Albright syndrome

Last Updated: 2023-07-07

Author(s): Anzengruber F., Navarini A.

ICD11: -

McCune 1936

Albright 1937

Polyostotic fibrous dysplasia

Genodermatosis, which is defined by the triad: café au lait spots, pubertas praecox and fibrous dysplasia.

Postzygotic mutation of the GNAS11 gene.

Sharply defined, irregular (Coast of Maine), brownish macules, following Blaschko lines.

Pubertas praecox.

Fibrous dysplasia (predisposition to fractures)

Frequent endocrinological abnormalities

Anamnesis (e.g. bone fractures)

Clinical

Genetic analysis.

Endocrinological consultation.

No dermatological therapy necessary.

At cosmetic request, bleaching of café au lait stains can be attempted

  • Hydroquinone
  • Azelaic acid
  • Laser

Endocrinological controls

  1. Schouten BJ, Suliman HM. Polyostotic fibrous dysplasia. JBR-BTR. 2013 Jul-Aug;96(4):246-7.
  2. Lietman SA, Levine MA. Fibrous dysplasia. Pediatr Endocrinol Rev. 2013 Jun;10 Suppl 2:389-96.
  3. Martin-Carreras T, Sanchez E, Bancroft LW. Polyostotic fibrous dysplasia. Orthopedics. 2014 Nov;37(11):722-82.

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