Ascher syndrome

Author: Brian Wu PhD. MD Candidate, Keck School of Medicine, Los Angeles, USA; Chief Editor: Hon A/Prof Amanda Oakley, Dermatologist, Hamilton, New Zealand, February 2016.


What is Ascher syndrome?

Ascher syndrome is a rare condition characterised by persistent swelling of the lip and eyelids and sometimes by nontoxic thyroid enlargement. 

It was first described in 1920 by Dr. Ascher, an ophthalmologist in Prague.  

Who gets Ascher syndrome?

Ascher syndrome appears to have no prevalence by gender, age, sex or geographical area.

What causes Ascher syndrome?

The exact cause of Ascher syndrome is unknown. If acquired, it is usually due to trauma. If congenital, it stems from some developmental anomaly.

  • Double lip deformity is due to persistence of horizontal sulcus with hypertrophy of the pars villosa.
  • Eyelid oedema is associated with a decrease in dermal elastin.

What are the clinical features of Ascher syndrome?

The three salient characteristics of Ascher syndrome include:

  • Blepharochalasis, or recurrent non-pitting, painless oedema, in 80%  
  • Lip deformity/double lip malformation
  • Thyroid gland enlargement/goitre, in 10–50%

Staging of blepharochalasis is as follows:

  • Stage 1: Intermittent, painless oedema
  • Stage 2: Ptosis due to dehiscence of levatur aponeurosis
  • Stage 3: Ptosis adiposa, characterized by medial fat pad atrophy, orbital fat prolapse and lacrimal gland prolapse

Associated signs and symptoms of this condition include:

Differential diagnosis of Ascher syndrome

Angioedema causing eyelid or lip oedema is not persistent, and last hours to a few days. Persistent eyelid oedema is sometimes a feature of phymatous rosacea. Persistent lip swelling arises in granulomatous cheilitis and other forms of orofacial granulomatosis.

How is Ascher syndrome treated?

Treatment for Ascher syndrome can include surgery:

  • To correct double lip if it interferes with speaking, eating or for aesthetic reasons
  • To correct visual acuity disturbances or ocular complications

What is the outcome of Ascher syndrome?

With surgical treatment, prognosis is good and functional and aesthetic improvement can be achieved. 

 

Related Information

References:

  • de Andrade-Santos, P.P. et. al. Double Lip Surgical Correction in Ascher’s Syndrome: Diagnosis and Treatment of a Rare Condition. Clinics. 2008. 63(5) 709-718. PubMed.
  • Ganesan, A. and Gautham, K. Ascher Syndrome: an Oral Medicine Perspective. International Journal of Dental Sciences and Research. 2014 2(4) 87-91.
  • Ramesh, B.A. Ascher Syndrome: A Review of Literature and Case Report. Indian Journal of Plastic Surgery. 2011. 44(1) 147-149. PubMed.
  • Schivcharan, L.C. Ascher’s Syndrome: a Rare Case Report. Indian Journal of Ophthalmology. 2015. 63(3) 264-267. PubMed.

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