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 Table of Contents  
CASE REPORT
Year : 2020  |  Volume : 32  |  Issue : 1  |  Page : 55-59

Tail of the whale appearance: A pathognomonic feature of scleroderma


Department of Oral Medicine and Radiology, I. T. S. Dental College and Hospital, Greater Noida, Uttar Pradesh, India

Date of Submission19-Nov-2019
Date of Decision08-Jan-2020
Date of Acceptance04-Mar-2020
Date of Web Publication17-Apr-2020

Correspondence Address:
Dr. Gayatri Mehrotra
240 Nehru Nagar, Agra - 282002, Uttar Pradesh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jiaomr.jiaomr_188_19

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   Abstract 


Scleroderma is a rare connective tissue disorder of unknown etiology characterized by fibrosis of skin, blood vessels, and internal organs. Orofacial changes include mask-like facies, microstomia, xerostomia, and stiffness of tongue with reduced mouth opening. The radiographic features are very characteristic which includes symmetrical widening of periodontal ligament space and bone resorption. We, hereby present a case of 55-year-old female patient of Scleroderma presenting with a typical radiographic feature of “Tail of the whale” appearance due to resorption of the ramus of mandible.

Keywords: Connective tissue disorder, mask-like face, scleroderma, tail of the Whale appearance


How to cite this article:
Sharma ML, Mehrotra G, Sharma K, Suman S. Tail of the whale appearance: A pathognomonic feature of scleroderma. J Indian Acad Oral Med Radiol 2020;32:55-9

How to cite this URL:
Sharma ML, Mehrotra G, Sharma K, Suman S. Tail of the whale appearance: A pathognomonic feature of scleroderma. J Indian Acad Oral Med Radiol [serial online] 2020 [cited 2020 May 30];32:55-9. Available from: http://www.jiaomr.in/text.asp?2020/32/1/55/282609




   Introduction Top


Scleroderma is a connective tissue disorder characterized by fibrosis and excessive deposition of type I and III collagen within the skin, blood vessels and internal organs.[1] It is also known as “Hidebound Disease” since “hidebound skin” is the key feature of this disease. The term Scleroderma is derived from a Greek word ”skleros” which means hard and derma meaning skin. The disease shows a female predilection with females being affected 3-4 times more than males. It mostly affects people from 3rd to 5th decade of life. The hypothesized etiopathogenesis to be considered is the damage to the vascular system and autoimmunity that plays a very significant role in the disease. During the initial stage of the disease, the T lymphocytes infiltrate the skin causing an abnormal activation of the fibroblasts and leading to excessive collagen deposition.[2] Moreover, proliferation of intima takes place resulting in narrowing of artery and arteriole.[3]

Depending on the extent of involvement, the disease can be classified as morphea or circumscribed, generalized or diffuse, acrosclerosis (a condition with scleroderma of extremities and Raynaud's disease).[4] Another variant of this disease is CREST Syndrome, which is an acronym for Calcinosis cutis, Raynaud's phenomenon, Esophageal dysmotility with dysphagia, Sclerodactyly, and Telangiectases.

Hereby, we present a case of Scleroderma with a unique radiographic finding of the mandible.


   Case Report Top


A 55-year-old female patient reported to the Department of Oral Medicine and Radiology with the chief complaint of missing teeth in lower right and left back tooth region. Further, patient also revealed that she was experiencing burning sensation on the tongue, which aggravated on intake of spicy food. Her medical history revealed her as a diagnosed case of Systemic Sclerosis since past 19 years. She was under ayurvedic medications for the same. Autoantibody profile ruled out any other associated syndrome. Patient also gave a history of Raynaud's phenomenon and got cervical sympathectomy done for the same. She was also diagnosed with interstitial lung disease, cholelithiasis and esophagitis. On general examination, it was found that the patient had an asthenic built with normal gait. Sparse hair and marked digital swelling of hands and feet were appreciated [Figure 1].
Figure 1: Thickening of the digits of hands

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Extraoral examination revealed that the skin was tense, smooth, shiny and had lost its elasticity giving her face a mask like appearance. Patient had microstomia along with the characteristic “purse string appearance” of the mouth. Areas of hypopigmentation were appreciated on the forehead [Figure 2] and [Figure 3]. Patient's interincisal mouth opening was reduced to 28 mm.
Figure 2: Lateral profile of the patient

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Figure 3: Frontal profile showing mask-like facies, purse string appearance of the mouth, sparse hair with hypopigmented areas on the forehead

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On intraoral examination, the patient had xerostomia. Blanching was present on the buccal mucosa and palate. The gingiva appeared pale pink and depapillation was observed on the dorsal surface of tongue [Figure 4]. On palpation of the buccal mucosa, fibrous bands could be appreciated bilaterally. Gingiva was firm in consistency and tongue blade test was positive.
Figure 4: Depapillation on dorsum surface of tongue

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Orthopantograph examination revealed multiple missing and endodontically treated teeth. Generalized moderate-to-severe horizontal bone loss was present. On the right ramus, erosions could be appreciated along the posterior border at the level of the middle and lower one-third, whereas the posterior border of the left ramus showed resorption at the level of upper and middle one-third with rounding of the angle of mandible giving it a characteristic “Tail of the Whale appearance [Figure 5]. These findings were confirmed on a temporomandibular joint view of CBCT. In addition, flattening could be appreciated on the anterio-superior aspect of the left head of the condyle giving it an angled shape. Grade 1 pneumatization of the mastoid air cells could be appreciated as per Al-Fateh and Ibrahim grading system[5] [Figure 6]. For treatment, patient was referred to the Department of Prosthodontics in lieu of the chief complaint.
Figure 5: OPG showing resorption at the left posterior border of the ramus giving a “Tail of the Whale” appearance

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Figure 6: CBCT showing a “Tail of the Whale” appearance, pnuematization of mastoid air cells appreciated between the mastoid process and glenoid fossa

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   Discussion Top


Scleroderma is a multisystem condition of complex pathogenesis. Vascular damage, activation of immune complexes such as stimulated interleukins, upregulated endothelin, MMP-2(matrix metalloproteinase-2), and interferons with excessive synthesis of extracellular matrix are known to cause the development of this condition.[6],[7]

Orofacial tissues are often affected, thus presenting with characteristic features such as thin and taut facial skin due to subcutaneous collagen deposition which leads to a “mask-like” or an “expressionless” or ”Mona-lisa” facies.[8] Hypopigmented/hyperpigmented areas of the skin are also appreciated in patients with scleroderma as a result of the skin lesions. These lesions initially start as indurated areas of the skin with hair loss and sweat gland dysfunction. As the disease progresses, these lesions “burn out” giving areas of hypopigmentation or hyperpigmentation.[9] Another characteristic feature of scleroderma is “purse string-like appearance” due to the circumoral fibrosis resulting in characteristic furrows radiating from the mouth.[10] Further, intraoral fibrosis leads to microstomia. Nutritional deficiencies can lead to atrophy of filiform papillae, which in turn leads to depapillation of the tongue. Initially edema causes swelling of digits which progresses to fibrosis giving it a “claw like” deformity.

The osteolytic changes and atrophy of the jaws are typical radiographic findings of scleroderma.[11] The other radiographic findings observed are resorption of the roots along with the widening of the periodontal ligament space. The condyles, coronoid process, posterior border of the ramus, and angle of mandible show erosions and resorption. Furthermore, these changes can invariably lead to pathological fractures. Few authors have also documented condylysis as findings associated with the TMJ.

The root resorption appreciated is due to osteolytic changes secondary to fibrosis of mucocutaneous tissues. It has been documented that these changes further initiate cell signals to inhibit the antiinvasion factors in the cementum or periodontal membrane cells surrounding the roots. Moreover, deranged calcium, phosphorus, and magnesium levels in dentine along with the scarcity of oxygen tension in the environment can also lead to root resorption.[12]

PDL widening is due to the involvement of the masticatory muscle, which becomes bulky and leads to an increased occlusal loading resulting in primary trauma from occlusion. An increase in the collagen synthesis in scleroderma may also be a possible cause of PDL space widening. In order to make way for the thickened PDL, underlying bone is resorbed which leads to changes in the lamina dura that can either get lost or thinned out.[13],[14]

The pathophysiology behind the bony changes appreciated in the jaws is postulated to be fibrosis of the arterial muscular walls leading to a decrease in the vascularity of the muscles, which further causes increased fibrosis of the muscles. This is followed by atrophy of muscle at the site of attachment. The atrophied muscle exerts pressure on the bone initiating osteolytic changes such as erosions and resorption as the disease progresses in the mandible.[15] Since, there was no sign of a middle ear infection, pneumatization was attributed to age changes in this case [Figure 7].
Figure 7: Postulated pathogenesis for osteolytic changes observed in the mandible

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Fibrosis of the muscles of mastication at the level of attachment of masseter which is at the lowest part of the lateral surface, middle and upper part of the ramus; temporalis at anterior border of ramus; medial pterygoid at medial surface of angle and mylohyoid groove often lead to a characteristic and pathognomonic appearance of the ramus, which is referred to Tail of the Whale appearance. The above mentioned clinical and radiographical findings can be corroborated with the findings documented in the literature [Table 1].
Table 1: Comparison of clinical and radiographic findings of Scleroderma documented in the literature with that of the present case[9],[10],[11],[16]

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Various serological tests can be performed to detect the presence or absence of autoantibodies that are generally associated with the diagnosis of scleroderma. They include antinuclear antibody (ANA) which is positive in various connective tissue and autoimmune disorders; Scl-70 antibody (Scleroderma antibody, Anti-topoisomerase I Antibody) which is positive in up to 60% of adults with systemic sclerosis, and centromere antibody (ACA)/centromere pattern which is present in 50%–96% of people with limited cutaneous scleroderma and strongly associated with CREST syndrome. Our case showed positive results for ANA and Scl-70 which helped in the diagnosis of Scleroderma.

Overall oral health-related quality of life is significantly reduced in Scleroderma. Therefore, it is imperative to have periodic dental evaluation for early diagnosis and management of oral manifestations of Scleroderma [Table 2]. As far as complications are concerned, these patients are at increased risk of pathological fracture even from minor trauma including dental extraction because of resorption at the angle of mandible. Moreover, when treating a patient with diffuse scleroderma, the extent of the heart, lung, or kidney involvement should be considered, and appropriate alteration should be made before, during, and after treatment to prevent any complication.[9]
Table 2: Dental considerations and Management[9],[16]

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   Conclusion Top


Systemic sclerosis has various manifestations in the jaws, “Tail of the whale appearance” being pathognomonic of the disease. As an oral physician knowledge of the clinical and radiologic features can aid in early diagnosis and an effective management of patients with Scleroderma.

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.
Takehara K., Sato S. Localized scleroderma is an autoimmune disorder. Rheumatology (Oxford) 2005;44:274-9.  Back to cited text no. 1
    
2.
Leroy EC. Connective tissue synthesis by scleroderma skin fibroblasts in cell nature. J Exp Med 1972;135:1351-62.  Back to cited text no. 2
    
3.
Haslett C, Chilvers ER, Boon NA, Coolidge NR. Davidson's Principles and Practice of Medicine. 19th ed. London: Churchill Livingstone; 2002. p. 1036-7.  Back to cited text no. 3
    
4.
Alexandridis C, White SC. Periodontal ligament changes in patients with progressive systemic sclerosis. Oral Surg Oral Med Oral Pathol 1984;58:113-8.  Back to cited text no. 4
    
5.
Al–Fateh W, Ibrahim ME. A tomographic study of air cell pneumatization of the temporal components of the TMJ in patients with temporomandibular joint disorders. Eygpt Dent J 2005;51:1835-42.  Back to cited text no. 5
    
6.
Raschi E, Chighizola CB, Cesana L, Privitera D, Ingegnoli F, Mastaglio C, et al. Immune complexes containing scleroderma-specific autoantibodies induce a profibrotic and proinflammatory phenotype in skin fibroblasts. Arthritis Res Ther 2018;20:187.  Back to cited text no. 6
    
7.
Eckes B, Wang F, Moinzadeh P, Hunzelmann N, Krieg T. Pathophysiological mechanisms in sclerosing skin diseases. Front Med (Lausanne) 2017;4:120.  Back to cited text no. 7
    
8.
Anbiaee N, Tafakhori Z. Early diagnosis of progressive systemic sclerosis (scleroderma) from a panoramic view: Report of three cases. Dentomaxillofac Radiol 2011;40:457-62.  Back to cited text no. 8
    
9.
Greenberg MS, Glick M, Ship JA. Burket's Oral Medicine. Hamilton: BC Decker; 2008.  Back to cited text no. 9
    
10.
Bali V, Dabra S, Behl AB, Bali R. A rare case of hidebound disease with dental implications. Dent Res J (Isfahan) 2013;10:556-61.  Back to cited text no. 10
    
11.
Jagger RG, Absi EG, Jeremiah HG, Sugar AW. Bilateral mandibular condylysis in a patient with systemic sclerosis. Dentomaxillofac Radiol 2006;35:461-3.  Back to cited text no. 11
    
12.
de Figueiredo MA., de Figueiredo JA., Porter S. Root resorption associated with mandibular bone erosion in a patient with scleroderma. J Endod 2008;34:102-3.  Back to cited text no. 12
    
13.
Hasan S, Khan R, Haroon MA, Khwaja KJ, Tarannum F, Hussain AR. Systemic sclerosis–a case report and review of literature. Int J Contemp Dent 2011;2:46-53.  Back to cited text no. 13
    
14.
Worth HM. Principles and Practice of Oral Radiologic Interpretation. Chicago: Year Book Medical Publishers, Incorporated; 1963.  Back to cited text no. 14
    
15.
Delantoni A, Matziari E. Osteolysis affecting the jaws in systemic sclerosis: Clinical and osseous changes based on a case presentation. Clin Med Insights Arthritis Musculoskelet Disord 2015;8:65-7.  Back to cited text no. 15
    
16.
Veale BJ, Jablonski RY, Frech TM, Pauling JD Orofacial manifestations of systemic sclerosis. Br Dent J 2016;221:305-10.  Back to cited text no. 16
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7]
 
 
    Tables

  [Table 1], [Table 2]



 

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