siraguse et al . Hypomelanosis of Ito: electron microscopical observation on two new cases. J. Dermatol Sci. 1996 Oct; 13(1):87-92.  Back to cited text no. 9    

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