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January 1988

Progressive, Persistent, Hyperkeratotic Lesions in Incontinentia Pigmenti

Author Affiliations

Department of Dermatology Academic Hospital Maastricht PO Box 1918 6201 BX Maastricht the Netherlands

Arch Dermatol. 1988;124(1):29-30. doi:10.1001/archderm.1988.01670010019011

To the Editor.—  Incontinentia pigmenti (IP) or Bloch-Sulzberger syndrome is a multiorgan disease with an X-linked dominant inheritance. Incontinentia pigmenti most frequently affects females and is usually lethal in males. Clinically, three stages of skin lesions in IP may be distinguished, which occur successively soon after birth, disappear gradually, and generally clear completely by maturity. This triphasic cutaneous eruption can be accompanied by a wide variety of developmental disorders.1 We describe a 24-year-old woman with the classic clinical and histopathologic picture of IP who continued to show hyperkeratotic, verrucous lesions as an adult.

Report of a Case.—  Soon after birth, linearly arranged erythematous, vesicular, and bullous lesions appeared on the trunk and extremities of our patient, which later developed into hyperkeratotic lesions and finally progressed into reticular pigmentations. This picture was associated with blood eosinophilia (54% of total leukocytes) in the first months of life. In this period our

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