Generic placeholder image

Current Pediatric Reviews

Editor-in-Chief

ISSN (Print): 1573-3963
ISSN (Online): 1875-6336

Review Article

Cutaneous Lupus Erythematosus in Children

Author(s): Vivian Tsang*, Alexander K.C. Leung and Joseph M. Lam

Volume 17, Issue 2, 2021

Published on: 24 February, 2021

Page: [103 - 110] Pages: 8

DOI: 10.2174/1573396317666210224144416

Price: $65

Abstract

Background: The skin is commonly involved in autoimmune diseases, such as lupus erythematous. The cutaneous lupus erythematosus (CLE) can manifest with or without systemic symptoms. It is advantageous from a patient and healthcare system standpoint for early diagnosis and intervention. Prevention of complications is especially important in the pediatric population.

Objective: To familiarize physicians with the clinical presentation, diagnosis, evaluation, and management of pediatric cutaneous lupus.

Methods: The search term “cutaneous lupus” was entered into a Pubmed search. A narrow scope was applied to the categories of “epidemiology”, “clinical diagnosis”, “investigations”, “comorbidities”, and “treatment”. Meta-analyses, randomized controlled trials, clinical trials, observational studies, and reviews were included. The search was restricted to English literature and children. A descriptive, narrative synthesis of the retrieved articles was provided.

Results: A variety of innate and adaptive immune responses are being investigated to explain the pathogenesis of CLE. There are a number of variations of cutaneous manifestations varying from localized malar rash as in the case of ACLE lesions and papulosquamous psoriasiform lesions as in the case of SCLE to the multiple subtypes within chronic CLE. First-line pharmacological treatments include topicals, such as typical calcineurin inhibitors and corticosteroids, or oral agents, such as glucocorticoids, antimalarial drugs, and hydroxychloroquine.

Conclusion: CLE is inclusive of a number of subtypes that have varying dermatological manifestations in adult and pediatric populations. The current treatment modalities will change based on the newly understood molecular targets. Ongoing research on the mechanisms underlying CLE is necessary to derive new interventions for pediatric patients.

Keywords: Lupus erythematosus, autoimmune disease, hydroxychloroquine, photoprotection, CLE, SLE.

Next »
Graphical Abstract

[1]
Kuhn A, Ruland V, Bonsmann G. Cutaneous lupus erythematosus: update of therapeutic options part II. J Am Acad Dermatol 2011; 65(6): e195-213.
[http://dx.doi.org/10.1016/j.jaad.2010.06.017] [PMID: 20800319]
[2]
Peterson LB, Bell CJM, Howlett SK, et al. A long-lived IL-2 mutein that selectively activates and expands regulatory T cells as a therapy for autoimmune disease. J Autoimmun 2018; 95: 1-14.
[http://dx.doi.org/10.1016/j.jaut.2018.10.017] [PMID: 30446251]
[3]
Tebbe B, Orfanos CE. Epidemiology and socioeconomic impact of skin disease in lupus erythematosus. Lupus 1997; 6(2): 96-104.
[http://dx.doi.org/10.1177/096120339700600204] [PMID: 9061657]
[4]
Grönhagen CMFC, Fored CM, Granath F, Nyberg F. Cutaneous lupus erythematosus and the association with systemic lupus erythematosus: a population-based cohort of 1088 patients in Sweden. Br J Dermatol 2011; 164(6): 1335-41.
[http://dx.doi.org/10.1111/j.1365-2133.2011.10272.x] [PMID: 21574972]
[5]
European League Against Rheumatism/American College of Rheumatology Classification Criteria for Systemic Lupus Erythematosus. Ann Rheum Dis 2019; 78(9): 1151-9.
[http://dx.doi.org/10.1136/annrheumdis-2018-214819] [PMID: 31383717]
[6]
Gilliam JN, Sontheimer RD. Distinctive cutaneous subsets in the spectrum of lupus erythematosus. J Am Acad Dermatol 1981; 4(4): 471-5.
[http://dx.doi.org/10.1016/S0190-9622(81)80261-7] [PMID: 7229150]
[7]
Arkin LM, Buhr K, Brandling-Bennett H, et al. Practice-based differences in paediatric discoid lupus erythematosus. Br J Dermatol 2019; 181(4): 805-10.
[http://dx.doi.org/10.1111/bjd.17780] [PMID: 30768778]
[8]
Cojocaru M, Cojocaru IM, Silosi I, Vrabie CD. Manifestations of systemic lupus erythematosus. Maedica (Bucur) 2011; 6(4): 330-6.
[PMID: 22879850]
[9]
Werth VP. Clinical manifestations of cutaneous lupus erythematosus. Autoimmun Rev 2005; 4(5): 296-302.
[http://dx.doi.org/10.1016/j.autrev.2005.01.003] [PMID: 15990077]
[10]
Rothfield N, Sontheimer RD, Bernstein M. Lupus erythematosus: systemic and cutaneous manifestations. Clin Dermatol 2006; 24(5): 348-62.
[http://dx.doi.org/10.1016/j.clindermatol.2006.07.014] [PMID: 16966017]
[11]
Grönhagen CM, Nyberg F. Cutaneous lupus erythematosus: An update. Indian Dermatol Online J 2014; 5(1): 7-13.
[http://dx.doi.org/10.4103/2229-5178.126020] [PMID: 24616847]
[12]
Dickey BZ, Holland KE, Drolet BA, et al. Demographic and clinical characteristics of cutaneous lupus erythematosus at a paediatric dermatology referral centre. Br J Dermatol 2013; 169(2): 428-33.
[http://dx.doi.org/10.1111/bjd.12383] [PMID: 23601021]
[13]
Wenzel J. Cutaneous lupus erythematosus: new insights into pathogenesis and therapeutic strategies. Nat Rev Rheumatol 2019; 15(9): 519-32.
[http://dx.doi.org/10.1038/s41584-019-0272-0] [PMID: 31399711]
[14]
Mak A, Kow N Y. The pathology of T cells in systemic lupus erythematosus. Journal of immunology researc 2014.
[15]
Herrada AA, Escobedo N, Iruretagoyena M, et al. Innate Immune Cells’ Contribution to Systemic Lupus Erythematosus. Front Immunol 2019; 10: 772.
[http://dx.doi.org/10.3389/fimmu.2019.00772] [PMID: 31037070]
[16]
Zhang YP, Wu J, Han YF, Shi ZR, Wang L. Pathogenesis of cutaneous lupus erythema associated with and without systemic lupus erythema. Autoimmun Rev 2017; 16(7): 735-42.
[http://dx.doi.org/10.1016/j.autrev.2017.05.009] [PMID: 28483542]
[17]
Sabrautzki S, Janas E, Lorenz-Depiereux B, et al. An ENU mutagenesis-derived mouse model with a dominant Jak1 mutation resembling phenotypes of systemic autoimmune disease. Am J Pathol 2013; 183(2): 352-68.
[http://dx.doi.org/10.1016/j.ajpath.2013.04.027] [PMID: 23791841]
[18]
Chan ES, Herlitz LC, Ali J. Ruxolitinib Attenuates Cutaneous Lupus Development in a Mouse Lupus Model. J Invest Dermatol 2015; 135(9): 2338-9.
[http://dx.doi.org/10.1038/jid.2015.216] [PMID: 26134782]
[19]
Peschke K, Friebe F, Zimmermann N, et al. Deregulated type I IFN response in TREX1-associated familial chilblain lupus. J Invest Dermatol 2014; 134(5): 1456-9.
[http://dx.doi.org/10.1038/jid.2013.496] [PMID: 24270665]
[20]
Vaglio A, Grayson PC, Fenaroli P, et al. Drug-induced lupus: Traditional and new concepts. Autoimmun Rev 2018; 17(9): 912-8.
[http://dx.doi.org/10.1016/j.autrev.2018.03.016] [PMID: 30005854]
[21]
Chang AY, Werth VP. Treatment of cutaneous lupus. Curr Rheumatol Rep 2011; 13(4): 300-7.
[http://dx.doi.org/10.1007/s11926-011-0180-z] [PMID: 21503694]
[22]
Watanabe T, Tsuchida T. Classification of lupus erythematosus based upon cutaneous manifestations. Dermatological, systemic and laboratory findings in 191 patients. Dermatology 1995; 190(4): 277-83.
[http://dx.doi.org/10.1159/000246716] [PMID: 7655105]
[23]
Ting W, Stone MS, Racila D, Scofield RH, Sontheimer RD. Toxic epidermal necrolysis-like acute cutaneous lupus erythematosus and the spectrum of the acute syndrome of apoptotic pan-epidermolysis (ASAP): a case report, concept review and proposal for new classification of lupus erythematosus vesiculobullous skin lesions. Lupus 2004; 13(12): 941-50.
[http://dx.doi.org/10.1191/0961203304lu2037sa] [PMID: 15645750]
[24]
Kole AK, Ghosh A. Cutaneous manifestations of systemic lupus erythematosus in a tertiary referral center. Indian J Dermatol 2009; 54(2): 132-6.
[http://dx.doi.org/10.4103/0019-5154.53189] [PMID: 20101308]
[25]
AlKharafi NN, Alsaeid K, AlSumait A, et al. Cutaneous Lupus Erythematosus in Children: Experience from a Tertiary Care Pediatric Dermatology Clinic. Pediatr Dermatol 2016; 33(2): 200-8.
[http://dx.doi.org/10.1111/pde.12788] [PMID: 27001318]
[26]
Walling HW, Sontheimer R. D. Cutaneous lupus erythematosus. Am J Clin Dermatol 2009; 10(6): 365-81.
[http://dx.doi.org/10.2165/11310780-000000000-00000] [PMID: 19824738]
[27]
Lee LA, David K. M. Cutaneous lupus erythematosus. Curr Probl Dermatol 1989; 1(6): 165-200.
[http://dx.doi.org/10.1016/S1040-0486(89)80003-1]
[28]
Ribero S, Sciascia S, Borradori L, Lipsker D. The cutaneous spectrum of lupus erythematosus. Clin Rev Allergy Immunol 2017; 53(3): 291-305.
[http://dx.doi.org/10.1007/s12016-017-8627-2] [PMID: 28752372]
[29]
Yu J, Brandling-Bennett H, Co DO, Nocton JJ, Stevens AM, Chiu YE. Toxic epidermal necrolysis-like cutaneous lupus in pediatric patients: a case series and review. Pediatrics 2016; 137(6): e20154497.
[http://dx.doi.org/10.1542/peds.2015-4497] [PMID: 27245834]
[30]
Merklen-Djafri C, Bessis D, Frances C, et al. Blisters and loss of epidermis in patients with lupus erythematosus: a clinicopathological study of 22 patients. Medicine (Baltimore) 2015; 94(46): e2102.
[http://dx.doi.org/10.1097/MD.0000000000002102] [PMID: 26579826]
[31]
Sontheimer RD, Thomas JR, Gilliam JN. Subacute cutaneous lupus erythematosus: a cutaneous marker for a distinct lupus erythematosus subset. Arch Dermatol 1979; 115(12): 1409-15.
[http://dx.doi.org/10.1001/archderm.1979.04010120007006] [PMID: 533284]
[32]
Lipsker DMS-GC, Schreckenberg-Gilliot C, Uring-Lambert B, et al. Lupus erythematosus associated with genetically determined deficiency of the second component of the complement. Arch Dermatol 2000; 136(12): 1508-14.
[http://dx.doi.org/10.1001/archderm.136.12.1508] [PMID: 11115162]
[33]
Callen JPHS, Hodge SJ, Kulick KB, Stelzer G, Buchino JJ. Subacute cutaneous lupus erythematosus in multiple members of a family with C2 deficiency. Arch Dermatol 1987; 123(1): 66-70.
[http://dx.doi.org/10.1001/archderm.1987.01660250072021] [PMID: 3467658]
[34]
Bangert JL, Freeman RG, Sontheimer RD, Gilliam JN. Subacute cutaneous lupus erythematosus and discoid lupus erythematosus. Comparative histopathologic findings. Arch Dermatol 1984; 120(3): 332-7.
[http://dx.doi.org/10.1001/archderm.1984.01650390054011] [PMID: 6703733]
[35]
Sontheimer RD. Subacute cutaneous lupus erythematosus: a decade’s perspective. Med Clin North Am 1989; 73(5): 1073-90.
[http://dx.doi.org/10.1016/S0025-7125(16)30620-4] [PMID: 2671533]
[36]
Hon KLLA, Leung AK. Neonatal lupus erythematosus. Autoimmune Dis 2012; 2012: 301274.
[PMID: 22973504]
[37]
Rivera TLIP, Izmirly PM, Birnbaum BK, et al. Disease progression in mothers of children enrolled in the Research Registry for Neonatal Lupus. Ann Rheum Dis 2009; 68(6): 828-35.
[http://dx.doi.org/10.1136/ard.2008.088054] [PMID: 18625627]
[38]
Levy R, Briggs L, Silverman E, Pope E, Lara-Corrales I. Cutaneous sequelae in neonatal lupus: A retrospective cohort study. J Am Acad Dermatol 2020; 83(2): 440-6.
[http://dx.doi.org/10.1016/j.jaad.2019.09.083] [PMID: 31626881]
[39]
Arkin LMAL, Ansell L, Rademaker A, et al. The natural history of pediatric-onset discoid lupus erythematosus. J Am Acad Dermatol 2015; 72(4): 628-33.
[http://dx.doi.org/10.1016/j.jaad.2014.12.028] [PMID: 25648823]
[40]
Sampaio MC dOZ, Machado MC, et al. Discoid lupus erythematosus in children–a retrospective study of 34 patients. Pediatr Dermatol 2008; 25: 163-7.
[41]
Drucker AM, Su J, Mussani F, Siddha SK, Gladman DD, Urowitz MB. Prognostic implications of active discoid lupus erythematosus and malar rash at the time of diagnosis of systemic lupus erythematosus: Results from a prospective cohort study. Lupus 2016; 25(4): 376-81.
[http://dx.doi.org/10.1177/0961203315610645] [PMID: 26453664]
[42]
Prystowsky SD, Gilliam JN. Discoid lupus erythematosus as part of a larger disease spectrum. Correlation of clinical features with laboratory findings in lupus erythematosus. Arch Dermatol 1975; 111(11): 1448-52.
[http://dx.doi.org/10.1001/archderm.1975.01630230050009] [PMID: 1081865]
[43]
David-Bajar KM, Bennion SD, DeSpain JD, Golitz LE, Lee LA. Clinical, histologic, and immunofluorescent distinctions between subacute cutaneous lupus erythematosus and discoid lupus erythematosus. J Invest Dermatol 1992; 99(3): 251-7.
[http://dx.doi.org/10.1111/1523-1747.ep12616582] [PMID: 1512459]
[44]
Mladick RA, Pickrell KL, Thorne FL, Hall JH. Squamous cell cancer in discoid lupus erythematosus. Case report. Plast Reconstr Surg 1968; 42(5): 497-9.
[http://dx.doi.org/10.1097/00006534-196811000-00022] [PMID: 4880840]
[45]
Sánchez Yáñez P. NCE, Díaz Cordovés-Rego G, Vera Casaño A, Urda Cardona A. Lupus erythematosus tumidus: Benign disease in children? Pediatr Int (Roma) 2018; 60(2): 196-7.
[http://dx.doi.org/10.1111/ped.13452]
[46]
Mostow ENRM, Reedy MS. A unique presentation of lupus erythematosus tumidus in an adolescent boy. Cutis 2019; 104(1): E6-7.
[PMID: 31487347]
[47]
Sonntag M, Lehmann P, Megahed M, Ruzicka T, Kuhn A. Lupus erythematosus tumidus in childhood. Report of 3 patients. Dermatology 2003; 207(2): 188-92.
[http://dx.doi.org/10.1159/000071793] [PMID: 12920372]
[48]
Taïeb A, Hehunstre JP, Goetz J, et al. Lupus erythematosus panniculitis with partial genetic deficiency of C2 and C4 in a child. Arch Dermatol 1986; 122(5): 576-82.
[http://dx.doi.org/10.1001/archderm.1986.01660170106030] [PMID: 3707176]
[49]
Sánchez NP, Peters MS, Winkelmann RK. The histopathology of lupus erythematosus panniculitis. J Am Acad Dermatol 1981; 5(6): 673-80.
[http://dx.doi.org/10.1016/S0190-9622(81)70129-4] [PMID: 7033308]
[50]
Ng PPL, Tan SH, Tan T. Lupus erythematosus panniculitis: a clinicopathologic study. Int J Dermatol 2002; 41(8): 488-90.
[http://dx.doi.org/10.1046/j.1365-4362.2002.01510.x] [PMID: 12207763]
[51]
Arai S, Katsuoka K. Clinical entity of Lupus erythematosus panniculitis/lupus erythematosus profundus. Autoimmun Rev 2009; 8(6): 449-52.
[http://dx.doi.org/10.1016/j.autrev.2008.12.011] [PMID: 19162248]
[52]
Hedrich CM, Fiebig B, Hauck FH, et al. Chilblain lupus erythematosus--a review of literature. Clin Rheumatol 2008; 27(8): 949-54.
[http://dx.doi.org/10.1007/s10067-008-0942-9] [PMID: 18543054]
[53]
König N, Fiehn C, Wolf C, et al. Familial chilblain lupus due to a gain-of-function mutation in STING. Ann Rheum Dis 2017; 76(2): 468-72.
[http://dx.doi.org/10.1136/annrheumdis-2016-209841] [PMID: 27566796]
[54]
Demirci GT, Altunay IK, Sarıkaya S, Sakiz D. Lupus erythematosus and lichen planus overlap syndrome:a case report with a rapid response to topical corticosteroid therapy. Dermatol Rep 2011; 3(3): e48.
[http://dx.doi.org/10.4081/dr.2011.e48] [PMID: 25386300]
[55]
Sekar CS, Rai R, Karthika N, Laila A. Scle-lp overlap syndrome. Indian J Dermatol 2011; 56(2): 209-10.
[http://dx.doi.org/10.4103/0019-5154.80420] [PMID: 21716551]
[56]
Werth V. Current treatment of cutaneous lupus erythematosus. Dermatol Online J 2001; 7(1): 2.
[PMID: 11328623]
[57]
Ruzicka T, Sommerburg C, Goerz G, Kind P, Mensing H. Treatment of cutaneous lupus erythematosus with acitretin and hydroxychloroquine. Br J Dermatol 1992; 127(5): 513-8.
[http://dx.doi.org/10.1111/j.1365-2133.1992.tb14851.x] [PMID: 1467292]
[58]
Kuhn A, Aberer E, Bata-Csörgő Z, et al. S2k guideline for treatment of cutaneous lupus erythematosus - guided by the European Dermatology Forum (EDF) in cooperation with the European Academy of Dermatology and Venereology (EADV). J Eur Acad Dermatol Venereol 2017; 31(3): 389-404.
[http://dx.doi.org/10.1111/jdv.14053] [PMID: 27859683]
[59]
Chasset F, Bouaziz JD, Costedoat-Chalumeau N, Francès C, Arnaud L. Efficacy and comparison of antimalarials in cutaneous lupus erythematosus subtypes: a systematic review and meta-analysis. Br J Dermatol 2017; 177(1): 188-96.
[http://dx.doi.org/10.1111/bjd.15312] [PMID: 28112801]
[60]
Chasset F, Arnaud L, Jachiet M, et al. Changing antimalarial agents after inefficacy or intolerance in patients with cutaneous lupus erythematosus: A multicenter observational study. J Am Acad Dermatol 2018; 78(1): 107-114.e1.
[http://dx.doi.org/10.1016/j.jaad.2017.08.045] [PMID: 29061479]
[61]
Lindskov R, Reymann F. Dapsone in the treatment of cutaneous lupus erythematosus. Dermatologica 1986; 172(4): 214-7.
[http://dx.doi.org/10.1159/000249338] [PMID: 3519302]
[62]
Chasset FTT, Cesbron E, et al. Efficacy and tolerance profile of thalidomide in cutaneous lupus erythematosus: a systematic review and metaanalysis. J Am Acad Dermatol 2018; 78: 342.e4-50.e4.
[http://dx.doi.org/10.1016/j.jaad.2017.09.059]
[63]
Correll CK, Miller DD, Maguiness SM. Treatment of Childhood-Onset Lupus Erythematosus Panniculitis With Rituximab. JAMA Dermatol 2020; 156(5): 566-9.
[http://dx.doi.org/10.1001/jamadermatol.2019.4984] [PMID: 32049306]
[64]
Shi H, Gudjonsson JE, Kahlenberg JM. Treatment of cutaneous lupus erythematosus: current approaches and future strategies. Curr Opin Rheumatol 2020; 32(3): 208-14.
[http://dx.doi.org/10.1097/BOR.0000000000000704] [PMID: 32141953]

Rights & Permissions Print Cite
© 2024 Bentham Science Publishers | Privacy Policy