Linear verrucous nevus syndrome

Orpha code: 2611OMIM code:

Definition

A rare skin disease characterized by a hamartomatous epidermal lesion presenting as a linear array of verrucous, hyperkeratotic papules that often coalesce into plaques and are formed along the lines of Blaschko. The condition is associated with involvement of other organ systems, mainly brain, eye, and skeletal system. It is the result of mosaic post-zygotic mutations and most commonly presents at birth, but may occur anytime during childhood, rarely also in adulthood.

Disease data
Classification

Disease

Synonyms
Linear hamartoma syndrome
Zespół linijnego harmatoma
ORPHA code
2611
OMIM code
-
ICD10 code
Q82.5
ICD11 code
-

No additional description.

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