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Genetic and Rare Diseases Information Center (GARD)

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Linear and whorled nevoid hypermelanosis


Other Names for this Disease

  • Linear and whorled hypermelanosis
  • LWNH
  • Nevoid hypermelanosis, linear and whorled
  • Reticulate hyperpigmentation of Iijima
  • Zebra-like hyperpigmentation
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.

Overview

What is linear and whorled nevoid hypermelanosis?

What are the signs and symptoms of linear and whorled nevoid hypermelanosis?

What causes linear and whorled nevoid hypermelanosis?

How might linear and whorled nevoid hypermelanosis be treated?

What is linear and whorled nevoid hypermelanosis?

Linear and whorled nevoid hypermelanosis is a skin condition characterized by swirling streaks of hyperpigmented (darkened) skin. The hyperpigmentation follows the lines of Blashko. The hyperpigmentation often occurs alone, but additional symptoms have been described in individual cases. Its occurrence is nearly always sporadic. The cause of linear and whorled nevoid hypermelanosis is not known.[1]
Last updated: 3/18/2013

What are the signs and symptoms of linear and whorled nevoid hypermelanosis?

Signs and symptoms of linear and whorled nevoid hypermelanosis include swirling streaks of hyperpigmented (darkened) skin.  The hyperpigmentation may or may not be apparent at birth, but tends to present by infancy or early childhood. Unlike other rare hyperpigmented skin conditions, the hyperpigmentation occurs without inflammation, blisters, or warty lesions. The hyperpigmentation follows the lines of Blashko and may involve part or much of the body (eyes, palms of the hands, soles of the feet, and mucous membranes are usually not affected). The skin symptoms may progress for one to two years before stabilizing. Children with linear and whorled nevoid hypermelanosis often have otherwise normal physical and neurologic development, however individual cases occurring along with neurologic, heart, growth, and skeletal abnormalities have been described.[2]
Last updated: 3/18/2013

What causes linear and whorled nevoid hypermelanosis?

The cause of linear and whorled nevoid hypermelanosis is not currently known. Around 45 cases have been described in the medical literature.[2][3] Nearly all have occurred sporadically, however apparent genetic transmission has been described in two families.[2][3]

Individual cases of linear and whorled nevoid hypermelanosis have occurred in association with an underlying chromosomal abnormality. People with linear and whorled nevoid hypermelanosis who also have a chromosome abnormality are more likely to have extra-skin symptoms which may impact learning, development, and growth.[2]
Last updated: 3/18/2013

How might linear and whorled nevoid hypermelanosis be treated?

There is very limited information regarding the treatment of linear and whorled nevoid hypermelanosis. Chemical peels and 2% hydroquinone for treatment of skin hyperpigmentation has been described.[2] Due to the possible association with chromosome abnormalities and other symptoms, each person with linear and whorled nevoid hypermelanosis should undergo a thorough evaluation for developmental delays, growth retardation, and skeletal anomalies, such as facial and body asymmetry, and congenital heart defects.[2]
Last updated: 3/18/2013

References
  1. Maruani A, Khallouf R, Machet MC, Lorette G. Diffuse linear and whorled nevoid hypermelanosis in a newborn. J Pediatr. 2012 Jan;160(1):171; http://www.ncbi.nlm.nih.gov/pubmed/21868034. Accessed 3/18/2013.
  2. Mehta V, Vasanth V, Balachandran C, Mathew M. Linear and whorled nevoid hypermelanosis. Int J Dermatol. 2011 Apr;50(4):491-2; http://www.ncbi.nlm.nih.gov/pubmed/21413967. Accessed 3/18/2013.
  3. Metta AK, Ramachandra S, Sadath N, Manupati S. Linear and whorled nevoid hypermelanosis in three successive generations. Indian J Dermatol Venereol Leprol. 2011 May-Jun;77(3):403; http://www.ncbi.nlm.nih.gov/pubmed/21508598. Accessed 3/18/2013.


Other Names for this Disease
  • Linear and whorled hypermelanosis
  • LWNH
  • Nevoid hypermelanosis, linear and whorled
  • Reticulate hyperpigmentation of Iijima
  • Zebra-like hyperpigmentation
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.