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Becker nevus syndrome

Disease definition

A rare, syndromic, benign, epidermal nevus syndrome characterized by the association of a Becker nevus (i.e. circumscribed, unilateral, irregularly shaped, hyperpigmented macules, with or without hypertrichosis and/or acneiform lesions, occuring predominantly on the anterior upper trunk or scapular region) with ipsilateral breast hypoplasia or other, typically hypoplastic, skeletal, cutaneous, and/or muscular defects, such as pectoralis major hypoplasia, supernumerary nipples, vertebral defects, scoliosis, limb asymmetry, odontomaxillary hypoplasia and lipoatrophy.


Classification level: Disorder
  • Synonym(s):
    • Pigmentary hairy epidermal nevus
  • Prevalence: Unknown
  • Inheritance: -
  • Age of onset: All ages
  • ICD-10: D22.5
  • ICD-11: LC02
  • OMIM: 604919
  • UMLS: C1858042
  • MeSH: C565735
  • GARD: 3856  5901
  • MedDRA: -
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