Background and aim of the work: Systemic lupus erythematosus (SLE) is a multisystem autoimmune disease. Although eyes are seldom affected, this case report presents a 13-year-old Iranian girl with Raccoon eyes and neuropsychiatric manifestations as an initial presentation of SLE. Case presentation: The patient was admitted to the pediatric rheumatology inpatient, Mofid Children's Hospital, Tehran with swelling and ecchymosis around the eyes (Racoon eyes) as well as anorexia, abdominal pain, weight loss, mood disorders and hallucinations. Complete blood count showed normocytic, normochromic anemia, leukopenia, lymphopenia and thrombocytopenia. The C-reactive protein, erythrocyte sedimentation rate, liver and renal functional tests, and urine analysis were normal. The complement levels decreased, antinuclear antibody (ANA) and anti-double stranded DNA (anti-dsDNA) tests were positive. Bilateral pleural effusion and mild pericardial effusion were seen. Bone marrow aspiration showed mild hypocellularity without any evidence of malignancy. The diagnosis of neuropsychiatric SLE (NP-SLE) was held. She was treated by the pulse methyl prednisolone (30 mg/kg/d) and intravenous cyclophosphamide (500 mg/m2), oral prednisolone and hydroxychloroquine. Her appetite improved while hallucination and aggressive behavior decreased. Peri-orbital swelling and ecchymosis decreased. After one year, her appetite became normal; mood disorders, panic, phobic attacks and hallucinations were completely remedied. Swelling and ecchymosis around the eyes were eliminated. Oral prednisolone 10 mgday and hydroxychloroquine (5 mg/kg/day) were continued. Conclusion: SLE may present with Raccoon eyes. Rapid detection and treatment of the disease based on clinical symptoms is critical for these patients. Prednisolone and cyclophosphamide are the best choice for treatment of the disease in children. Â© 2020 Egyptian Society of Rheumatic Disease

Ghasemi, S.

Jari, M.

Shiari, R.

eprints Iran University of Medical Sciences

The Egyptian Rheumatologist 42 (2020) 241–243Contents lists available at ScienceDirectThe Egyptian Rheumatologistjournal homepage: www.elsevier .com/locate /e j rA 13-year-old systemic lupus erythematosus girl initially presentingwith Raccoon eyes and neuropsychiatric manifestationshttps://doi.org/10.1016/j.ejr.2020.02.0011110-1164/ 2020 Egyptian Society of Rheumatic Diseases. Publishing services provided by Elsevier B.V.This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).Peer review under responsibility of Egyptian Society of Rheumatic Diseases.⇑ Corresponding author at: Department of Pediatric Rheumatology, ImamHossein Children,s Hospital, Isfahan University of Medical Sciences, Isfahan, Iran.E-mail address: mjari14@yahoo.com (M. Jari).Mohsen Jari a,⇑, Reza Shiari b, Saeideh Ghasemi caDepartment of Pediatric Rheumatology, Imam Hossein Children,s Hospital, Isfahan University of Medical Sciences, Isfahan, IranbDepartment of Pediatric Rheumatology, Mofid Children’s Hospital, Shahid Beheshti University of Medical Sciences, Tehran, IrancDepartment of Pediatrics, Ali Asghar Children’s Hospital, Iran University of Medical Sciences, Tehran, Irana r t i c l e i n f o a b s t r a c tArticle history:Received 12 January 2020Accepted 1 February 2020Available online 7 February 2020Keywords:Systemic lupus erythematosusOcular involvementRaccoon eyesNeuropsychiatricBackground and aim of the work: Systemic lupus erythematosus (SLE) is a multisystem autoimmune dis-ease. Although eyes are seldom affected, this case report presents a 13-year-old Iranian girl with Raccooneyes and neuropsychiatric manifestations as an initial presentation of SLE.Case presentation: The patient was admitted to the pediatric rheumatology inpatient, Mofid Children’sHospital, Tehran with swelling and ecchymosis around the eyes (Racoon eyes) as well as anorexia,abdominal pain, weight loss, mood disorders and hallucinations. Complete blood count showed normo-cytic, normochromic anemia, leukopenia, lymphopenia and thrombocytopenia. The C-reactive protein,erythrocyte sedimentation rate, liver and renal functional tests, and urine analysis were normal. Thecomplement levels decreased, antinuclear antibody (ANA) and anti-double stranded DNA (anti-dsDNA)tests were positive. Bilateral pleural effusion and mild pericardial effusion were seen. Bone marrow aspi-ration showed mild hypocellularity without any evidence of malignancy. The diagnosis of neuropsychi-atric SLE (NP-SLE) was held. She was treated by the pulse methyl prednisolone (30 mg/kg/d) andintravenous cyclophosphamide (500 mg/m2), oral prednisolone and hydroxychloroquine. Her appetiteimproved while hallucination and aggressive behavior decreased. Peri-orbital swelling and ecchymosisdecreased. After one year, her appetite became normal; mood disorders, panic, phobic attacks and hallu-cinations were completely remedied. Swelling and ecchymosis around the eyes were eliminated. Oralprednisolone 10 mgday and hydroxychloroquine (5 mg/kg/day) were continued.Conclusion: SLE may present with Raccoon eyes. Rapid detection and treatment of the disease based onclinical symptoms is critical for these patients. Prednisolone and cyclophosphamide are the best choicefor treatment of the disease in children. 2020 Egyptian Society of Rheumatic Diseases. Publishing services provided by Elsevier B.V. This is anopen access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).1. IntroductionSystemic lupus erythematosus (SLE) is a complex, chronic, mul-tisystem autoimmune disease affecting almost all body organs. Theprevalence of SLE varies in different parts of the world [1,2]. Theonset of clinical manifestations in SLE is variable and may haveimplications on the disease course, remission and clinical relapse.SLE is rarely associated with ocular involvement and may affectfewer than 5% of patients during the first year. However, more than30% of patientsmay eventually present ocular involvement over thecourse of the disease or during the disease evolution. Lupusretinopathy is one of themost prevalent vision-threatening compli-cations [3,4]. The initial presentation of SLE with neuropsychiatric(NP) symptom is very rare. However, over the course of the disease,22–95% of the patients finally develop NP symptoms such as head-ache [5]. Gene polymorphisms of TNF-a and IL-6were implicated inSLEwhile their serum levels tended to be related to the NPmanifes-tations [6]. In this study, we reported a teenage girl with SLE whowas initially presented with Raccoon eyes (peri-orbital ecchymo-sis), NP symptoms, and peripheral neuropathy.2. Case reportA 13-year-old Iranian girl, who presented with ecchymosis andswelling signs around both eyes (peri-orbital), especially on the leftside, was admitted in Pediatric Rheumatologic ward, Mofid Chil-dren,s Hospital, Shahid Beheshti University of Medical Sciences,242 M. Jari et al. / The Egyptian Rheumatologist 42 (2020) 241–243Tehran, Iran. The swelling and ecchymosis around the eyes arose inthe preceding 20 days and then gradually increased. After that, theleft eye had blurred vision and lessened the eye sight. The patientwas admitted to another hospital for a week with a diagnosis ofseptal cellulitis and received intravenous clindamycin. The pastmedical history of the patient revealed that she had anorexia,abdominal pain and constipation about 18 months ago and wastreated with omeprazole as an endoscopic outpatient with gastri-tis. Since then, the patient had mood disorder, panic attacks, andsleep disorders. In the last few months, she had aggressive, phobicbehaviors and escaped from school. The patient had also motionimpairment in the right leg about 10 months ago. In the precedingtwo months, she developed urinary incontinence. On examination,her weight and height were 44 kg and 156 cm, respectively, withbody mass index (BMI) 18.1. There was ecchymosis and swellingaround both eyes, especially on the left (Raccoon eyes) (Fig. 1).Painless ocular movements, visual acuity, ophthalmoscopic andfundoscopic examinations were normal. There was right footinversion, reduced right leg deep tendon reflex and reduced painand heat sensation. The Babinski sign of the right foot was positive.Her greater sister was a known case of SLE.Complete blood count (CBC) showed leukopenia and mild lym-phopenia. Other blood parameters or indices (e.g. serum elec-trolytes, ammonia and lactate, coagulation tests, kidney and liverfunction tests, and urine analysis) were normal. Erythrocyte sedi-mentation rate (ESR), C-reactive protein (CRP), serumimmunoglobulins (Igs) and complement tests were normal.Rheumatoid factor (RF), anti-nuclear antibody (ANA), anti -double stranded DNA (anti-dsDNA) and anti-phospholipid anti-bodies were negative. To rule-out neuroblastoma, Vanillin-mandelic acid (VMA) was measured in 24-hour urine and was neg-ative. Chest X-ray and echocardiography were normal. Orbitalcomputerized tomography (CT), total spinal magnetic resonanceimaging (MRI), brain MRI were normal. Electromyography (EMG)and nerve conduction velocity (NCV) tests showed right footperipheral neuropathy. Serological tests were negative for humanimmunodeficiency viruses (HIV), herpes simplex virus (HSV),Epstein–Barr virus (EBV), Cytomegalovirus (CMV), brucella andmycoplasma. Based on the historical, clinical, and laboratorial find-ings, there was a probable diagnosis of SLE in view of the neurolog-ical and hematological disorders and positive family history. Shewas recommended to be followed, and oral gabapentin andamitriptyline were prescribed for symptomatic treatment.2.1. Second hospitalizationAfter eight months, the patient was re-admitted with chiefcomplaints of anorexia, abdominal pain, weight loss, mood disor-ders and hallucinations, as well as increased ecchymosis and swel-ling around the eyes. On physical examination her weight andheight were 35 kg and 157 cm respectively, (BMI 14.2). The patientwas pale with hepatosplenomegaly. The rest of clinical examina-Fig. 1. Ocular involvement of the patient before (tions did not change and remained constant compared to the pre-ceding admission. The CBC showed normocytic, normochromicanemia, leukopenia, lymphopenia and thrombocytopenia (pancy-topenia). The patient had hypokalemia and hyponatremia. CRP,ESR, coagulation tests, serum ammonia and lactate, liver and renalfunctional tests, and urine analysis were normal. Complementlevels (CH50, C3, and C4) were clearly decreased, and ANA andds-DNA were positive. Chest x-ray showed bilateral pleural effu-sion and echocardiography revealed mild pericardial effusion.Her abdominal-pelvic CT revealed mild hepatosplenomegaly. Bonemarrow aspiration showed mild hypocellularity in all blood pat-terns without any evidence of malignancy. Based on the AmericanCollege of Rheumatology and Systemic Lupus International Collab-orating Clinics Classification Criteria for SLE [7], the diagnosis ofneuropsychiatric SLE (NP-SLE) was held: neurological and hemato-logical manifestations, serositis, ANA and anti-dsDNA positive. Shewas treated by pulse methyl prednisolone (30 mg/kg at a max of1 g/dose) and intravenous cyclophosphamide (CYC) (500 mg/m2)monthly for 6 months, then every 3 month, oral prednisoloneand hydroxychloroquine (HCQ).2.2. Outcomes and follow-upAfter treatment with first pulse methylprednisolone and CYC,her appetite and energy improved and her hallucination andaggressive behaviors decreased. Finally, the swelling and ecchymo-sis around her eyes were beginning to decrease. Treatment withoral prednisolone (2 mg/kg/day; max 60 mg/day) and HCQ(5 mg/kg/day) was continued and the patient received pulse CYCfor 6 months. The oral prednisolone was tapered after 2 monthsof treatment with decreasing 5 mg every two weeks until 20 mgdaily was reached at the 6th month of treatment. HCQ (5 mg/kg/-day) continued during this period.Subsequently, CYC pulse was infused (500 mg/m2) every monthfor 6 months, then every 3 months along with the continuation oforal medications. One year after the second admission and abovementioned therapies, her appetite reached back to normal; herweight and height increased to 47 kg and 160 cm, respectively.Also all of her mood disorder symptoms, panic and phobic attacks,and hallucinations were completely remedied. The antidepressantand antipsychotic drugs were discontinued and the patient had noproblems at school. Swelling and ecchymosis around the eyes wereeliminated (Fig. 1). Oral prednisolone 10 mg daily and HCQ (5 mg/kg/day) were continued.3. DiscussionSystemic lupus erythematosus (SLE) is a heterogeneous autoim-mune disease characterized by excessive inflammatory andimmune responses and tissue damage and involves various organsand systems [8]. Measuring organ damage in SLE is important withspecial concern to juvenile-onset patients to allow for designingleft: Racoon eye) and after (right) treatment.M. Jari et al. / The Egyptian Rheumatologist 42 (2020) 241–243 243new treatments that improve control of disease activity and mini-mize the development of irreversible damage [9]. Since about 20%of cases are diagnosed before age 16, SLE in children under 5 isreally rare. There are multiple risk factors involved in its develop-ment and sex is the first and the strongest disease determinantwith about 90% being females [1–4]. Estrogen plays an importantrole in disease exacerbation stimulating B cells to produce autoan-tibodies. C1q, C2, and C4 deficiencies are associated with diseasedevelopment. A family history of other autoimmune diseases anda genetic background are also effective factors [10,11]. Further-more, environmental factors (e.g. sunlight) and viruses (e.g. EBV)raise the risk of SLE development [10].Clinical symptoms of the juvenile patients are different from theadults [9]. The most common primary clinical symptoms in chil-dren with SLE include fever, fatigue, loss of appetite, musculoskele-tal pain, hair loss, and painless oral ulcers [12]. LN is very commonin childhood-onset SLE and begins early in the disease course [13].Neuropsychiatric manifestations of SLE may be associated with thedisease activity or could be misdiagnosed and treated as a mooddisorders, anxiety or psychosis. Musculoskeletal pain may also bemisdiagnosed in children as nonspecific growing pain for a longtime [12].Ocular involvement of SLE is rare and could be orbital, peri-orbital edema, keratoconjunctivitis sicca, episcleritis and retinopa-thy [14]. Eye affection in SLE is very rare at the onset of the diseaseand it is thus important to reveal [4,14]. Other ocular features inSLE include involvement of the anterior and posterior segments,proptosis, orbital pain, blurred vision, chemosis, enophthalmos,and movement restrictions. Vasculitis is a potential key player inSLE [15] and associated myositis may contribute to the eye affec-tion and may be initially misdiagnosed as bacterial cellulitis [4].Peri-orbital edema is seldom present at disease onset and mayoccur in 4.8% throughout the course of the disease which may bedue to the increased vascular permeability, renal involvement, sec-ondary angioedema, and mucin substitution in the skin [4,16]. Rac-coon eyes in our patient may be due to increased vascularpermeability. Ocular involvement in SLE should be treatedpromptly with systemic corticosteroids [4,14].Neuropsychiatric lupus is rare in children at the onset of thedisease and is an indication of severity [17]. In spite of its contro-versy in children’s NP-SLE, pulse CYC still seems to be an alterna-tive of choice [17,18]. The diagnosis of SLE in a patient withinitial ocular involvement is a clinical challenge [4]. Raccoon eyesmay be caused by trauma to the surrounding eye or soft tissuearound it, fracture of the skull, malignancies, amyloidosis, rhino-plasty or severe and prolonged intravenous pressure followinglong-term coughing or sneezing [19,20].Diagnosis of SLE with initial NP manifestations is a hindranceissue for a physician with an old hand at clinical problems and isdelayed in many cases [17]. However, it is necessary to rapidlydiagnose and treat SLE with rare symptoms such as ocular andNP to control the disease promptly and prevent further damageand progress [4,17].In conclusion, ocular involvement in the form of Racoon eyescould be the initial presentation in juvenile SLE. Neuropsychiatricmanifestations could be associated with the eye affection at thedisease onset. Prompt diagnosis and management of the diseaseis critical for these patients. Prednisolone and cyclophosphamideare effective treatment options.Declaration of Competing InterestThe authors declare that they have no known competing finan-cial interests or personal relationships that could have appeared toinfluence the work reported in this paper.References[1] Danchenko N, Satia JA, Anthony MS. Epidemiology of systemic lupuserythematosus: a comparison of worldwide disease burden. Lupus2006;15:308–18.[2] Schwartzman-Morris J, Putterman C. Gender differences in the pathogenesisand outcome of lupus and of lupus nephritis. Clin Dev Immunol2012;2012:604892.[3] Gomez-Puerta JA, Levy S, Khamashta MA, Hughes GR. Periorbital oedema insystemic lupus erythematosus. Lupus 2003;11:866–9.[4] Palejwala NV, Walia H, Yeh S. Ocular manifestations of systemic lupuserythematosus: a review of the literature. Autoimmune Dis2012;2012:290898.[5] Yu HH, Lee JH, Wang LC, Yang YH, Chiang BL. Neuropsychiatric manifestationsin pediatric systemic lupus erythematosus: a 20-year study. Lupus2006;15:651–7.[6] Azkalany GS, Gheita TA, GaberW, Mohey A. Clinical significance of serum TNFaand -308 G/A promoter polymorphism and serum Il-6 and -174 G/C promoterpolymorphism in systemic lupus erythematosus patients. Egypt Rheumatol2012;34(3):119–25.[7] Petri M, Orbai AM, Alarcón GS, Gordon C, Merrill JT, Fortin PR, et al. Derivationand validation of the Systemic Lupus International Collaborating Clinicsclassification criteria for systemic lupus erythematosus. Arthritis Rheum2012;64(8):2677–86.[8] Yang F, He Y, Zhai Z, Sun E. Programmed cell death pathways in thepathogenesis of systemic lupus erythematosus. J Immunol Res2019;2019:3638562.[9] Gheita TA, Fawzy SM, Nour El-din AM, El-Fishawy HS. Juvenile and adult onsetsystemic lupus erythematosus outcome in Egyptian patients. Egypt Rheumatol2011;33(2):99–105.[10] Graham RR, Ortmann W, Rodine P, Espe K, Langefeld C, Lange E, et al. Specificcombinations of HLA-DR2 and DR3 class II haplotypes contribute graded riskfor disease susceptibility and autoantibodies in human SLE. Eur J Hum Genet2007;15:823–30.[11] Taha S, Gamal SM, Nabil M, Naeem N, Labib D, Siam I, et al. Vascularendothelial growth factor G1612A (rs10434) gene polymorphism andneuropsychiatric manifestations in systemic lupus erythematosus patients.Rev Bras Reumatol Engl Ed 2017;57(2):149–53.[12] Brunner HI, Gladman DD, Ibanez D, Urowitz MD, Silverman ED. Difference indisease features between childhood-onset and adult-onset systemic lupuserythematosus. Arthritis Rheum 2008;58:556–62.[13] Gheita TA, Abd El Baky AM, Assal HS, Farid TM, Rasheed IA, Thabet EH. Serumcystatin C, urinary neutrophil gelatinase-associated lipocalin and N-acetyl-beta-D-glucosaminidase in juvenile and adult patients with systemic lupuserythematosus: correlation with clinical manifestations, disease activity anddamage. Saudi J Kidney Dis Transpl 2015;26(3):497–506.[14] Brunner HI, Silverman ED, To T, Bombardier C, Feldman BM. Risk factors fordamage in childhood-onset systemic lupus erythematosus: cumulativedisease activity and medication use predict disease damage. Arthritis Rheum2002;46:436–44.[15] Gheita TA, Abaza NM, Sayed S, El-Azkalany GS, Fishawy HS, Eissa AH.Cutaneous vasculitis in systemic lupus erythematosus patients: potentialkey players and implications. Lupus 2017. epub ahead of print.[16] Hiraki LT, Benseler SM, Tyrrell PN, Hebert D, Harvey E, Silverman ED. Clinicaland laboratory characteristics and long-term outcomes of pediatric systemiclupus erythematosus: a longitudinal study. J Pediatr 2008;152:550–6.[17] Ferraria N, Rocha S, Fernandes VS, Correia T, Gonçalves E. Juvenile systemiclupus erythematosus with primary neuropsychiatric presentation. BMJ CaseRep 2013. pii: bcr2012008270.[18] Barile-Fabris L, Ariza-Andraca R, Olguin-Ortega L, Jara LJ, Fraga-Mouret A,Miranda-Limón JM, et al. 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A 13-year-old systemic lupus erythematosus girl initially presenting with Raccoon eyes and neuropsychiatric manifestations

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A 13-year-old systemic lupus erythematosus girl initially presenting with Raccoon eyes and neuropsychiatric manifestations

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