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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 blepharochalasis – 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.
• Hereditary angioedema – May present with episodic facial swelling but often involves other areas of the body including the limbs and airway.
• Orofacial granulomatosis – Characterized by persistent lip swelling related to chronic noncaseating granulomatous inflammation.
• Melkersson-Rosenthal syndrome – 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.
• Cutis laxa – 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.
• Crohn disease – May initially present with cutaneous manifestations such as lip or genital swelling.

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