Embed Size (px)
Citation preview
Case ReportSystemic Lupus Erythematosus Presented with Bilateral OrbitalEdema and Negative Serology
Manal Al-Khaldi 1 and Manal Alsabbagh2
1Registrar, Internal Medicine Department, King Hamad University Hospital, AL-Muharraq, Bahrain2Registrar, Internal Medicine Department, Dermatology, King Hamad University Hospital, AL-Muharraq, Bahrain
Correspondence should be addressed to Manal Al-Khaldi; [email protected]
Received 7 April 2019; Revised 26 June 2019; Accepted 21 July 2019; Published 23 September 2019
Academic Editor: Gregory J. Tsay
Copyright © 2019Manal Al-Khaldi andManal Alsabbagh.*is is an open access article distributed under the Creative CommonsAttribution License, which permits unrestricted use, distribution, and reproduction in anymedium, provided the original work isproperly cited.
Isolated bilateral periorbital edema with negative serology is an extremely rare presentation of cutaneous lupus erythematosus thatmay lead to eyelid scarring, infection, or even corneal involvement. *e treatment usually comprises a combination ofhydroxychloroquine and a tapering dose of systemic steroids. Patients require long-term follow-up as they may develop systemiclupus erythematosus with positive serology later in life. We report a case of a 32-year-old female who presented with chronicbilateral periorbital edema, and the histopathology confirmed cutaneous lupus erythematosus.
1. Introduction
Lupus erythematous is a chronic inflammatory autoimmunedisease which encompasses a wide range of clinical mani-festations affecting multiple organ systems. *ere are fourmain types of lupus: neonatal lupus, discoid lupus, drug-induced lupus, and systemic lupus erythematosus [1]. *emodified Gilliam grouping system is a modified classifica-tion for cutaneous lupus erythematosus which comprisesacute cutaneous lupus erythematosus (ACLE), subacutecutaneous lupus erythematosus (SCLE), and chronic cuta-neous lupus erythematosus (CLE) [2].
Clinically, each type of cutaneous lupus presents withsome specific features. For instance, discoid lupus presentswith thick, firm, erythematous, scaly, and well-demarcatedplaques that have the potential to heal with scarring, alo-pecia, and hypopigmentation [3].
*e presentation of eyelid edema is extremely rare; thereported incidence is 0.1% as for the presenting manifes-tation of systemic lupus erythematous with an overall in-cidence of 4.8% [4]. Diagnosis for these cases is essential toprevent scarring, and long-term follow-up is needed as 5% ofthe population has the risk of developing systemic lupuserythematosus.
In general, the key characteristic that unites cutaneouslupus erythematosus is the histopathology presentationsof the following features: hyperkeratosis, epidermal at-rophy, vacuolar interface dermatitis, perivascular, andperifollicular mononuclear cell inflammatory infiltrate,with or without the presence of civatte bodies and fol-licular plugging [5].
2. Case Presentation
A 32-year-old African female presented to the emergencydepartment complaining of persisted periorbital swelling,joint pain, and fever. *e periorbital swelling was initiallynoticed 3months before her presentation with mild pruritusand no visual disturbance. In addition, she was having in-termittent joint pain involving the wrists, fingers, andshoulders. *e fever was subjective and not documentedwith any history of sweating.
On presentation, she also complained of a two-weekhistory of sore throat and difficulty in breathing. Para-cetamol was the only medicine the patient was taking tomanage her symptoms. She denied using any new medi-cation or product and denied any previous history ofallergies.
HindawiCase Reports in RheumatologyVolume 2019, Article ID 7140534, 5 pageshttps://doi.org/10.1155/2019/7140534
On her arrival, her vitals were normal and stable. Clinicalexamination revealed bilateral erythematous periorbitaledema with violaceous hue, right cervical lymphadenopathy,and mild friction rub on the lower zone of the right lung. Shehad tenderness of the wrists, proximal metacarpal joints, andshoulders; movement restriction due to pain was noticed.Cardiovascular, breast, and abdominal examinations wereuneventful.
Electrocardiography showed a sinus rhythm with aregular rate and no abnormalities. A chest X-ray showedpleural thickening at the right lung base.
*e patient was admitted to the internal medicine de-partment for further investigations. Abdominal ultrasoundwas normal with no abdominal lymphadenopathy. CT chestwith contrast showed right lateral pleurodiaphragmaticadhesions and fibrotic bands.
Laboratory investigations revealed mild lymphopenia,an elevated serum ferritin level, and a mildly elevatederythrocyte sedimentation rate (Tables 1 and 2).
*e echocardiogram showed a normal left ventricularcavity size with a preserved ventricular ejection fraction of 55%,and the systolic pulmonary artery pressure was 21mmHg.
For this case, it was of interest to draw a list of differentialdiagnosis that includes sarcoidosis, Still’s disease, systemiclupus erythematosus, rheumatoid arthritis, amyloidosis,chronic blepharitis, eczema, contact dermatitis, psoriasis,and lymphoma. Still’s disease was brought to attention dueto an elevated ferritin level; however, there was absence ofthe classic triad of persistent high-grade fever, joint pain, anda distinctive salmon-colored skin lesion.
*erefore, it remained unclear whether it was systemiclupus erythematosus or amyloidosis until skin biopsy fromthe eyelid was attempted, and the histopathology resultsshowed degeneration with follicular plugging and dermalinflammatory cell infiltrate. *ese features were consistentwith lupus erythematosus with skin involvement (Figure 1).
*e patient was started on prednisolone 40mg orallyonce per day with noticeable improvement in periorbitaledema and joint tenderness; however, unfortunately, thepatient had presented once in the internal medicine clinicafter her discharge, while the skin biopsy results were stillpending and did not attend further appointments.
3. Discussion
Chronic bilateral periorbital edema that poorly responds totopical treatment should raise the suspicion of rare
Table 1: Laboratory investigations.WBC 4.2×109/L ASO quantitative NegativeLymphocytes 0.88×109/L Creatine kinase 110.6 u/lNeutrophil absolute 2.89×109/L Ferritin 1330 ng/mlMonocyte absolute 0.28×109/L Serum iron 4.46 μmol/LEosinophil absolute 0.15×109/L TIBC 34 μmol/LBasophil absolute 0.03×109/L Transferrin saturation 12.97%
HGB 12.5 g/dl TFTTSH 2.387 iu/mlT4 1.09 ng/dlT3 2.86 pn/ml
PLT 162×109/LSerum creatinine 82mmol/L ESR 48mm/hrUrea 7mmol/L CRP 6.5mg/LNa 141mmol/LK 4.6mmol/L 24 h urine creatinine and volume 1300ml 9.3mmol/24 hrs.Serum albumin 32.5 g/l 24 h protein 0.21 gm/24 hrs.Total bilirubin 5.57 μmol/lALT 43 u/l Urinalysis Glucose 100mg/dl, protein 30mg/dlAST 59 u/lGGT 88.2 u/l *roat swab for beta hemolytic streptococci NegativeWBC, white blood cell count; HBG, serum hemoglobin; PLT, platelets; Na, serum sodium; K, serum potassium; ALT, alanine transaminase; AST, aspartateaminotransferase; GGT, gamma-glutamyl transferase; ASO, antistreptolysin O; TIBC, total iron-binding capacity; TSH, thyroid-stimulating hormone; TFT,thyroid function test; ESR, erythrocyte sedimentation rate; CRP, C-reactive protein.
Table 2: Immunochemistry.ANA CTD screen Negative Anti-RNP70 NegativeAnti-CCP IgG Negative Anti-Jo1 NegativeAntirheumatoidfactor IgA Negative Anti-Scl-70 Negative
Antirheumatoidfactor IgM 1.1 Antismith Negative
Rheumatoid factorquantitative
9.12 iu/ml (<20)
Anti-SS-A/RO,Anti-SSB/LA Negative
Anticardiolipin IgG,IgM, and IgA Negative Anti-U1RNP Negative
Lupus anticoagulant Negative C3 1.5 (normalrange?)
Anticentromereprotein B Negative C4
0.364(normalrange?)
Anti-ds DNA Negative Vitamin D 11.5 ng/ml
ACE Normalrange
ANA: antineutrophil antibody; CTD screen: connective tissue diseasescreen; anti-ds DNA: anti-double-stranded DNA; ACE: angiotensin-con-verting enzyme; anti-RNP70 antibody: anti-ribonucleoprotein antibody;anti-Jo1 antibody: myositis-specific autoantibodies directed against thehistidyl-tRNA synthetase; anti-Scl-70: anti-topoisomerase antibody; anti-SSA/Ro autoantibodies: anti-Sjogren’s-syndrome-related antigen A; anti-U1RNP: anti-ribonucleoprotein.
2 Case Reports in Rheumatology
Figure 1: Skin with focal basal cell damage and vacuolar degeneration. *ere is follicular plugging with a mild dermal inflammatory cellinfiltrate. *ere is no evidence of malignancy. *e features are consistent with cutaneous lupus.
Table 3: A summary of similar presentations that found in different case reports.
Case Date Clinical presentation Laboratory finding Histopathology Treatment
A case of discoid lupuserythematosus of theeyelid [6]
2006
A 39-year-old manpresented with erosiveerythema of the left
lower eyelid
Antinuclear antibody,anti-double-stranded
(ds) DNA,anti-Sm antibody,anti-SS- A, and
anti-SS-B were allnegative
A biopsy from the eyelidshowed liquefaction anddegeneration of the basallayer of the epidermisand the appendage
epithelium. *e findingis consistent with DLE.*e patient response to
prednisolone
Prednisolone 10mg/day,antiallergic drug
(cetirizinehydrochloride), and
betamethasone sodiumphosphate eye drop for
2 years.*e skin lesion resolved
over 8months
A case report of lupuserythematosus tumidusconverted from discoidlupus erythematosus [7]
2018
A 62-year-old Chineseman presented with aone-year history of
recurrent erythematousfacial plaques and
bilateral swelling of theeyelid
Antinuclear antibodieswere positive. Anti-
double-stranded DNAantibodies, anti-RO/SS-A, and anti-La/SS-Bantibodies were all
negative
Histopathologydemonstratedliquefaction
degeneration of basalcells and perivascular
and periadnexalinfiltration lymphocytes
Prednisolone 1mg/kg/day combined withhydroxychloroquine200mg twice per dayand topical tacrolimus.
*e patient hadcomplete recovery over4months with no relapse
over 6months
Cheek and periorbitalpeculiar discoid lupuserythematosus: a rareclinical presentationmimicking tinea facieicutaneousgranulomatous diseaseor blepharitis [8]
2015
A 39-year-old Japaneseman was found to haveerythema on his right
eyelid and right cheek in2010
Anti-double-strandedDNA antigen, anti- SSAantigen, and anti-SBBantigen were all negative
Histopathology showedparakeratosis and
hyperkeratosis of thehorny layer and
hydropic degenerationand vacuolar changes in
the basal layer; thefindings were consistent
with DLE
Tacrolimus ointmentwas used with a good
response
Discoid lupuserythematosusmasquerading as chronicblepharoconjunctivitis[9]
2005
A 33-year-old Caucasianman complaining of his
lower eyelid for8months was diagnosed
with discoid lupus
Antinuclear antibodywas negative
Biopsy showedhyperkeratosis of theepithelium, thickenedbasement membrane,basal cell vacuolation,
and dermalinflammation
6 weeks ofhydroxychloroquine
improved with 6monthsfollow-up
A 58-year-old Caucasianwoman presented with a
complain of eyelidredness and thickeningon the right side looks
greater
Antinuclear antibodywas negative
Biopsy results showedbenign chronic
inflammation in thedermis, hyperkeratosisof the epithelium,thickened basementmembrane, basal cellvacuolation, and
telangiectasias, whichwas consistent with
discoid lupus
Treatment withhydroxychloroquine
Case Reports in Rheumatology 3
Table 3: Continued.
Case Date Clinical presentation Laboratory finding Histopathology Treatment
A 54-year-old Caucasianwoman presented with25 years of chronicinflammation and
scarring of the eyelid andphiltrum
Antinuclear antibodieswere negative
Biopsy from the rightlower eyelid showed
epithelial atrophy, focaldyskeratosis, and athickened basement
membrane with an areaof focal destruction
Treated withhydroxychloroquine
200mg twice a day andshowed marked
improvement over2months
A 41-year-old Caucasianwoman presented with2-year history of eyelidredness and conjunctival
infection
Antinuclear antibodieswere negative
Biopsy showed granulardeposition of IgM, IgG,IgA, and C3 along withthe dermo-epidermaljunction which wasconsistent with DLE
Hydroxychloroquine200mg was bid orally
and showedimprovement over2weeks of treatment
Discoid lupuserythematosus of theperiorbital edema:clinical dilemmas anddiagnostic delays [10]
2012
A 50-year-old Caucasianwoman presented with acomplain of painless andslowly progressive rightperiorbital swelling
*e article did notmention the serology
study
Biopsy showedperivascular
lymphocytic infiltratethroughout the dermisand the basal epidermal
layer, with floridlichenoid changes andvacuolar degenerationwhich were consistent
with DLE
Oral corticosteroid wasstarted, with
improvement noticedover 12months
A 48-year-old Afro-Caribbean female
complains of blepharitiswith a demarcated areaof depigmentation ofboth lower lid margins
Biopsy showed chronicinflammation which wasconsistent with DLE
*e patient was treatedwith
hydroxychloroquinewith good response
A 23-year-old Afro-Caribbean male
complains of persistentright lower lid swelling
Biopsy showed featuresthat confirmed DLE
*e patient was startedon hydroxychloroquine,
but due to poorcompliance there was no
response
Eyelid discoid lupuserythematosus andcontact dermatitis: a casereport [11]
2004
A 71-year-old whitefemale complains ofwatery eyelids with
itching and redness for10 years
Antinuclear antibodies,anti-DNA, anti-Sm,
anti-RNP,anticardiolipin, SS-A
(Ro), and SS-B (La) wereall negative
Biopsy showed basal cellvacuolar alteration.
Showed IgA, IgG, IgM,and C3 intense granulardeposits in a band-like
pattern along thedermalepidermal
junction
Treatment withchloroquine 250mg/dayfor 3months. But thiswas stopped due to sideeffects and treatment
was changed tocorticosteroid
Periorbital edema anderythema: an unusuallocalization of DLE in apatient with psoriasis[12]
2010
A 33-year-old womanpresented with complainof malar erythema andleft eye periorbitalswelling for 2 years
Antinuclear antibodiesand anti-DNA
antibodies were negative
Biopsy showed vacuolardegeneration at the basallayer of the epidermis
with mildhyperkeratosis.Perivascular
lymphocytic infiltrationand scattered
melanophages on theupper dermis
Hydroxychloroquine400mg od po and topical
corticosteroid
Severe chronicblepharitis and scarringectropion associatedwith discoid lupuserythematosus [13]
2013
A 45-year-old Caucasianwoman presented withcomplain of eyelid
redness and irritation for21 years
Antinuclear antibodieswere negative
Biopsy showedhyperkeratosis of theepithelium and a thickbasement membraneand a sign of chronic
inflammation which wasconsistent with DLE
*e patient was startedon hydroxychloroquine200mg bid po, withimprovement noticed
over 2months
4 Case Reports in Rheumatology
disorders, with possibility of neoplasm or connective tissuedisease. Wu et al. reviewed 25 patients presented primarilywith periorbital edema with a diagnosis of cutaneous lupusin addition to reviewing 10 cases from the literature. *emajority of the patients were females (68%), and only four ofthe 25 patients had bilateral involvement. Seventy-twopercent had their upper eyelid involved. Majority of thepatients were misdiagnosed or suspected to have contactdermatitis, dermatomyositis, lymphoproliferative neoplasm,facial cellulitis, and severe angioedema. Our patient was afemale with bilateral eyelid erythematous edema. *e initialimpression was of an allergic reaction and angioedema.Nineteen of the 25 patients as well as our patient had anegative ANA rate in that study (Table 3).
*ere is a broad implication of shared histological fea-tures in lupus erythematosus; 80% of patients had basal cellvacuolar degeneration, 44% had lymphocytic infiltration,and 40% had follicular plugging. *ose findings wereconsistent with our case.
Although our case showed features such as arthritis,pleuritic thickening, and initial lymphopenia, the serologicprofile came negative. Wu et al. reported 6 patients, whoinitially presented with periorbital edema and developedsystemic involvement, meeting the American College ofRheumatology criteria for diagnosing systemic lupus. Fur-thermore, Wu et al. found that 10 cases with lupus eryth-ematosus were reported in the literature with primarypresentation of periorbital erythematous edema. Of those, 9were females, and 6 had a positive ANA. Similarly, caseswere misdiagnosed as lymphoproliferative disease, ble-pharitis, and chalazion.
4. Conclusion
Isolated bilateral orbital edema is an extremely rare pre-sentation and found possible to be presented before otherskin lesions, the challenges with negative serology impact ondiagnosis for cutaneous lupus erythematous, and therefore,these cases are treated as chronic eczema or angioedema. It isessential for patients presenting with eyelid lesions and whofail the treatment for eczema or infections to have a fullcareful skin examination and skin biopsy for diagnosis.
Conflicts of Interest
*e authors declare that they have no conflicts of interest.
References
[1] W. Maidhof and O. Hilas, “Lupus: an overview of the diseaseand management options,” Physical %erapy, vol. 37, no. 4,pp. 240–249, 2012.
[2] J. F. Merola and S. L. Moschella, UpToDate, T. W. Post, Ed.,UpToDate, Waltham, MA, USA, 2019.
[3] L. A. Lee and V. P. Werth, “Lupus erythematosus,” in Der-matology, J. L. Bolognia, J. V. Schaffer, and L. Cerroni, Eds.,vol. 1, 4th edition, Elsevier, Amsterdam, Netherlands, 2018.
[4] M. I. Costner and R. D. Sontheimer, “Lupus erythematosus,”in Fitzpatrick’s Dermatology in General Medicine,
L. A. Goldsmith, S. I. Katz, B. A. Gilchrest et al., Eds., vol. 2,8th edition, McGraw-Hill, New York, NY, USA, 2012.
[5] S. Erras, L. Benjilali, and L. Essaadouni, “Periorbital edema asinitial manifestation of chronic cutaneous lupus eryth-ematosus,” Pan African Medical Journal, vol. 12, p. 57, 2012.
[6] K.Monji, K. Yumiko, K. Hiromaro, and N. Juichiro, “A case ofdiscoid lupus erythematosus of the eyelid,” %e Journal ofDermatology, vol. 33, no. 5, pp. 368–371, 2006.
[7] C. Xiaomei, S. Wang, and L. Li, “A case report of lupuserythematosus tumidus converted from discoid lupuserythematosus,” Medicine, vol. 97, no. 16, Article no e0375,2018.
[8] N. Satoshi, T. Yamada, and N. Umemoto, “Cheek and peri-orbital peculiar discoid lupus erythematosus: rare clinicalpresentation mimicking tinea faciei, cutaneous gransuloma-tous disease or blepharitis,” Case Reports in Deramtology,vol. 7, no. 1, pp. 56–60, 2015.
[9] N. Acharya, R. Pinedaii, H. Uy, and C. Foster, “Discoid lupuserythematosusmasquerading as chronic blepharoconjuctivitis,”Ophthalmology, vol. 112, no. 5, pp. e19–e23, 2005.
[10] T. Gupta, M. Beaconsfield, G. E. Rose, and D. H. Verity,“Discoid lupus erythematosus of the periorbita:clinical di-lemmas diagnostic delays,” Eye, vol. 26, no. 4, pp. 609–612,2012.
[11] M. A. B. Trindade, A. O. A Alchorne, E. B. da Costa, andM. M. S. S. Enokihara, “Eyelid discoid lupus erythematosusand contact dermatitis: a case report,” Journal of the EuropeanAcademy of Dermatology and Venereology, vol. 18, no. 5,pp. 577–579, 2004.
[12] G. Serarslan, E. Atik, and G. Sarikaya, “Periorbital edema anderythema: an unusual localization of DLE in a patient withpsoriasis,” %e Journal of Dermatology, vol. 38, no. 5,pp. 486–488, 2011.
[13] N. Kopaschilis, K. T. Tsaousis, T. Tourtas, andI. T. Tsinopoulos, “Severe chronic blepharitis and scarringectropion associated with discoid lupus erythematosus,”Clinical and Experimental Optomerty, vol. 96, no. 1,pp. 124-125, 2013.
Case Reports in Rheumatology 5
Stem Cells International
Hindawiwww.hindawi.com Volume 2018
Hindawiwww.hindawi.com Volume 2018
MEDIATORSINFLAMMATION
of
EndocrinologyInternational Journal of
Hindawiwww.hindawi.com Volume 2018
Hindawiwww.hindawi.com Volume 2018
Disease Markers
Hindawiwww.hindawi.com Volume 2018
BioMed Research International
OncologyJournal of
Hindawiwww.hindawi.com Volume 2013
Hindawiwww.hindawi.com Volume 2018
Oxidative Medicine and Cellular Longevity
Hindawiwww.hindawi.com Volume 2018
PPAR Research
Hindawi Publishing Corporation http://www.hindawi.com Volume 2013Hindawiwww.hindawi.com
The Scientific World Journal
Volume 2018
Immunology ResearchHindawiwww.hindawi.com Volume 2018
Journal of
ObesityJournal of
Hindawiwww.hindawi.com Volume 2018
Hindawiwww.hindawi.com Volume 2018
Computational and Mathematical Methods in Medicine
Hindawiwww.hindawi.com Volume 2018
Behavioural Neurology
OphthalmologyJournal of
Hindawiwww.hindawi.com Volume 2018
Diabetes ResearchJournal of
Hindawiwww.hindawi.com Volume 2018
Hindawiwww.hindawi.com Volume 2018
Research and TreatmentAIDS
Hindawiwww.hindawi.com Volume 2018
Gastroenterology Research and Practice
Hindawiwww.hindawi.com Volume 2018
Parkinson’s Disease
Evidence-Based Complementary andAlternative Medicine
Volume 2018Hindawiwww.hindawi.com
Submit your manuscripts atwww.hindawi.com
