HYPOMELANOSIS OF ITO - REPORT OF 3 CASES AND REVIEW OF THE LITERATURE

  • 1 January 1986
    • journal article
    • research article
    • Vol. 113  (1) , 15-23
Abstract
Initially described as incontinentia pigmenti achromians, Ito''s hypomelanosis is a congenital disease characterized dermatologically by depigmented maculae arranged in a specific pattern. These maculae appear suddenly, unheralded by an inflammatory process, and are arranged on the limbs as lines and on the trunk as whirlwinds or mottled cakes. Other abnormalities, notably neurological, ophthalamic or musculoskeletal may be associated with this spray-like depigmentation of the skin. We report here three cases of this disease, which is probably more common than the scarcity of cases hitherto published would suggest. A 9-year-old boy presented since the age of 5 with generalized convulsive fits predominant on the right side. Neurological examinations between fits were negative. IQ, FO and CT scans of the brain were normal. During the first months of life, the child had developed a spray-like depigmentation on the right half of his back and on the anterior and posterior aspects of his right arm. A 2-year-old girl was examined for a cutaneous depigmentation which had developed when she was about 3 months'' old and had progressively extended from her left knee to her left hypochondrium. An 8-year-old girl presented with mental retardation and myopia, but also with a spray-like depigmentation on the left part of her chest. The skin lesion had been noticed by her parents after she had exposed herself to the sun in a tropical country. Histological examination performed in the first two patients showed some degree of hypopigmentation of the epidermis without pigmentary incontinence. At electron microscopy the melanocytes were found to contain very few and small melanosomes. Ito''s hypomelanosis is seldom described in the literature. The sex ratio is about 2 to 5. The skin lesions are usually present at birth or appear in the first year of life; they are more obvious under Wood''s light. Ultrastructural examinations are too few for a consistent general picture to be drawn, but the anomaly essentially lies in the melanocytes, leading to the formation of abnormal melanosomes.

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