| کلیدواژههای انگلیسی مقاله |
Systemic Lupus Erythematosus (SLE), Herpetic gingivostomatitis, Autoimmune disorder, IntroductionChildhood-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,- 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,- 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-&apos, 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. Case ReportA 3-year-old girl was referred to Pediatric Dentistry Department with severe white (keratotic) lesions on palate and buccal mucosa in oral cavity and lips along with odynophagia and inability to eat for three months. She had general gingival involvement and received analgesics and antibiotics including acetaminophen and Cefixime without any recovery. She had a history of malaise, fever, and fatigue at the onset of the disease along with weight loss (4 kg during 3 months). After one month, were observed that oral and facial lesions were gradually progressed. On physical examination, there was no lesion in the mucosa of eye and nose while a pale lesion on the face especially around the cheek and nose was observed. There were some cutaneous lesions on her scalp but no lesions on arms or legs. She had no history of primary gingivostomatitis. A positive family history (her grandfather and uncle) of rheumatoid arthritis was reported. The patient was the third and the last child of the family, being 15years younger than her siblings. There was no history of congenital disorders. Consultation with the Department of Diagnosis of Oral Diseases was carried out and according to the age of the child and acute oral symptoms, the primary diagnosis of the disease was established as primary herpetic gingivostomatitis according to epidemiology, clinical sign, and history. Based on response to the treatment regimen, other differential diagnoses including aphthous lesion, vesiculo bullous lesions, and Hand-Foot-Mouth disease were ruled out.The first impressions of patient were considered primary herpetic gingivostomatitis and the girl was treated with Nystatin suspension, Diphenhydramine, and Magnesium aluminum. The patient was followed up by supportive care and after 10 days, the oral aphthous lesions and the malar rash were exacerbated (Figure 1,). Concerning the worsening of the lesions, further evaluations were scheduled. Subsequently by assessing the complete blood cell count and differential count (CBC &,amp amp diff), biopsy of facial lesion (patient did not cooperate for oral lesion biopsy), and consultation with a rheumatologist, the SLE was diagnosed. Figure 1. Oral lesions, ulcer in hard palate and buccal mucosaBlood investigations revealed a hemoglobin concentration of 15.4 g/dL, and white blood cell count of 13.2&,times 109/L and platelet count of 486&,times 109/L. The lymphocyte percentage was 58% compared to 35% neutrophil. Her erythrocyte sedimentation rate (ESR) was 31 mm at the first hour, and C-reactive protein (CRP) was positive. Her serum creatinine level was 0.52 mg/dL, and blood urea nitrogen (BUN) was 14 mg/dL. To rule out SLE and other collagen vascular diseases, the serologic tests showed positive antinuclear antibody (ANA), negative Anti-dsDNA and positive cytoplasmic antineutrophil cytoplasmic antibodies (C-ANCA). The serum level of C3-C4 complement fractions were reported normal (Table 1,). Skin biopsy from the malar rash revealed non-specific chronic inflammation (dermatitis) with mononuclear cells without viral inclusion bodies.TestResultNormal ValueW.B.C130004000-11000R.B.C5.024.3-5.2&,times 106Hemoglobin13.512-16Hematocrit4147-52M.C.V81.778-98M.C.H26.921-26M.C.H.C32.932-36Platelet386000150000-400000Creatinine0.520.5-1.1AST3715-45ALT2015-45ALKP355180-1200ZN7170-120ESR310-20CH5090101-300Anti-dsDNA9+/-C31.5883-177 mg/dlC40.3515-45 mg/dlANA3.51.2P-ANCA1.2-C-ANCA10.6-G6PDNORMAL-ANA-Screen1.1-CRP Quantitative90/8-4 ALKP, alkaline phosphatase ALT, Alanine Aminotransferase ANA, antinuclear antibodies AST, aspartate Aminotransferase CRP, C-reactive protein ESR, erythrocyte sedimentation rate G6PD, Glucose-6-phosphate dehydrogenase MCH, mean corpuscular hemoglobin MCHC, mean corpuscular hemoglobin concentration MCV, mean corpuscular volume RBC, red blood cell WBC, white blood cell |