FIGURE 1 - uploaded by Heleia Nestal Zibo
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Clinical photograph showing stretched and smooth skin, with a loss of normal facial animation lines (masklike appearance). Sclerotic and inelastic skin surrounded the mouth and the ocular area, and telangiectasias were present all over the face and neck.
Context in source publication
Context 1
... maximum in- cisal opening was 20 mm. The skin of the ocular internal canthus was fibrotic, inelastic, and less pigmented than in the other facial areas (Fig 1). An erythematous and ulcer- ated, calcified, thick bar was noted along the inferior border of the mandibular symphysis and bilaterally on the inferior borders of the mandibular body. ...
Citations
... We have previously found calcification in the nasal spine of patients with SSc and MERR (Memida et al., 2019). There are several reports of calcification in the facial region, such as in the lower jaw and (Chikazu et al., 2008;Nestal-Zibo et al., 2009). The calcification was removed, and histopathological analysis revealed dystrophic calcification confined by collagen fibers (Chikazu et al., 2008). ...
Calcinosis is frequently observed in patients with systemic sclerosis (SSc). The fundamental treatment of calcinosis has not yet been established. During follow-up, calcinosis in the subcutaneous surface is often spontaneously extracted or remains confined by fibrous tissues. We previously identified a new symptom in SSc patients, multiple external root resorption (MERR), and these patients had calcifications in the nasal spine. Here, we report for the first time that calcinosis at the nasal spine in patients with MERR can be replaced by cancellous bone-like tissue. Patients 1 and 2 were a 62-year-old Japanese female and a 45-year-old Japanese female (respectively) with MERR who had been previously treated for SSc (Patient 1: limited type, positive for anti-centromere antibody; Patient 2: diffuse type, positive for anti-Scl70 and anti-SS-A antibodies). Patient 3 was a 57-year-old female with MERR who had been previously treated for SSc (diffuse type, positive anti-Scl-70 antibody) and underwent denosumab injection for osteoporosis. Cone-beam computed tomography (CBCT) and CT images in the calcifications at the nasal spine in Patient 1 and 2 were replaced with cancellous bone-like tissue, but not in Patient 3. Serum laboratory examination was performed to assess the systemic bone disease. All three patients had normal clinical data within the references, apart from slightly higher 1,25-dihydroxyvitamin D levels in Patient 1. SSc patients with calcinosis in the maxillofacial area need to be examined carefully for bone replacement using CBCT or CT.
... Acredita-se que, em decorrência da deposição crônica de colágeno, 10 a 20% dos pacientes tenham reabsorção posterior do ramo da mandíbula ou processo coronoide, mento e côndilo, em níveis variados [17]. A excessiva tensão da pele e dos músculos, associada a um processo isquêmico local, parece explicar a reabsorção óssea que ocorre na ES [15,23]. O processo coronoide e as reabsorções condilares são menos frequentes. ...
Introdução: A esclerodermia é uma desordem pertencente a um grupo de doenças autoimunes do tecido conjuntivo, caracterizada por esclerose na pele, pulmões, coração, trato gastrointestinal, rins e sistema musculoesquelético. Das manifestações orais e periorais, são evidentes aumento do espaço do ligamento periodontal, limitação da função mastigatória, dificuldade no processo de reparo, fibrose da pele, diminuição da abertura bucal, xerostomia, cárie, entre outros.Em virtude das alterações na microcirculação dos tecidos gengivais e dos medicamentos sistêmicos, pode haver inflamação gengival e desenvolvimento de hiperplasia gengival. A xerostomia e as alterações escleróticas das mãos e dedos contribuem para um maior acúmulo de placa nesses pacientes. Objetivo: Relatar o caso clínico de uma paciente portadora de esclerodermia sistêmica (ES) e a associação dessa doença com as manifestações orais e periodontais. Relato decaso: Paciente A.M.S., 47 anos, sexo feminino, diagnosticada com ES há 16 anos, apresentou em associação à doença sistêmica diferentes manifestações orais e periodontais, como aumento do espaço do ligamento periodontal, diminuição da abertura bucal, limitação da função mastigatória, dificuldade no processo de reparo, fibrose da pele, xerostomia, cárie, sangramento gengival, presença de cálculo dental e mobilidade dentária. Conclusão: Os sinais e sintomas apresentados podem interferir na qualidade de vida dos pacientes. Portanto, é de extrema importância o cirurgião-dentista estar atento às manifestações, a fim de orientar e motivar os pacientes com o intuito de diminuir os fatores de risco, proporcionar um tratamento apropriado ou até mesmo realizar um possível diagnóstico da doença.
... Acredita-se que, em decorrência da deposição crônica de colágeno, 10 a 20% dos pacientes tenham reabsorção posterior do ramo da mandíbula ou processo coronoide, mento e côndilo, em níveis variados [17]. A excessiva tensão da pele e dos músculos, associada a um processo isquêmico local, parece explicar a reabsorção óssea que ocorre na ES [15,23]. O processo coronoide e as reabsorções condilares são menos frequentes. ...
... Bone damages were observed, consisting in mandibular resorption, which is a major concern in scleroderma. Excessive skin and muscle tension associated with a local ischemic process seems to explain bone resorption occurring in SD [22,23]. Mandibular angles are most affected, often bilaterally, with changes in patient's profile. ...
Introduction:
Scleroderma is a rare disease of the connective tissue (50 to 200 patients/1 million people; 60,000 patients in France). We conducted a literature review about the orofacial manifestations of scleroderma that have been little studied.
Material and methods:
The 45 articles found in 6 different databases by using the keywords "scleroderma", "systemic sclerosis", "oral medicine", "face" and published between 1944 and 2016 were selected, for a total of 328 patients.
Results:
A total of 1187 orofacial manifestations of scleroderma were identified, occurring mainly in women (84.5%) with a mean age of 40.2 years, 10 years on average after the first manifestation of the disease. The main ones were limitation of mouth opening (69.8%), widening of the periodontal ligament (67.3%), xerostomia (63.4%), telangiectasia (36.2%) and bone lesions (34.5%). Dental root resorptions, pulp and nose calcifications were also reported but with no evident link with scleroderma.
Discussion:
Orofacial manifestations of scleroderma are probably more common than reported. They mostly affect women with a mean age of 40. The most common oral manifestations are limitation of mouth opening, widening of the periodontal ligament and xerostomia. Because of the handicap they may be responsible for, these manifestations must be detected early in order to prevent from functional impairments and from dental and periodontal lesions.
... Calcifications are also frequently observed at sites that are exposed to recurrent microtrauma and where tissue integrity has been compromised (Pugashetti et al, 2011;Reiter et al, 2011). To our knowledge, only 4 SSc patients presenting with calcinosis in the facial area have been reported so far (coronoid process of mandible, along mandibular arch, nose, TMJ) (Temekonidis and Drosos, 2001;Alpöz et al, 2007;Chikazu et al, 2008;Nestal-Zibo et al, 2009). Calcifications within the periodontal ligament space (PDL) and the pulp chamber, that may also be related to the general spectrum of dystrophic calcinosis, have also been evidenced by Cone-Beam Computed Tomography (CBCT) approach in a patient with diffuse SSc (Jung et al, 2013). ...
Systemic sclerosis (SSc) is a rare multisystem connective-tissue disorder characterized by the triad fibrosis, vasculopathy and immunity dysregulation. This chronic disease has a significant impact on the orofacial region that is involved in more than two-thirds of the cases. SSc patients can show a wide array of oral manifestations, which are usually associated with a severe impairment of the quality of life. They often present a decreased the salivary flow and a reduced mouth opening that contribute substantially to the worsening of the oral health status. Therefore, SSc patients require specific and multidisciplinary interventions that should be initiated as early as possible. The identification of specific radiological and clinical signs at the early stage will improve the management of such patients. This study reviews the wide spectrum of orofacial manifestations associated with systemic sclerosis and suggests clues for the oral management that remains challenging. This article is protected by copyright. All rights reserved.
... We know of 3 previously published reports of calcinosis in the facial area. [4][5][6] The most recent article reports a 62year-old patient with bilateral calcified lesions along the mandibular body associated with diffuse systemic progressive sclerosis in addition to lesions in other areas. The lesions were excised through the subcutaneous layer. ...
Antisynthetase syndrome is a subgroup of the idiopathic inflammatory muscle diseases and is characterised by myositis, interstitial pulmonary disease, arthritis, and Raynaud phenomenon. We report the case of a 34-year-old woman with known antisynthetase syndrome who presented with subcutaneous calcinosis bilaterally in the submandibular region.
... [8][9][10][11][12] Ischemia caused by limited mobility and extrinsic pressure from rigid tissues may be responsible for these abnormalities. 13,14 Case 1 demonstrated a remarkable loss of alveolar bone associated with a deep periodontal pocket between the affected teeth. The bone resorption and the loss of periodontal attachment were localized without evidence of subgingival calculus or endodontic infection. ...
A multicenter, open-label study was performed to investigate the safety and tolerability of bosentan in Japanese patients with systemic sclerosis (SSc) and secondary digital ulcers. Twenty-eight patients were enrolled. The safety and tolerability of bosentan was monitored over 52 weeks of study treatment (primary end-point), while incidence and healing of digital ulcers were also assessed up to week 16. The following adverse events occurred in 5% or more of patients during the 52-week treatment period: upper respiratory tract infection (50.0%), abnormal liver function tests (42.9%), digital ulcers (25.0%), anemia (17.9%), peripheral edema (14.3%), diarrhea (10.7%), urinary tract infection (7.1%), arthralgia (7.1%), constipation (7.1%) and herpes zoster (7.1%). Eight patients experienced at least one serious adverse event, including drug-related serious adverse events in two patients, which were abnormal liver function tests and fluid retention (pericardial effusion) in one patient each. During the 16-week observation period, seven out of 28 patients (25%) developed new digital ulcers. In this study, adverse events were comparable with those previously reported with bosentan. Approximately half of the patients had adverse events associated with abnormal liver function tests, thus we conclude that liver function should be monitored regularly during treatment with bosentan.
