Linear and whorled nevoid hypermelanosis
Prevalence estimates on Rare Medical Network websites are calculated based on data available from numerous sources, including US and European government statistics, the NIH, Orphanet, and published epidemiologic studies. Rare disease population data is recognized to be highly variable, and based on a wide variety of source data and methodologies, so the prevalence data on this site should be assumed to be estimated and cannot be considered to be absolutely correct.
Age of Onset
Autosomal dominant ?A pathogenic variant in only one gene copy in each cell is sufficient to cause an autosomal dominant disease
Autosomal recessive ?Pathogenic variants in both copies of each gene of the chromosome are needed to cause an autosomal recessive disease and observe the mutant phenotype
X-linked dominant ?X-linked dominant inheritance, sometimes referred to as X-linked dominance, is a mode of genetic inheritance by which a dominant gene is carried on the X chromosome.
X-linked recessive ?Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder
Mitochondrial or multigenic ?Mitochondrial genetic disorders can be caused by changes (mutations) in either the mitochondrial DNA or nuclear DNA that lead to dysfunction of the mitochondria and inadequate production of energy.
Multigenic or multifactor ?Inheritance involving many factors, of which at least one is genetic but none is of overwhelming importance, as in the causation of a disease by multiple genetic and environmental factors.
Other Names (AKA)
LWNH; Nevoid hypermelanosis, linear and whorled; Linear and whorled hypermelanosis;
Congenital and Genetic Diseases; Skin Diseases
Linear and whorled nevoid hypermelanosis (LWNH) is a rare benign condition affecting skin pigment occurring in newborns or early infancy. The primary symptom is swirling streaks of darkened (hyperpigmented) skin, mainly on the trunk and limbs. The dark streaks appear along the lines of normal skin development (lines of Blaschko). LWNH typically spreads for one to two years and then becomes stable. The dark streaks sometimes fade with age. Most of the time, only the skin is affected, but other conditions, such as growth, developmental, or heart problems may be present. The cause for LWNH is unknown. Genetic factors, especially chromosomal mosaicism, may be involved. LWNH is not thought to be
- Swirling streaks of darkened (hyperpigmented) skin
- Asymmetric growth
- Cardiovascular defects
The dark skin streaks appear in early infancy and are generally found on the trunk and limbs. The face, palms of the hand, and soles of the feet are not generally affected. The hyperpigmentation may get progress for a few years, and then become stable.. In some people, the darkened skin streaks fade with time. Most people with LWNH have no medical problems related to their condition.
This table lists symptoms that people with this disease may have. For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed. This information comes from a database called the Human Phenotype Ontology (HPO) . The HPO collects information on symptoms that have been described in medical resources. The HPO is updated regularly. Use the HPO ID to access more in-depth information about a symptom.
|Medical Terms||Other Names||
|Percent of people who have these symptoms is not available through HPO|
High blood eosinophil count
Onset in first year of life
Onset in infancy
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No previous family history
One specialist who might be involved in the care of someone with LWNH is a
Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.
These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.
- The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
- Online Mendelian Inheritance in Man (OMIM) is a catalog of human genes and genetic disorders. Each entry has a summary of related medical articles. It is meant for health care professionals and researchers. OMIM is maintained by Johns Hopkins University School of Medicine.
- Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.
- PubMed is a searchable database of medical literature and lists journal articles that discuss Linear and whorled nevoid hypermelanosis. Click on the link to view a sample search on this topic.
- The American Academy of Dermatology provides kids, teens, and parents with information on skin conditions.
- Nevoid hypermelanosis, linear and whorled; LWNH. OMIM. Updated 10/24/2016; https://omim.org/entry/614323.
- Di Lernia V. Linear and whorled hypermelanosis review. Pediatr Dermatol. 2007; 24(3):205-210. https://pubmed.ncbi.nlm.nih.gov/17542865.
- Sinha P, Chatterjee M, Singh KK, Sood A. Linear and Whorled Nevoid Hypermelanosis with Depigmentation. Indian Dermatol Online J. 2017; 8(2):131-133. https://pubmed.ncbi.nlm.nih.gov/28405555.
- Mehta V, Vasanth V, Balachandran C, Mathew M. Linear and whorled nevoid hypermelanosis. Int J Dermatol. Apr 2011; 50(4):491-2. https://pubmed.ncbi.nlm.nih.gov/21413967.
- Vashi N, Kundu R. Congenital and inherited hyperpigmentation disorders. UpToDate. Waltham, MA: UpToDate; Updated Nov 12, 2019; https://uptodate.com/contents/congenital-and-inherited-hyperpigmentation-disorders.
- Yao L, Zhou DD, Lu CW. Linear and Whorled Nevoid Hypermelanosis. Am J Med Sci. January, 2017; 353(1):1-3.
- Metta AK, Ramachandra S, Sadath N, Manupati S. Linear and whorled nevoid hypermelanosis in three successive generations. Indian J Dermatol Venereol Leprol. May-June, 2011; 77(3):403.