Double lip is secondary to labial mucosal hyperplasia and persistence of the horizontal sulcus of the outer cutaneous lip and inner mucosal lip. In most cases, this finding is limited to the upper lip, but rarely the lower lip may be affected.
Blepharochalasis describes lid laxity and sagging formed from repeated episodes of painless edema of the eyelids; it may impede eyesight in severe cases.
Nontoxic thyroid enlargement is found in 10%-50% of cases and is nonessential to the diagnosis. When present, it is not accompanied by abnormal thyroid levels.
E07.89 – Other specified disorders of thyroid
28599006 – Ascher's syndrome
Differential Diagnosis & Pitfalls
- Congenital or acquired double lip – May occur without blepharochalasis or nontoxic enlarged thyroid. The acquired form is typically the result of habitual trauma.
- Isolated – Recurrent episodes of nonerythematous edema of the eyelids resulting in ptosis with onset in childhood may occur outside of the context of Ascher syndrome.
- Dermatochalasis – A loss of elasticity of the skin leading to eyelid droop, which may obstruct vision. Most often seen in elderly patients.
- – May present with episodic facial swelling but often involves other areas of the body including the limbs and airway.
- – Characterized by persistent lip swelling related to chronic noncaseating granulomatous inflammation.
- – Triad of orofacial swelling, facial nerve palsy, and furrowed tongue. Like Ascher syndrome, all components of the triad are not necessarily present. Unlike Ascher syndrome, this has been shown to respond to steroid treatment.
- – Disorder of hypoelastic, loose skin with initial presentation often on the face before progressing caudally. Most commonly presents in adulthood; however, variants may also present in children.
- – May initially present with cutaneous manifestations such as lip or genital swelling.