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CASE REPORT
Year : 2019  |  Volume : 22  |  Issue : 7  |  Page : 1029-1031

Ascher syndrome: A case report


1 Department of Periodontology, Faculty of Dentistry, Harran University, Şanlıurfa, Turkey
2 Department of Periodontology, Faculty of Dentistry, Bingöl University, Diyarbakır, Turkey

Date of Acceptance04-Feb-2019
Date of Web Publication11-Jul-2019

Correspondence Address:
Dr. D D Uner
Department of Periodontology, Harran University, Faculty of Dentistry, Şanlıurfa
Turkey
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/njcp.njcp_661_18

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   Abstract 


Ascher syndrome is a disease that is characterized by upper eyelid edema, double lip, and swelling in the thyroid glands whose etiology is unknown, and it is usually seen in young people over the age of 20. Blepharochalasis and double lip are observed in these patients as a result of the recurring lip and upper eyelid edema. The disease is benign and seen in both sexes and all races in about the same amounts. Although this syndrome rarely leads to functional problems, it usually leads to psychological problems in the patients it affects due to the malformations on their faces. In this case presentation, we reported the surgical treatment of a 27-year-old patient diagnosed with Ascher syndrome and displayed double lip based on this syndrome.

Keywords: Ascher syndrome, blepharochalasis, double lip


How to cite this article:
Uner D D, Izol B S. Ascher syndrome: A case report. Niger J Clin Pract 2019;22:1029-31

How to cite this URL:
Uner D D, Izol B S. Ascher syndrome: A case report. Niger J Clin Pract [serial online] 2019 [cited 2019 Sep 22];22:1029-31. Available from: http://www.njcponline.com/text.asp?2019/22/7/1029/262535




   Introduction Top


Ascher syndrome was first defined in 1920 by an ophthalmologist.[1] It is a rare disease that is characterized by upper eyelid edema, double lip deformity, and nontoxic goiter.[2] Idiopathic nontoxic thyroid swelling is seen in 10% of cases. Nontoxic goiter is sometimes not accepted as a basis for the diagnosis of Ascher syndrome as it appears a few years after upper eyelid edema.[3] As Laffer (1909) previously defined a relationship between eyelid edema and double lip, some authors call this condition Laffer–Ascher syndrome.[1],[4] Up to now, more than 100 cases have been reported in the literature as Ascher syndrome.[5] As this syndrome is rare, patients are often undiagnosed.[2] Although it starts with puberty, it was reported to be found in patients under the age of 8 in some cases. Although this syndrome rarely leads to functional problems, it usually leads to psychological problems in the patients it affects due to the malformations on their faces.[6]

In this case presentation, we will report the surgical treatment of a 27-year-old male patient diagnosed with Ascher syndrome and displayed double lip based on this syndrome.


   Case Presentation Top


The 27-year-old patient visited the Periodontology clinic at Harran University with complaints of swelling in his eyelids and upper lip. The patient stated that he did not remember when the swelling in his lip started, and the swelling in the eyes increased when he stayed awake. It was learned that no other person in the patient's family had these complaints. The patient did not have any systemic disease.

In the clinical examination of the patient, in the upper lip of the patient, asymmetrical, painless excess soft mucosa was detected by palpation [Figure 1]. The patient was directed to an ophthalmologist for the swelling on his upper eyelids [Figure 2]. As a result of the examination, no swelling could be detected in the neck area of the patient. Considering the clinical signs of the patient, he was diagnosed with Ascher syndrome, and the patient was subjected to tests regarding thyroid, renal, and liver functions. In the thyroid hormone tests, the T3, T4, and TSH values were found, respectively, as 5.53, 17.34, and 0.891. According to the results, the patient's thyroid glands were working normally.
Figure 1: Double upper lip

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Figure 2: Blepharochalasis

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The swelling on the lip of the patient who was diagnosed with Ascher syndrome was excised by a periodontist.


   Method of Operation Top


The patient had no systemic disease or periodontal problem that would obstruct surgery. The area inside and outside the mouth was disinfected by a 2% betadine solution. Conventional local anesthesia (jetokain, vial-Lidocaine HCl 20 mg/mL, epinephrine HCl 0.0125 mg/mL—ADEKA, Turkey) was applied between the upper first premolars. After the tissues were dried using a sterile pen, the excess tissues in the labial mucosa were elliptically marked. The incision was made by using a size 15 scalpel (Beybi, Turkey) on half thickness by taking the marked regions as a reference. Another incision was made in parallel to the first incision at a distance of 10–12 mm from the first one over the previously drawn border. The two incisions were then joined elliptically on the level of canine teeth. The epithelial tissue that had an approximate width of 1 cm between the incision points was removed along with the connective tissue under it [Figure 3]. After bleeding was taken under control, saturation was achieved by 3/0 silk suture (DOĞSAN, Turkey). In the postoperative period, Etol Fort 400 mg tb. was prescribed for use four times a day (Etodolac 400 mg—MOBELFARMA, Turkey) as mouthwash, Largopen 500 mg tb. was prescribed for use three times a day (Amoxicillin trihydrate—Bilim İlaç, Turkey), and Kloroben 200 mL three times a day was prescribed (chlorhexidine gluconate 0.012%, benzydamine hydrochloride 0.15%—DROGSAN, Turkey) as precautions, and the patient was recommended to use these for a week.
Figure 3: Removal of edematous tissue on the lip during surgery

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The patient was recommended to apply ice compression to prevent edema after the operation and limit his lip movements while laughing and speaking. The sutures of the patient were removed 10 days after the operation [Figure 4]. After soft tissue recovery (post-op 3 months), the patient was invited for checking the outcome of the operation [Figure 5].
Figure 4: Aspect of the lip 10 days after the operation

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Figure 5: Aspect of the lip 3 months after the operation

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   Discussion Top


This clinical case shows the surgical excision of a mucosal growth in the upper lip, which is seen in Ascher syndrome. Ascher syndrome is a disease that is characterized by upper eyelid edema, double lip, and swelling in the thyroid glands whose etiology is unknown, and it is usually seen in young people over the age of 20.[7] The disease is benign and seen in both sexes and all races in about the same amounts.[2]

In relation to double lip syndrome, studies in the literature have reported conditions such as bifid uvula, cleft palate, cheilitis, and facial hemangioma.[8] In our case, there was no indication of conditions such as bifid uvula, cleft palate, cheilitis, and facial hemangioma, while we diagnosed our patient with Ascher syndrome due to the presence of swelling in upper eyelids in addition to double lip.

In most cases, double lip formation takes place only in the upper lip. The lower lip or both lips are rarely involved. Its formation mechanism is based on the permanence of the horizontal sulcus that is located between the outer cutaneous region (pars glabrosa) and the inner mucosal region (pars villosa).[9] The treatment for double lip syndrome is surgical excision. As double lip syndrome obstructs the patient's speech and biting and leads to aesthetical problems, its surgical excision arises as an indication.[8] Various surgical methods are used in the treatment of double lip syndrome, and the easiest way of excision is by elliptical incision.[10],[11] In this case, although the patient had difficulty in speaking and biting, he visited our clinic with aesthetical concerns, and we used the easy method of the elliptical incision as a surgical method.

Blepharochalasis is observed in approximately 80% of the cases.[2] The blepharochalasis that is observed in these patients is characterized by atrophy and wrinkling. As a result of prolapse caused by orbital fats, the eyelid skin that is affected hangs. Additionally, narrowing of the palpebral fissure in these patients leads to visual impairments.[12] Blepharochalasis is clinically observed as painless, erythema-free edema of the upper eyelids.[13]

In this case presentation, our patient stated that swelling at his upper eyelids appeared mainly when he was tired. However, no situation obstructed his vision.


   Conclusion and Recommendations Top


Although Ascher syndrome is a rarely encountered disease, the combined observation of double lip, upper eyelid edema, and thyroid growth helps to make a diagnosis. The swelling that is seen in the upper lip of patients leads to aesthetical problems in the patients, and it is the main reason that leads patients toward dentists. Therefore, it is important for the diagnosis and treatment of this disease that dentists, especially periodontists, have sufficient knowledge about Ascher syndrome.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Mühlendyck H, Hundeiker M. [Blepharoachalasis (Fuchs) and the Laffer–Ascher syndrome]. Hautarzt 1978;29:474-7.  Back to cited text no. 1
    
2.
Ramesh B. Ascher syndrome: Review of literature and case report. Indian J Plast Surg 2011;44:147-9.  Back to cited text no. 2
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3.
Ruiz-Villaverde R, Anguita-Carazo JL, Sánchez-Cano D. Laffer–Ascher's syndrome mimicking hereditary angio-edema. Int J Dermatol 2009;48:914-5.  Back to cited text no. 3
    
4.
Mallikarjunappa B, Chandan G, Singh M, Gupta S. Fraser syndrome: A case report. J Int Med Sci Acad 2015;28:147.  Back to cited text no. 4
    
5.
Ascher KW. Blepharochalasis mit Struma und Doppellippe. Klin Monbl Augenheilkd 1920;65:86-97.  Back to cited text no. 5
    
6.
Molina S, Medard P, Galdeano M. Ascher syndrome: Report of a case with early manifestations. Craniomaxillofac Trauma Reconstr 2015;8:150-2.  Back to cited text no. 6
    
7.
Dalmau J, Puig L, Roé E, Peramiquel L, Pimentel CL, Alomar A. Blepharochalasia and double lip: Diagnosis and treatment of Ascher's syndrome [44]. J Eur Acad Dermatol Venereol 2006;20:1390-1.  Back to cited text no. 7
    
8.
Suliman MT, Alhassan M. Double lip: Report of five cases and review of the literature. Aesthetic Surg J 2007;27:289-91.  Back to cited text no. 8
    
9.
Ali K. Ascher syndrome: A case report and review of the literature. Oral Surg. Oral Med Oral Pathol Oral RadiolEndod 2007;103:26-8.  Back to cited text no. 9
    
10.
Reddy KA, Rao AK. Congenital double lip: A review of seven cases. Plast Reconstr Surg 1989;84:420-3.  Back to cited text no. 10
    
11.
Eski M, Nisanci M, Aktas A, Sengezer M. Congenital double lip: Review of 5 cases. Br J Oral Maxillofac Surg 2007;45:68-70.  Back to cited text no. 11
    
12.
Huxley A. Positivists and Buddhists: The rise and fall of anglo-Burmese ecclesiastical law. Law Soc Inq 2001;26:113-42.  Back to cited text no. 12
    
13.
Tsang AKL, Taverne A, Holcombe T. Marfan syndrome: A review of the literature and case report. Spec Care Dent 2013;33:248-54.  Back to cited text no. 13
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]



 

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