Ascher's syndrome

Ascher's syndrome, is a rare disorder first described in 1920.[1] It is characterized by repeated episodes of lip and eyelid edema and occasionally euthyroid goiter. The syndrome generally occurs within the first 20 years of life.[2] About 100 cases had been described by 1998.[3]

Ascher's syndrome
Other namesLaffer-Ascher Syndrome

Signs and Symptoms
  • Blepharochalasis : Recurrent episodes of swelling cause stretching and atrophy of the upper eyelid skin. This results in the relaxation of the tarsal fold allowing tissue to slack over the palpebral fissure. In severe cases, the lower eyelid is also involved.
  • Double Upper Lip : Swelling causes duplication between the inner and outer parts of the upper lip. Occasionally the lower lip is involved.[1]
  • Euthyroid Goiter : Occurs in 10% of cases.[2] It is not usually associated with toxic symptoms. Goiter usually presents several years after initial eyelid and lip edema.[1]

Treatment

Cosmetic surgery is generally the treatment of choice.[4]

References
  1. Gorlin RJ, Pindborg JJ, CohenMM.Syndromes of the head and neck, 4th ed.New York:McGraw-Hill, 1976: 500-501.
  2. Sanchez MR, Lee M, Moy JA et al. Ascher syndrome: a mimicker of acquired angioedema. J Am Acad Dermatol 1993;29:650–651.
  3. U. Beinhoff; H. Piza-Katzer (1998). "Double lip in a patient with Ascher's syndrome". European Journal of Plastic Surgery. 21 (7): 370–373. doi:10.1007/s002380050120.
  4. Atzeni M, et al. Surgical correction and MR imaging of double lip in Ascher syndrome: record of a case and a review of the literature. Eur Rev Med Pharmacol Sci 2009;13:309-311.
Classification
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