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Table of Contents
Year : 2021  |  Volume : 5  |  Issue : 3  |  Page : 148-154

Challenges in the management of oral manifestations in a patient with limited systemic sclerosis

1 Oral Medicine Specialist Program, Universitas Padjadjaran, Bandung, Indonesia
2 Department of Oral Medicine, Faculty of Dentistry, Universitas Padjadjaran, Bandung, Indonesia

Date of Submission17-Jun-2021
Date of Decision03-Aug-2021
Date of Acceptance30-Aug-2021
Date of Web Publication18-Oct-2021

Correspondence Address:
Yannie Febby Martina Lefaan
Oral Medicine Specialist Program, Faculty of Dentistry, Universitas Padjadjaran University, Bandung 40132,
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/SDJ.SDJ_93_21

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Background: Limited systemic sclerosis (SSc), or scleroderma, is characterized by widespread vasculopathy, excessive multiorgan fibrosis, and autoantibody. The early stages of SSc are challenging to diagnose because of their similarity to other autoimmune conditions. Inappropriate SSc treatment can increase the risk of disability, morbidity, and mortality. Different pathogenesis pathways incur various manifestations in the skin and the oral cavity. In this scenario, dentists play an essential role in managing oral SSc manifestations. Proper oral examination, diagnosis, and therapy help to increase the confidence and patient’s quality of life. Case Report: A 24-year-old female patient with SSc was referred from the Internal Medicine Department to the Oral Medicine Department because of lip soreness for 2 weeks, resulting in difficulty eating and opening the mouth. A complete anamnesis and clinical examination were done. The patient was diagnosed with an oral ulcer caused by SSc, cheilitis exfoliative, drug-induced pigmentation, xerostomia, and acute pseudomembranous candidiasis. The oral lesion in this patient was concluded as an oral ulcer caused by SSc because the patient had already stopped using methotrexate for 2 weeks before the ulceration appeared. The oral treatment included sodium chloride (NaCl 0.9%), vaseline album, hyaluronic acid mouthwash, and nystatin. Oral lesions had a significant improvement after 3 days of treatment. Conclusion: The SSc manifestation that appeared on the oral cavity of the patient as microstomia, tongue stiffness, and oral ulcer resulted in inadequate clinical examination, and diagnosis. Treatments for this oral SSc were challenging.

Keywords: Fibrosis, limited systemic sclerosis, microstomia, scleroderma, xerostomia

How to cite this article:
Lefaan YF, Setiadhi R. Challenges in the management of oral manifestations in a patient with limited systemic sclerosis. Sci Dent J 2021;5:148-54

How to cite this URL:
Lefaan YF, Setiadhi R. Challenges in the management of oral manifestations in a patient with limited systemic sclerosis. Sci Dent J [serial online] 2021 [cited 2021 Nov 27];5:148-54. Available from: https://www.scidentj.com/text.asp?2021/5/3/148/328429

  Background Top

Systemic sclerosis (SSc), or scleroderma, is an autoimmune multisystem condition characterized by extensive fibrosis at the skin and organs, such as joints, tendons, gastrointestinal tract, lungs, heart, blood vessels, and kidneys.[1],[2],[3],[4] SSc is a rare condition, first described by Maurice Raynaud in 1865. Risk factors like chemicals, thermal or mechanical injuries, hormonal imbalances, steroids/stress-induced trigger epigenetic changes in microRNA (miR) expression, especially miR-29 and miR-155.[2]

The incidence of SSc in the United States is 9–19 cases per million people a year, with a prevalence of 25.6 cases per million people a year.[5] The SSc incidence is more often predominant in women than in men at a ratio of 4:1, with onset at the age of 30–50 years.[6] SSc is in the third rank of the most occurring autoimmune diseases at Dr. Hasan Sadikin Hospital, Bandung, with the number of cases increasing from 189 cases in 2013 to 196 cases in 2014.[4]

The management of patients with SSc is challenging to diagnose and treat. The early SSc stages are difficult to diagnose because they are similar to other autoimmune conditions. The main SSc characteristics are widespread vasculopathy, excessive multiorgan fibrosis, and autoantibody production. Multiorgan fibrosis and autoantibody conditions can cause disability, increasing morbidity, and mortality if the treatment is inappropriate. The success of SSc therapy depends on its pathogenesis, so multiple drugs are needed according to the pathogenesis to treat SSc. Until now, there has been no specific treatment for SSc.[7]

SSc can also give manifestations in the oral cavity, including microstomia, xerostomia, telangiectasia, increased caries, tooth loss, tongue stiffness, pseudoankylosis, temporomandibular joint disorders, loss of gingival attachment (recession), periodontal tissue damage, and mandibular bone resorption.[8],[9] Dentists and patients may be significantly affected by the condition of microstomia and fibrosis in the oral cavity. It causes the mouth opening to be less than 40 mm, limiting clinical examination of the oral cavity, maintaining oral health becomes difficult, impairing speech, and mastication functions.[8],[9],[10] Therefore, the purpose of this case report was to describe the challenges in treating the oral manifestations of an SSc patient.

  Case Report Top

A 24-year-old female patient was referred from the Internal Medicine Department with limited SSc, interstitial lung disease, a drug-induced liver injury that cause by methotrexate, and inflammatory anemia. The chief complaint was sore lips that were started 2 weeks before and resulted in difficulty eating and opening the mouth. The patient denied a history of recurrent stomatitis, toothache, and food and drug allergies. The patient was known to have suffered from SSc since 2013 but only went to the rheumatology department regularly after 2016 onward. The diagnosis of scleroderma was confirmed with an antinuclear antibody test. The result was a reactive nucleolar pattern, titer 1:10,000, and a positive result for fibrillarin was obtained with the SSc panel. Medication that the patient had routinely taken since 2016 included methotrexate, methylprednisolone, folic acid, calcium carbonate, nifedipine, and acetylsalicylic acid. Clinical examination reveals sclerodactyly [Figure 1]A, multiple brownish red macules, 0.5–1 cm in size spreading over the face and beak-pick nose [Figure 1]B, numerous brown macules on the upper lip and lower lip (0.3–0.5 cm in size, apparently dry and exfoliative) [Figure 1]C, and a fish-mouth [Figure 1]D.
Figure 1: The extraoral conditions: (A) Sclerodactyly. (B) Multiple brownish red macules spread over the face and beak-pick nose. (C) A fish-mouth. (D) Microstomia

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Erosive lesions covered with pseudomembranes, multiple, irregular, at the upper and lower labial mucosa accompanied by telangiectasia, and fibrosis were observed with an intraoral examination [[Figure 2]A and B]. A single erythematous painful lesion, covered with a hyperkeratotic, irregular shape at the patient’s left buccal mucosa and anterior ventral tongue, was observed [[Figure 2]C and H]. We also found multiple dark brown macules, irregular shapes at the palate [Figure 2]D, and painful multiple erythematous lesions on the midline of the soft palate [Figure 2]E. Raynaud’s phenomenon is associated with blanching of the dorsum of the tongue [Figure 2]F. There were two painful oval ulcerations at the left lateral tongue with a yellowish-white base [Figure 2]G.
Figure 2: Clinical characteristics of the patient during the first visit. (A) and (B) Multiple erosive lesions covered with pseudomembranes accompanied by telangiectasia and fibrosis. (C) and (F) A single irregular and painful erythematous lesion covered with hyperkeratotic at the left buccal mucosa and anterior ventral tongue. (D) Multiple irregular dark brown macules on the palate. (E) Painful multiple erythematous lesions on the midline of the soft palate. (F) A blenching area at the dorsum of the tongue, with a fissure and lobulated tongue. (G) and (H) Oval ulceration with a yellowish-white base on the anterior dorsum and lateral of the left tongue

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The symptoms were diagnosed as oral ulcer caused by SSc with differential diagnosis of drug-induced oral ulcer caused by methotrexate and acetylsalicylic acid and or paracetamol, cheilitis exfoliative, and drug-induced pigmentation in the palate and lip of the patient. Patients were treated with a hyaluronic acid (HA) mouthwash, sodium chloride (0.9% NaCl) to compress the lesion on the lips, and a vaseline album.

Three days later, the patient reported that the pain on the lips began to improve, but the ulcers on the left tongue worsened. The mouth opening was decreasing, but she was still able to eat well. Extraoral and intraoral examinations were found to be challenging because of muscle stiffness and trismus. The mouth opening of the patient was less than two fingers. The erosive lesions previously covered with pseudomembrane at the upper and lower labial mucosa were healed [Figure 3]A and B. Erythematous hyperkeratotic lesions on the left buccal mucosa and anterior dorsum of the tongue were reduced [Figure 3]C and D. There were irregular multiple dusky-brown macules on the palate [Figure 3]E. White plaques could not be scrapped off at the dorsal part of the tongue [Figure 3]D. The ulceration at the left lateral part of the tongue worsened [Figure 3]F. The diagnosis at this visit was an oral ulcer caused by SSc (healing), drug-induced pigmentation, xerostomia, and oral thrush. The patient was instructed to continue the therapy at this visit, and a nystatin oral suspension was given to treat the oral thrush.
Figure 3: Clinical characteristics during the first control. (A) and (B) The erosive lesions were covered with pseudomembranous (healing). (C) Erythematous lesions with hyperkeratosis in the buccal mucosa and anterior dorsum of the tongue (reduced). (D) White plaque could not be scraped off. (E) Irregular multiple dusky-brown maculae on the palate. (F) The ulcerated lesions at the lateral part of the tongue worsened

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In the third evaluation, the general condition worsened a week later because of septic shock, hospital-acquired pneumonia (HAP), and the influence of a sedative. The patient was transferred to an intensive care unit (ICU). The extraoral examination revealed dry lips, peel-off, and a crack at the left commissure, which tend to bleed easily. Meanwhile, the intraoral examination cannot be assessed [Figure 4].
Figure 4: Clinical characteristics during the second control a week. The patient had dry lips and a crack at the left commissure that tended to bleed

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The diagnosis at this visit was xerostomia and angular cheilitis. An oral hygiene instruction was given to the nurse in the isolation room to clean the oral cavity of the patient using 0.12% chlorhexidine digluconate at least twice a day and compressed the lips with 0.9% NaCl as often as possible. Nonetheless, the lungs of the patient worsened, and the patient died 5 days later.

  Discussion Top

Our patient was diagnosed with limited SSc with calcinosis, Raynaud’s phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia (CREST syndrome) based on anamnesis, physical examination, and laboratory examinations at the rheumatology department.[1-4],[6] There are two known types of SSc. The diffuse cutaneous SSc is characterized by a thickening of the skin that can affect the skin, muscles, joints, blood vessels, lungs, kidneys, heart, and other organs. The limited cutaneous SSc is characterized by symptoms in the head, face, feet, and elbows and exhibits the characteristics of CREST syndrome.[4],[11]

SSc with oral manifestations is rare, and only a few cases have been discussed in the literature. SSc manifestations in the oral cavity that we found, in this case, included the beak-pick nose, fish-mouth, microstomia, tongue fibrosis, muscle stiffness, erosive and ulceration in the oral cavity telangiectasia xerostomia, and fungal infections.[1],[9],[12]

A patient with SSc has a change in facial appearance caused by the fibroblast abnormality. Fibroblast of patients with SSc does not respond to tumor growth factor-α, which, under normal conditions, is responsible for the suppression of connective tissue growth factor (CTGF), so that basal CTGF levels are higher than normal fibroblasts.[2],[3],[6] The skin and other organs, such as the gastrointestinal tract, liver, heart, lungs, and salivary glands, are affected by fibroblasts in scleroderma. Deposits of fibroblasts in the acinar cells of the salivary glands resulting changes in the anatomy and function of the salivary glands and causing xerostomia.[13],[14]

Our patient did not report dryness in the oral cavity, but in the intraoral examination, the mouth mirror stuck to her tongue and buccal mucosa, debris stuck on her teeth, and no saliva pooling was observed. Therefore, for these symptoms, the patient was diagnosed with mild xerostomia (Challacombe scale).[15] Xerostomia is a condition resulting from reduced salivary flow. In this case, the xerostomia etiology was caused by hypofunction of salivary glands caused by SSc and long-term use of methotrexate. After prolonged use, xerostomia might be caused by methotrexate, an immunosuppressive or chemotherapy agent, incurring in acinar cells after prolonged use. In general, some drugs that act on the central nervous, such as antidepressants, anticholinergics, antispasmodics, antihistamines, antihypertensives, sedatives, diuretics, and bronchodilators, can affect the saliva secretion mechanism, which is dependent on the autonomic nervous system and through specific receptors found in the salivary glandular (nerve-mediated salivary).[16]

The flow rate of saliva and changes in saliva function and composition are affected by the hypofunction of the salivary glands. One of the functions of saliva is as a lubricant to reduce the mechanical irritation of the oral mucosa while eating and speaking. The dry conditions in the oral mucosa of the patient were caused by the absence of lubricant lining in the oral cavity, worsening the ulceration on the left lateral side of the tongue. Saliva comprises 99.5% water and 5% solid components with antimicrobial effects, such as thiocyanates, lysozyme, lactoperoxidases, lactoferrin, and immunoglobulin proteins, which maintain homeostasis of the oral cavity.[17] The altered saliva composition composes an opportunity for the oral microbiome, especially fungal, to colonize the oral cavity and become opportunistic infections. This oral candidiasis condition was seen on the dorsum of the tongue.[18],[19]

Instruction to compress the lesion on the lips using 0.9% NaCl three times a day and apply a thin layer of vaseline album to the dry, exfoliative lips were given to the patient and family. The 0.9% NaCl and vaseline album application was expected to moisten the lips and delay water evaporation, preventing dry lips and accelerating healing. Humid conditions could help cells grow, divide, and migrate rapidly to optimize the formation of new tissue.[20],[21]

Ulceration of the oral cavity can also be caused by SSc and drug-induced oral ulceration. A complete anamnesis and clinical examination are required to establish the diagnosis. The condition of the oral lesion in this patient concluded that it was not a drug-induced oral ulcer because the patient had already stopped using methotrexate 2 weeks before the ulceration appeared. Prescription of HA mouthwash to treat oral ulceration, rather than another anti-inflammatory drug like corticosteroid mouthwash, was selected because of its efficacy and safety. Faster pain relief, lower risk of complications, discomfort, and drug interaction are provided by HA. In contrast, the oral corticosteroid group has more side effects than HA, such as developing oral thrush.[22] HA is a linear polymer of gluconic acid and N-acetylglucosamine disaccharides having a primary tissue healing function. HA has a hygroscopic macromolecular nature and is a highly osmotic solution. It may control tissue hydration during inflammatory processes in the oral mucosa or as a response to tissue injury, resulting in ulcer formation and acceleration of re-epithelialization through the proliferation of basal keratinocyte cells.[23]

Sclerodactyly and microstomia in the patient worsened in the third control, affecting the treatment. Sclerodactyly characteristics are shiny and tightening or stretching of fingers and toes.[12],[24] The affected fingers are difficult to move.[6],[11],[12] Muscle stiffness and sclerodactyly conditions influenced the ability of the patient to clean the oral cavity and tongue routinely. The patient needed to be helped by her family to clean her mouth. However, as the mouth opening was getting smaller day by day, the relatives of the patient found it challenging to help, especially considering the posterior area. Therefore, the ulceration healing process in the left lateral part of the tongue of the patient was interrupted and worsened.

The patient stayed in bed at the hospital for 2 weeks making the patient susceptible to contracting HAP. HAP is an infection of the upper respiratory tract that occurs due to bacteria obtained from the hospital environment.

A week later, the patient’s general condition worsened because of septic shock, which is one of the most common serious complications from HAP.[25] Patients with autoimmune disease are at high risks of bacterial infections, particularly a tendency to have lung infections because of the immune response effect to immunosuppressive therapy.[26] Patients with severe difficulty breathing may need a ventilator in ICU.[27]

Patients who stay in the ICU may feel anxious and depressed; therefore, many of them may need sedatives and analgesics.[27] The patient was admitted to the ICU, but the general condition did not improve, and the patient passed away 5 days after being treated at ICU. Successful SSc treatment concerning both general and oral manifestations depends on the pathogenesis progression and therapy, in general. The absence of therapeutic markers causes difficulty in managing patients with SSc.[6]

  Conclusion Top

SSc manifestation appeared in the oral cavity of a patient diagnosed with microstomia, tongue stiffness, and oral ulcers, resulting in an inadequate clinical examination, diagnosis, and treatment. Proper diagnosis and treatment are the current challenges in treating oral SSc manifestations.

  Acknowledgments Top

We thank the Department of Internal Medicine and Rheumatology staff, Faculty of Medicine Dr. Hasan Sadikin Hospital for their interprofessional collaboration and especially thankful to the patient’s family who has permitted to publish this case.

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.

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Conflicts of interest

There is no conflict of interest.

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