Linear and whorled nevoid hypermelanosis

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Synonyms: LWNH

A rare hyperpigmentation of the skin disease characterized by the congenital to infantile-onset of bilateral diffuse (occasionally localized) reticulate (swirls and streaks) macular hyperpigmentation following the lines of Blaschko typically involving the trunk limbs head and neck (but sparing palms soles and mucosa) without preceding inflammation blistering or atrophy. Occasionally extracutaneous abnormalities including autism seizures cardiac defects skeletal abnormalities and developmental delay may be associated. Histologically basal and/or suprabasal melanosis without pigment incontinence is observed.

Data from Orphanet are used to provide information on a disease's name, synonym(s), and overview. Reference: Access aggregated data from Orphanet at Orphadata. Orphadata: Free access data from Orphanet. © INSERM 1999. Available on Data version June 2024

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Linear and whorled nevoid hypermelanosis?

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