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Progressive, Persistent, Hyperkeratotic Lesions in Incontinentia Pigmenti

Progressive, Persistent, Hyperkeratotic Lesions in Incontinentia Pigmenti Abstract 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 References 1. Carney RG: Incontinentia pigmenti: A world statistical analysis . Arch Dermatol 1976;112:535-542.Crossref 2. Mascaro JM, Palou J, Vives P: Painful subungual keratotic tumors in incontinentia pigmenti . J Am Acad Dermatol 1985; 13:913-918.Crossref 3. Simmons A, Kegel M, Scher R, et al: Subungual tumors in incontinentia pigmenti . Arch Dermatol 1986;122:1431-1434.Crossref 4. Dahl MV, Matula G, Leonards R, et al: Incontinentia pigmenti and defective neutrophil chemotaxis . Arch Dermatol 1975; 111:1603-1605.Crossref 5. O'Brien J, Feingold M: Incontinentia pigmenti . AJDC 1985; 139:711-712. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Dermatology American Medical Association

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Publisher
American Medical Association
Copyright
Copyright © 1988 American Medical Association. All Rights Reserved.
ISSN
0003-987X
eISSN
1538-3652
DOI
10.1001/archderm.1988.01670010019011
Publisher site
See Article on Publisher Site

Abstract

Abstract 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 References 1. Carney RG: Incontinentia pigmenti: A world statistical analysis . Arch Dermatol 1976;112:535-542.Crossref 2. Mascaro JM, Palou J, Vives P: Painful subungual keratotic tumors in incontinentia pigmenti . J Am Acad Dermatol 1985; 13:913-918.Crossref 3. Simmons A, Kegel M, Scher R, et al: Subungual tumors in incontinentia pigmenti . Arch Dermatol 1986;122:1431-1434.Crossref 4. Dahl MV, Matula G, Leonards R, et al: Incontinentia pigmenti and defective neutrophil chemotaxis . Arch Dermatol 1975; 111:1603-1605.Crossref 5. O'Brien J, Feingold M: Incontinentia pigmenti . AJDC 1985; 139:711-712.

Journal

Archives of DermatologyAmerican Medical Association

Published: Jan 1, 1988

References