Ascher Syndrome

Ascher syndrome is 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. It is also sometimes called blepharochalasis and double lip.

Causes of Ascher Syndrome #

• The exact cause of Ascher syndrome is unknown.

• If acquired, swelling of the lips and eyelids 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.

Symptoms of Ascher Syndrome #

• Blepharochalasis, or recurrent non-pitting, painless oedema of the eyelids in 80%

• Lip deformity/double lip malformation due to folding of the oral mucosa. The upper lip is more often affected than the lower lip

• Thyroid gland enlargement/goitre, in 10–50%

• Bifid uvula

• Cleft palate

• Cheilitis

• Facial vascular malformations

• Thickened lips and gums

Staging of blepharochalasis #

• 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

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.

Treatment of Ascher Syndrome #

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