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Linear and whorled nevoid hypermelanosis (LWNH) is a rare skin condition characterized by swirling streaks of hyperpigmented (darkened) skin. The pigmentation follows the lines of Blashko and is mainly located on the trunk and limbs. It is present at birth or appears in the first few weeks of life. It typically progresses for one to two years and then stabilizes. Hyperpigmentation is usually the only symptom but there are isolated reports of other symptoms, involving mostly the central nervous system, musculoskeletal system, and heart. While most cases of LWNH are sporadic, apparent genetic transmission rarely has been described. A few people with LWNH have been diagnosed with chromosomal mosaicism.
Features include: Increased total eosinophil count and Hyperpigmented streaks.
Organ System | Phenotype Count | Example Features |
|---|---|---|
Skin | 1 | Hyperpigmented streaks |
Estimated prevalence: <1 in 1,000,000 (VERY_RARE).
No clinical trials have been registered for linear and whorled nevoid hypermelanosis.
22 publications have been identified in PubMed for linear and whorled nevoid hypermelanosis. Research spans Case Report / Case Series (50%), Review / Meta-Analysis (23%), and Other (14%).
Research Type | Count | % of Total |
|---|---|---|
Patient case studies | 11 | 50% |
Data assembled from 5 of 12 sources · Last updated Sep 19, 2026, 6:56 PM UTC
Online Mendelian Inheritance in Man
European rare disease database
Genetic and Rare Diseases Info Center
5 |
23% |
Other research | 3 | 14% |
Disease patterns and progression | 2 | 9% |
Laboratory research | 1 | 5% |
Zhu J (2026). [PMID: 41491585](https://pubmed.ncbi.nlm.nih.gov/41491585/). *Int J Dermatol*. [Other]
Castillon E (2026). [PMID: 42206741](https://pubmed.ncbi.nlm.nih.gov/42206741/). *Clin Genet*. [Basic Science / Preclinical]
Nicholson CL (2026). [PMID: 32644429](https://pubmed.ncbi.nlm.nih.gov/32644429/). *Unknown Journal*. [Review / Meta-Analysis]
Ma MX (2026). [PMID: 40801523](https://pubmed.ncbi.nlm.nih.gov/40801523/). *J Dtsch Dermatol Ges*. [Case Report / Case Series]
Descos M (2026). [PMID: 41885577](https://pubmed.ncbi.nlm.nih.gov/41885577/). *J Eur Acad Dermatol Venereol*. [Other]
Alonso-Mtz de Salinas L (2026). [PMID: 41345742](https://pubmed.ncbi.nlm.nih.gov/41345742/). *J Cutan Pathol*. [Review / Meta-Analysis]
Colmant C (2026). [PMID: 41669896](https://pubmed.ncbi.nlm.nih.gov/41669896/). *Pediatr Dermatol*. [Case Report / Case Series]
Zheng J (2026). [PMID: 41832788](https://pubmed.ncbi.nlm.nih.gov/41832788/). *J Cutan Med Surg*. [Review / Meta-Analysis]
Krouse AH (2025). [PMID: 39754330](https://pubmed.ncbi.nlm.nih.gov/39754330/). *J Cutan Pathol*. [Case Report / Case Series]
Zhang AD (2025). [PMID: 40507749](https://pubmed.ncbi.nlm.nih.gov/40507749/). *J Clin Med*. [Review / Meta-Analysis]
AI-curated news mentioning linear and whorled nevoid hypermelanosis
Updated Mar 26, 2026
A multicenter retrospective study analyzed 33 patients with linear and whorled hypermelanosis, contributing to the understanding of this rare skin condition. The findings may help inform future research and clinical approaches.