Jordi | Journal of Oral DiagnosisOnline SubmissionReview an ArticleSOBEP - Sociedade Brasileira de Estomatologia e Patologia Oral
Volume 2 - 2017


Original Article

DOI: 10.5935/2525-5711.20170027

Oral signs in juvenile dermatomyositis

Mauricio Rocha Dourado1; Tiago João da Silva Filho2; Tuula Salo3

1. DDS MSc. Medical Research Center, University of Oulu, Oulu, Finland. Department of Oral Diagnosis, Piracicaba Dental School (PhD student), Unicamp, Piracicaba, Brazil
2. DDS, MSc.Oral Pathology Post-Graduate Program, Federal University of Rio Grande do Norte, UFRN, Natal, Brazil. Cancer and Translational Medicine Research Unit (PhD student), University of Oulu, Oulu, Finland
3. DDS, PhD. Medical Research Center, University of Oulu, Oulu, Finland. Department of Oral Diagnosis, Piracicaba Dental School (Professor), Unicamp, Piracicaba, Brazil

Corresponding authors: Mauricio Rocha Dourado
E-mail: mauricio_mrd@hotmail.com

Article received on March 1, 2017.
Article accepted on August 11, 2017.

 

ABSTRACT

Juvenile Dermatomyositis (JDM) is a severe inflammatory myopathy characterized by multisystemic vasculopathy during childhood. The general profile consists of symmetric proximal muscle weakness and the presence of characteristic rashes. This case describes an 11-year-old girl with hyperplastic gingival inflammation, telangiectasia and lichenoid retroalveolar striae detected by dental examination visit, when she also showed rash and papules in her hands. Histopathological diagnosis of the retroalveolar mucosa was indicative of unspecific chronic inflammation. Later when muscle symptoms and serum changes were present the JDM diagnosis was confirmed. The patient was successfully treated with systemic corticosteroids and regular professional dental cleaning. In order to get an early diagnosis and effective treatment for the patients, it is important that oral health care providers are aware of the oral manifestations related to systemic diseases, such as JDM.

Keywords: Dermatomyositis; Telangiectasis; Gingiva; Mouth Mucosa.

 

INTRODUCTION

Juvenile dermatomyositis (JDM) is a systemic vasculopathy of unknown etiology, characterized by acute and chronic inflammation of the skeletal muscle and skin1,2. Although rare, JDM is the most common subset of the juvenile idiopathic inflammatory myopathies (JIIMs), representing about 85% of the cases among children3. Advances in treatment with corticosteroids and immune suppressants have reduced the mortality rate of JDM to 1-5%, but the disease is still a cause of significant morbidity4.

Clinically, JDM is characterized by muscle weakness and typical skin involvement. It may also involve multiple other organs, including the gastrointestinal tract, heart, lungs, kidneys and eyes1. The orofacial region can also be affected, but only few studies have focused on its changes in JDM5-7. However, oral lesions can be initial manifestations of the disease, and especially gingival telangiectasia has been proposed as an important diagnostic marker8. Here we present a case of JDM, where the oral mucosa changes together with rash in the hands were the first findings and indicative of diagnosis.


CASE REPORT

An 11-year-old girl was referred to the Oulu University Dental School due to the gingivitis which did not response to the traditional treatment. She had an erythematous generalized lesion in the attached and free gingiva of both upper and lower jaws (Fig. 1A). An accurate intraoral examination showed dilation of the gingival capillaries close to teeth crowns, and also bilateral erythema and non-detachable white lichenoid striae in the retroalveolar mucosa (Fig. 1B and D).


Figure 1. JDM patient at the first appointment had generalized erythematous free gingiva with multiple telangiectasia (A); whitish lichenoid striae in the both retroalveolar mucosal areas, and telangiectasia in the posterior teeth gingiva, presenting also some spontaneous bleeding points (B and D). Biopsy specimen was taken from the area marked as a black circle in B; it shows slight epithelial hyperparakeratosis, pseudoepitheliomatous hyperplasia and a connective tissue with mild chronic inflammation and permeated small vessels (C, x100). Biopsy was signed out as "suspected lupus-like reaction".



The patient had intact teeth without any dental restorative material. A biopsy from the retroalveolar gingiva was carried out. The tissue H&E staining showed unspecific pseudoepiteliomatous epithelial hyperplasia, mild chronic inflammation and was signed out as a "suspected as a lupus-like reaction" (Fig. 1C). The physical examination was remarkable for her hands, presenting papules and erythematous rash in the distal, proximal and metacarpophalangeal joints, compatible with Gottron's papules (Fig.2 A).


Figure 2. Characteristic Gottron's papules in the fingers joints (A). Histological section from one of the papules showing epithelial hyperplasia and mild chronic inflammatory infiltrate within the connective tissue (B, x100). Mild myositis and necrosis are shown in the section of muscle biopsy (C, x200). Calcified tissue removed from the lower jaw gingiva presented some inflammatory cells with areas of dystrophic calcification (D, x200).



The medical history of the patient was non-significant. Because of the skin findings the patient was referred for a skin biopsy. The tissue H&E staining showed epithelial hyperplasia and mild chronic inflammatory infiltrate within the connective tissue (Fig. 2B). A frozen tissue section was analyzed by immunofluorescence for Polyclonal Igs, IgG, IgM, IgA, C3 and fibrinogen to rule out autoimmune diseases, such as lupus erythematosus. The immunofluorescence findings were negative. A couple of months later the patient developed muscle symptoms. Serum analysis showed the presence of Jo-1 autoantibodies and a biopsy of muscle tissue revealed mild myositis and necrosis (Fig. 2C).

The diagnosis of JDM was confirmed based on the clinical, histological and serum findings. The treatment was based on systemic corticosteroids (prednisolone 50 mg daily) and oral cleaning by dental hygienist every two months. The dermatological changes became worse in the middle of the treatment and prednisolone doses were increased.

After the remission of the signs and symptoms the steroid was gradually stopped. After the treatment, the skin appearance improved and muscle power was within normal limits. Despite the main remission, enlarged and condensed tissues in the gingival regions of the teeth 43-44 and 33-36 developed, and histopathological analyses showed necrotic areas with dystrophic calcifications within connective tissue (Fig. 2D). After 23 years of follow-up the patient has remained with no recurrent disease.


DISCUSSION

JDM is the most common phenotype among the JIIMs (onset before 16 years old), with an annual incidence from 1.9-4 cases per million children, depending on the population9-13. Several studies have shown a female predominance, in a ratio as high as 5:1 in a British survey, and an average onset of 7 years1,9,11,14-17. The disease seems to have a bimodal behavior in girls with two peaks, before 6 and around 11 years old (when our patient was diagnosed), but a single peak around 10 years in boys11,18.

Symmetric proximal and axial muscle weakness and the presence of characteristic rashes, i.e., Gottron's papules (erythematous plaques overlying the extensor joint surfaces) or heliotrope rash (a purplish or erythematous rash over the eyelids) are general characteristics of JDM. In addition, the patients often have other photosensitive rashes, including malar erythema, photosensitivity, linear extensor erythema, as well as other cutaneous findings19,20.

Besides the presence of the pathognomonic rash, diagnosis of JDM requires three of four Bohan and Peter criteria: proximal muscle weakness, elevated serum levels of muscle enzymes, electromyographic changes of chronic inflammatory myositis, and histopathological changes of inflammatory myositis7,21. Our patient presented the characteristic rash in her hands, muscle symptoms, serum changes and myositis confirmed by biopsy, which taken together led to a JDM diagnosis.

Interestingly, the patient was referred to the clinic reporting a "gingivitis" resistant to the traditional approaches. The first oral signs of disease were prominent erythema and telangiectasia along gingival margins and lichenoid lesion in retroalveolar mucosa. Later on, a calcified tissue in the lower maxillary gingiva was biopsied. Prominent dilated capillaries along the facial anterior marginal gingiva has been reported previously as one of the diagnostic signs of JDM.

The gingival vasculopathy pattern associated with hyperplasia and gingival bleeding, distinct from periodontal disease and associated with cutaneous disease activity, suggests that gingiva is a possible target tissue for JDM6. Differing from the adult form, the pathogenesis of JDM can be based on vasculopathy rather than simply an inflammation of skin and muscle sites18.

The Table 1 summarizes our case and all previously published JDM cases presenting oral involvement. The most common oral mucosa findings were telangiectasia, erythematous lesions and dysphagia, which can be related to the capillary changes. JDM patients seem to share a unique gingival pattern characterized by gingival erythema, capillary dilation and bush-loop formation6.




Similar to the lesion presented in retroalveolar area in our patient, Ghali et al.5 described reticulated whitish patches on the buccal mucosa and tongue of a 7-year-old girl diagnosed with JDM. Another patient presented depapillated and erosive patches over the dorsum of the tongue7. Ulcers, whitish and erosive patches are changes also described in patients with the adult form of dermatomyositis28,29,30. In the case presented here, the lesion regressed after systemic corticosteroid therapy, indicating close relation with the general disease. The relation between oral whitish/lichenoid lesions and dermatomyositis (juvenile and adult form) needs further investigation.

Calcinosis are not rare in JDM and can occur in 20-40% of the patients19. Cutaneous calcinosis is frequently located on the elbows, knees, and the acral and proximal parts of extremities, whereas calcification of oral soft tissues are uncommon. Only one case of oral soft tissue calcinosis related to JDM has been reported before: bilateral, superficial and painful subcutaneous protrusions, visualized in x-ray as symmetric calcifications in the mandible26. In our case, the calcified gingival tissue was surgically excised and had no remission. Calcified focal points in facial bones should be investigated for systemic diseases such as JDM26.

In the recent years, mortality due to JDM has declined considerably but morbidity related to the myopathy, calcinosis and long-term use of corticosteroids remains a major challenge9. Although the majority (50-60%) of patients with JDM experience a chronic illness course, approximately 24-40% of the patients have a monocyclic course, recovering with appropriate therapy within a 2-year period19. Since our patient did not show recurrence after 16 years from the treatment, we consider that she has fully recovered. Oral health care providers should be aware of the JDM signs in oral cavity, specially aiming to early diagnosis of this systemic disease. As in our case, the oral and skin findings followed by muscle and blood analysis led rapidly to the right diagnosis of JDM.


ACKNOWLEDGMENTS

The author M.R.D. acknowledges the Coordination for the Improvement of Higher Education Personnel, CAPES/Brazil (PDSE Process number 99999.003270/2015-07) and the Medical Research Center MRC/University of Oulu, for providing research grants that partially supported this research.

The author T.J.S.F. acknowledges the National Counsel of Technological and Scientific Development, CNPq-Brazil, for providing research grants through the program Science Without Borders that partially supported this research.


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