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 Table of Contents  
CASE REPORT
Year : 2019  |  Volume : 11  |  Issue : 4  |  Page : 222-224

Surgical correction of bilateral fusion of oral commissure in a patient with stevens–Johnson syndrome


1 Oral and Maxillofacial Surgeon, Department of Health and Family Welfare, Himachal Dental College, Sunder Nagar, Himachal Pradesh, India
2 Department of Conservative Dentistry and Endodontics, Himachal Dental College, Sunder Nagar, Himachal Pradesh, India

Date of Submission29-Jul-2019
Date of Decision13-Aug-2019
Date of Acceptance13-Aug-2019
Date of Web Publication1-Oct-2019

Correspondence Address:
Shweta Verma
Department of Conservative Dentistry and Endodontics, Himachal Dental College, Sunder Nagar, Himachal Pradesh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/IJDS.IJDS_86_19

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  Abstract 


The present case report highlights the surgical correction of bilateral fusion of oral commissure in a 10-year-old male patient suffering from Stevens–Johnson syndrome associated with Mycoplasma pneumoniae infection. It was treated using a modified commissuroplasty technique and was quite successful.

Keywords: Modified commissuroplasty, Mycoplasma, oral commissure, Stevens–Johnson syndrome


How to cite this article:
Singh M, Verma S, Goel M, Kumar V. Surgical correction of bilateral fusion of oral commissure in a patient with stevens–Johnson syndrome. Indian J Dent Sci 2019;11:222-4

How to cite this URL:
Singh M, Verma S, Goel M, Kumar V. Surgical correction of bilateral fusion of oral commissure in a patient with stevens–Johnson syndrome. Indian J Dent Sci [serial online] 2019 [cited 2019 Nov 15];11:222-4. Available from: http://www.ijds.in/text.asp?2019/11/4/222/268426




  Introduction Top


Stevens–Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare, acute, serious, and potentially fatal skin reactions with severe erosion of at least two mucosal surfaces, including extensive necrosis of oral and nasal mucosa and purulent conjunctivitis but less commonly involving vaginal, urethral, gastrointestinal, or respiratory mucous membranes.[1]

The estimated incidence is at 1–2/million each year for SJS and 0.4–1.2/million each for TEN.[2] It has been found to affect all age groups and all races. An association with genetic factors has been proved. There are Human Leukocyte Antigen (HLA) associations in some races to anticonvulsants and allopurinol. Polymorphisms to specific genes have been detected (e.g., CYP2C coding for cytochrome P450 in patients reacting to anticonvulsants.[3],[4],[5]

More than 200 medications have been reported in association with SJS/TEN. It is more often seen with drugs with long half-lives compared to even a chemically similar related drug with a short half-life. The medications are usually systemic, but TEN has been reported after topical use.[6] The drugs that cause SJS/TENS are sulfonamides, beta lactam antibiotics, anticonvulsants like; lamotrigine, carbamazepine, phenytoin, phenobarbitone, alupurinol, non-steroidal anti-inflammatory drugs and nonnucleoside reverse transcriptase inhibitor.[6] SJS/TEN has rarely been found associated with vaccination and infections such as Mycoplasma and Cytomegalovirus.[7],[8]

Following a phase of prodromal symptoms, there is an abrupt onset of a tender/painful red skin rash starting on the trunk and extending rapidly over hours to days onto the face and limbs (but rarely affecting the scalp, palms, or soles). The skin lesions include macules or purpuric spots, diffuse erythema, targetoid lesions as in erythema multiforme, or flaccid blisters.

The blisters then merge to form sheets of skin detachment, exposing red, oozing dermis. The Nikolsky's sign is positive in areas of skin redness. Mucosal involvement is prominent and severe. The mucosal surfaces affected include; eyes which manifests as conjunctivitis, corneal ulcerations, anterior uveitis and panopthalmitis; lips/mouth, pharynx, eosophagus, genital and urinary tract erosions. The patient has red-crusted lips, painful mouth ulcers, cough, respiratory distress, diarrhea and difficulty in eating.

In recent literature, there have been few reports of isolated mucositis associated with Mycoplasma infection labeled as atypical SJS mostly in ages ranging from 8 to 21 years.[7],[8] Incomplete fusion of the oral commissures (bands connecting the lateral aspect of the upper and lower lips but sparing the very corner of the mouth) in the cases of SJS has been reported before by several authors.[9],[10],[11],[12] The cases of complete fusion have also been reported. The present case report describes the successful surgical management of a patient with complete bilaterally fused oral commissures, developed as a complication of SJS associated with Mycoplasma infection using a modified commissuroplasty technique.


  Case Report Top


A 10-year-old male patient was referred from pediatric surgery clinic for severe microstomia caused by bilateral oral commissure fusion post-SJS. The patient was diagnosed with SJS due to Mycoplasma pneumoniae (MP) infection.

Three weeks back, the patient had a fever with sore throat, running nose, red eyes, and generalized body aches and malaise. This was followed by generalized swelling and full-thickness epidermal necrolysis of the skin with involvement of the face and mucosa of the lips and eyes. He had severe stomatitis and cheilitis with hemorrhagic crusting of lips and painful mouth ulcers.

There was no history of consumption of any medication. Routine blood investigations and HSV DNA, polymerase chain reaction (PCR), and Mycoplasma serology were carried out. MP immunoglobulin M titer was positive on enzyme assay. Finally, he was diagnosed with SJS with associated Mycoplasma infection.

The patient was treated in an intensive care unit with intravenous (IV) fluids, IV adrenaline, and Vitamin A injection. He was administered tablet linezolid 300 mg BD and azithromycin 500 mg OD for 7 days. A paraffin-based local anesthetic gel was given for application over mucosal erosions. Once the patient's condition stabilized, re-epithelization of the affected areas commenced. During the re-epithelization process, the patient's oral commissures fused resulting in impairment of normal speech, oral intake, and compromised esthetic appearance. On examination, the fused tissues at corners of the lips were found to be supple [Figure 1]a, [Figure 1]b, [Figure 1]c.
Figure 1: (a-c) Preoperative images depicting bilateral lip fusion

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After obtaining the written consent from the patient's parents, it was planned to carry out a modified commissuroplasty technique for esthetic and functional reconstruction of corners of mouth. The incisions were given at the level of commissure in the shape of a four-sided polygon with a long diagonal of 1.5 cm and short diagonal of 1 cm. The full-thickness excision of the scar tissue present was performed leaving behind the deep mucosal layer [Figure 2]a. The remaining mucosa was then sectioned in triangular shape creating three independent flaps [Figure 2]b and [Figure 2]c. The lateral flaps were used to resurface the corner of the mouth, while preventing the overlapping of sutures lines and refusion during healing process. The superior flap was used to resurface the upper and inferior flaps to resurface the lower lip. The flaps were secured in place using interrupted deep dermal Polydioxanone Sutures (PDS) sutures. The superficial lip mucosa was closed with a running 6–0 absorbable suture [Figure 2]d, [Figure 2]f.
Figure 2: (a) Polygon-shaped incision. (b) Excision of fused tissue. (c) An open triangular flap to resurface defect. (d-f) Resurfacing of flaps and suturing

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The patient was found to be stable postoperatively and was discharged home. The follow-up at 6 weeks showed complete healing with a normalized opening, function, and esthetically pleasing smile [Figure 3]a, [Figure 3]b, [Figure 3]c.
Figure 3: (a-c) Healed defect and normal mouth opening seen at 6 weeks

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  Discussion Top


Extrapulmonary manifestations of MP infection are unusual and include SJS, arthritis, and encephalitis. Serology is the mainstay of laboratory diagnosis. When available, the PCR is a rapid and helpful test.[13] In the present case, the diagnosis was confirmed using serology. Multiple retrospective reports showed that MP infection may be the most common infectious agent associated with SJS.[14],[15]

Microstomia is a challenging condition to treat. Its surgical reconstruction usually involves three steps: reestablishing the intended location of the commissure, excision of the existing scar, and resurfacing of the resulting defect. While maintaining continuity of the orbicularis oris, the resurfacing can be achieved by primary closure, split or full-thickness skin grafting, or local tissue rearrangement.[16],[17]

First employed by Dieffenbach in 1831, modified by Converse and later by Friedlander and Millard, the Y-V advancement technique is a popular choice for the treatment of commissural microstomia in burn patients.[17],[18] The procedure described in the present report represents a slight modification of the technique. As the dry vermilion of the commissures was not affected by the scar tissue, it was not resected in our patient. The three mucosal flaps used to reconstruct each commissure were used to resurface the wet vermilion only. They were advanced and sutured to the intact dry vermilion at the level of the red line, replacing only similar tissue.


  Conclusion Top


Modified commissuroplasty technique involving scar excision and mucosal advancement, is an effective treatment modality to correct microstomia that occurs secondary to SJS.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Mockenhaupt M. The current understanding of Stevens-Johnson syndrome and toxic epidermal necrolysis. Expert Rev Clin Immunol 2011;7:803-13.  Back to cited text no. 1
    
2.
Hötzenecker W, Prins C, French LE. In: Bolognia JL, Schaffer JV, Cerroni L, editors. Erythema Multiforme, Stevens-Johnson Syndrome, and Toxic Epidermal Necrolysis: Dermatology. 4th ed., Vol. 1. Elsevier; 2018. p. 332-47.  Back to cited text no. 2
    
3.
Roujeau JC, Huynh TN, Bracq C, Guillaume JC, Revuz J, Touraine R, et al. Genetic susceptibility to toxic epidermal necrolysis. Arch Dermatol 1987;123:1171-3.  Back to cited text no. 3
    
4.
Roujeau JC, Bracq C, Huyn NT, Chaussalet E, Raffin C, Duédari N, et al. HLA phenotypes and bullous cutaneous reactions to drugs. Tissue Antigens 1986;28:251-4.  Back to cited text no. 4
    
5.
Chung WH, Hung SI, Hong HS, Hsih MS, Yang LC, Ho HC, et al. Medical genetics: A marker for Stevens-Johnson syndrome. Nature 2004;428:486.  Back to cited text no. 5
    
6.
Inada A, Oyama S, Niinomi I, Wakabayashi T, Iwanaga K, Hosohata K, et al. Association of Stevens-Johnson syndrome and toxic epidermal necrolysis with antiepileptic drugs in pediatric patients: Subgroup analysis based on a Japanese spontaneous database. J Clin Pharm Ther 2019. doi: 10.1111/jcpt (ahead of print).  Back to cited text no. 6
    
7.
Schalock PC, Dinulos JG. Mycoplasma pneumoniae-induced cutaneous disease. Int J Dermatol 2009;48:673-80.  Back to cited text no. 7
    
8.
Ravin KA, Rappaport LD, Zuckerbraun NS, Wadowsky RM, Wald ER, Michaels MM, et al. Mycoplasma pneumoniae and atypical Stevens-Johnson syndrome: A case series. Pediatrics 2007;119:e1002-5.  Back to cited text no. 8
    
9.
Sakamoto H, Nagashima T, Imai Y. Angular webbing associated with Stevens-Johnson syndrome. Int J Oral Maxillofac Surg 1993;22:118.  Back to cited text no. 9
    
10.
Marinho LH, Haj M, Pereira LF. Lip adhesion: An unusual complication of erythema multiforme. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 1999;88:167-9.  Back to cited text no. 10
    
11.
Karincaoglu Y, Coskun BK, Seyhan M. An unusual complication of erythema multiforme and its treatment. Skinmed 2005;4:53-5.  Back to cited text no. 11
    
12.
Royan SJ. Lip adhesion: Unusual complication of Stevens-Johnson syndrome. J Oral Maxillofac Surg 2010;68:901-3.  Back to cited text no. 12
    
13.
Berger RP, Wadowksy RM. Rhabdomyolysis associated with infection by Mycoplasma pneumoniae: A case report. Pediatrics 2000;105:433-6.  Back to cited text no. 13
    
14.
Léauté-Labrèze C, Lamireau T, Chawki D, Maleville J, Taïeb A. Diagnosis, classification, and management of erythema multiforme and Stevens-Johnson syndrome. Arch Dis Child 2000;83:347-52.  Back to cited text no. 14
    
15.
Vanfleteren I, Van Gysel D, De Brandt C. Stevens-Johnson syndrome: A diagnostic challenge in the absence of skin lesions. Pediatr Dermatol 2003;20:52-6.  Back to cited text no. 15
    
16.
Kazanjian VH, Roopenian A. The treatment of lip deformities resulting from electric burns. Am J Surg 1954;88:884-90.  Back to cited text no. 16
    
17.
Zweifel CJ, Guggenheim M, Jandali AR, Altintas MA, Künzi W, Giovanoli P, et al. Management of microstomia in adult burn patients revisited. J Plast Reconstr Aesthet Surg 2010;63:e351-7.  Back to cited text no. 17
    
18.
Zak M, Means O, Cason B, Brooks R. Management of severe burn microstomia. Eplasty 2016;16:ic45.  Back to cited text no. 18
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

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