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Linear and whorled nevoid hypermelanosis.

D C Kalter1, W A Griffiths, D J Atherton

  • 1St. John's Hospital for Diseases of the Skin, London, England.

Journal of the American Academy of Dermatology
|December 1, 1988
PubMed
Summary

Congenital linear and whorled hypermelanosis presents as gradual skin hyperpigmentation along Blaschko's lines. This rare condition, possibly due to somatic mosaicism, requires differentiation from other genetic disorders.

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Area of Science:

  • Dermatology
  • Medical Genetics

Background:

  • Congenital linear and whorled hypermelanosis (CLWH) is a rare pigmentary disorder.
  • It presents with characteristic patterns along Blaschko's lines.

Observation:

  • Two cases of CLWH are described, showing gradual onset of hyperpigmented macules after birth.
  • Histology revealed increased epidermal melanocytes and basal layer hyperpigmentation.

Findings:

  • The condition exhibits a distinct linear and whorled pattern, inversely mirroring hypomelanosis of Ito.
  • Developmental somatic mosaicism is a potential underlying mechanism for this patterned hyperpigmentation.

Implications:

  • Accurate diagnosis is crucial to differentiate CLWH from conditions like incontinentia pigmenti and epidermal nevi.
  • Sporadic incidence and potential association with other congenital anomalies warrant further investigation.

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