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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.

ORPHA:64755

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: -

  A summary on this disease is available in Deutsch (2019) Español (2019) Français (2019) Italiano (2019) Nederlands (2019)

Additional information

Further information on this disease

Patient-centred resources for this disease

Research activities on this disease

Specialised Social Services

The documents contained in this web site are presented for information purposes only. The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment.