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Childhood Systemic Lupus Erythematosus; a Rare Multisystem Disorder: Case Report of a 3-year-old Girl with Oral Involvement as a Primary Sign

Childhood-onset systemic lupus erythematosus (cSLE) is a severe, chronic, multi-organ, and systemic autoimmune disorder characterized by inflammatory and autoimmune reaction in several organs. The occurrence of systemic lupus erythematosus (SLE) in children is very rare. About 20% of all SLE cases are diagnosed during the first two decades of life and the disease is extremely rare before age of 5 years. In this case report, we present a 3-year-old girl presented with SLE symptoms similar to primary herpetic gingivostomatitis. Early diagnosis lead to proper treatment of the disease and it is important to decrease oral complications in children. Diagnosis could be improved by introduce new cases to provide valuable information for dentists based on diagnostic criteria, therapeutic steps and complication of treatment of SLE in Children. Therefore, it could be concluded that dentists involved in pediatric dentistry should consider and work out on the clinical signs of SLE in children with history of oral herpes virus infection.


Introduction
Childhood-onset systemic lupus erythematosus (cSLE) is a severe, chronic, multi-organ, systemic autoimmune disorder presented by inflammatory and autoimmune reaction in multiple organs [1][2]. Although the cSLE have the same pathophysiology compared to adult type SLE, but the initial clinical presentation of cSLE is observed more sever [3][4][5][6][7][8]. In addition, the abnormal appearance common in this age group is frequently responsible for major diagnostic delay [5]. Considering the most report series, the oral lesions are minor [4][5][6][7][8][9][10], therefore, they usually do not come to dental pediatric centers. The incidence of cSLE varies from 0.3 to 0.9 per 100,000 children-years [11][12]. It has been demonstrated that both renal and central nervous system (CNS) organs tend to be more involved in pediatric patients than in adults [13][14].
Only 20% of all patients with SLE are diagnosed during the first two decades of life and the disease is extremely rare in those below 5 years of age [1,15].
The diagnosis and treatment of patients with cSLE is based on the European evidence-based recommendations for diagnosis and treatment of cSLE [16]. Although several studies have reported the clinical and laboratory characteristics of patients with cSLE [1,3,17], based on literature review, there is no presentation of the disease in younger children (below 5 years of age). In this paper, we report a cSLE case of 3-year-' old girl, referred to Pediatric Department, Dental School, Medical University of Kerman, Iran, to provide a valuable information for dentists to improve diagnostic criteria, therapeutic steps in children with oral complications.  There was epidermal atrophy, mononuclear cell infiltration and orthokeratosis but there was no blister for mation. The pathologist differential diagnosis included lupus erythematosus and pemphigus vulgaris.  and oral hydroxychloroquine (5mg/kg once daily) was administered. The patient was followed up and after 10 days of therapy, the oral aphthous lesions were recovered and the malar rash disappeared (Figure 2). The patient was able to eat and drink and her weight increased after a month. In 6-month follow-up visit, there was no relapse and no lesion was detected. There was no renal and CNS involvement. The patient was recommended to continue the therapeutic regimen until 6 years of age. Relevant written consents have been obtained before publishing the case report.

Discussion
Infantile SLE is an extremely rare and to the best of our knowledge, only 13 cases have been reported in the world [19][20] ( Table 3). The clinical course of the cSLE is progressive and associated with more severe symptoms and more progressive course with permanent sequellae [1,3,14].  [20] demonstrated that infantile SLE had a more progressive course and was associated with more destructive symptoms than adult SLE. They also reported anemia and thrombocytopenia as frequent findings in infantile SLE, whereas eucopenlia was rare. Conversely, leukocytosis has been reported to be more common in infantile SLE [3,20]. In our case, there was no anemia and thrombocytopenia   [3,[19][20][21][22]. In the current report, we observed significant and abrupt response of the symptoms to the initial dose of prednisolone. However, in previously reported cases, the response to the corticosteroids were limited and thus adding other agents were required [3,[19][20]. In brief, the rare case of infantile SLE can be treated successfully treated with oral prednisolone.
Timely diagnosis and treatment is the key step in treatment of cSLE. The diagnosis in this age group should be based on the clinical suspicion.

Conclusion
Since cSLE is extremely rare in individuals younger than 5 years of age, and concerning the oral symptoms of SLE in children, dentists' awareness of these symptoms leads to earlier diagnosis and therefore better treatment.