Korean J Dermatol.  2002 May;40(5):574-576.

A Case of Incontinentia Pigmenti Associated with Right Cerebral Hemiatrophy

Affiliations
  • 1Department of Dermatology, Catholic University of Daegu School of Medicine, Daegu, Korea.

Abstract

Incontinentia pigmenti is an uncommon genodermatosis presented by characteristic cutaneous stages and frequent association with one or more ectodermal or mesodermal anomalies. A new-born girl, 2 days old, had had crops of erythematous papules and vesicles on the trunk and extremities. At 2 years of age, she had the whorled hyperpigmentation. There were no anomalous features in teeth, eyes and hair, but she had a neurologic disorder. Left hemiplegia, related to upper motor neuron weakness, showed brisk left DTR, spastic Achilles tendon with walking disorders, decreased grasping power of left hand and weakness of left upper extremity. Brain MRI revealed marked loss of volume in the white matter of the right lateral ventricle in T1 weighted images. We report herein an explicit case of incontinentia pigmenti associated with right cerebral hemiatrophy.

Keyword

Incontinentia pigmenti; Right cerebral hemiatrophy

MeSH Terms

Achilles Tendon
Brain
Ectoderm
Extremities
Female
Hair
Hand
Hand Strength
Hemiplegia
Humans
Hyperpigmentation
Incontinentia Pigmenti*
Lateral Ventricles
Magnetic Resonance Imaging
Mesoderm
Motor Neurons
Muscle Spasticity
Nervous System Diseases
Tooth
Upper Extremity
Walking
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