Skip to main content

Advertisement

SpringerLink
Log in

Photosensitivity in Lupus Erythematosus and Dermatomyositis

  • Photodermatology (B Adler and V DeLeo, Section Editor)
  • Published:
Current Dermatology Reports Aims and scope Submit manuscript

Abstract

Purpose of Review

Photosensitivity is a common symptom in the autoimmune diseases lupus erythematosus (LE) and dermatomyositis (DM). This review provides a framework for dermatologists, rheumatologists, and general practitioners to aid in recognizing and understanding photosensitivity reactions within the scope of these conditions.

Recent Findings

Photosensitivity reactions encompass not only cutaneous symptoms but also include systemic manifestations. Prevalence of photosensitivity is high in both LE and DM and tends to vary based on race and geographical location. Pathophysiology implicates the various effects of ultraviolet (UV) light on immunoreactants in the skin. Photoprotection remains the leading method of prevention of photosensitive response.

Summary

Photosensitivity heavily impacts quality of life (QoL) in patients with LE and DM. By accurately recognizing these reactions, physicians can better treat these autoimmune conditions.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Similar content being viewed by others

References

Papers of particular interest, published recently, have been highlighted as: • Of importance •• Of major importance

  1. Tan EM, Cohen AS, Fries JF, Masi AT, McShane DJ, Rothfield NF, et al. The 1982 revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum. 1982;25:1271–7. https://doi.org/10.1002/art.1780251101.

    Article  CAS  PubMed  Google Scholar 

  2. Scheinfeld N, Deleo VA. Photosensitivity in lupus erythematosus. Photodermatol Photoimmunol Photomed. 2004;20:272–9. https://doi.org/10.1111/j.1600-0781.2004.00094.x.

    Article  PubMed  Google Scholar 

  3. Vila LM, Mayor AM, Valentin AH, Rodriguez SI, Reyes ML, Acosta E, et al. Association of sunlight exposure and photoprotection measures with clinical outcome in systemic lupus erythematosus. P R Health Sci J. 1999;18:89–94 Doi:10461313.

    CAS  PubMed  Google Scholar 

  4. Kuhn A, Beissert S. Photosensitivity in lupus erythematosus. Autoimmunity. 2005;38:519–29. https://doi.org/10.1080/08916930500285626.

    Article  CAS  PubMed  Google Scholar 

  5. • Foreing K, Chang AY, Piette EW, Cucchiara A, Okawa J, Werth VP. Characterization of clinical photosensitivity in cutaneous lupus erythematosus. J Am Acad Dermatol. 2013;69:205–13. https://doi.org/10.1016/j.jaad.2013.03.015Explains the systemic manifestations of photosensitive reactions.

    Article  Google Scholar 

  6. • Stafford R, Farrar MD, Kift R, Durkin MT, Berry JL, Webb AR, et al. The impact of photosensitivity disorders on aspects of lifestyle. Br J Dermatol. 2010;163:817–22. https://doi.org/10.1111/j.1365-2133.2010.09905.xStates the effects of photosensitive reactions on quality of life in patients with autoimmune diseases.

    Article  CAS  PubMed  Google Scholar 

  7. Walchner M, Messer G, Kind P. Phototesting and photoprotection in LE. Lupus. 1997;6:167–74. https://doi.org/10.1177/096120339700600212.

    Article  CAS  PubMed  Google Scholar 

  8. • Kuhn A, Ruland V, Bonsmann G. Photosensitivity, phototesting, and photoprotection in cutaneous lupus erythematosus. Lupus. 2010;19:1036–46. https://doi.org/10.1177/0961203310370344Discussess prevalence of photosensitve reactions in different subtypes of lupus. Explains prevalence of PMLE reaction in LE patients. Discusses various factors of pathogenesis of LE.

    Article  CAS  PubMed  Google Scholar 

  9. Epstein JH, Tuffanelli D, Dubois EL. Light sensitivity and lupus Erythematosus. Arch Dermatol. 1965;91:483–5. https://doi.org/10.1001/archderm.1965.01600110069013.

    Article  CAS  PubMed  Google Scholar 

  10. Freeman RG, Knox JM, Owens DW. Cutaneous lesions of lupus erythematosus induced by monochromatic light. Arch Dermatol. 1969;100:677–82 Doi:5365215.

    Article  CAS  Google Scholar 

  11. Lehmann P, Holzle E, Kind P, Goerz G, Plewig G. Experimental reproduction of skin lesions in lupus erythematosus by UVA and UVB radiation. J Am Acad Dermatol. 1990;22:181–7. https://doi.org/10.1016/0190-9622(90)70020-i.

    Article  CAS  PubMed  Google Scholar 

  12. Kuhn A, Sonntag M, Richter-Hintz D, Oslislo C, Megahed M, Ruzicka T, et al. Phototesting in lupus erythematosus: a 15-year experience. J Am Acad Dermatol. 2001;45:86–95. https://doi.org/10.1067/mjd.2001.114589.

    Article  CAS  PubMed  Google Scholar 

  13. Kind P, Lehmann P, Plewig G. Phototesting in lupus erythematosus. J Invest Dermatol. 1993;100:53s–7s. https://doi.org/10.1111/2F1523-1747.ep12355594.

    Article  CAS  PubMed  Google Scholar 

  14. • Kim A, Chong BF. Photosensitivity in cutaneous lupus erythematosus. Photodermatol Photoimmunol Photomed. 2013;29:4–11. https://doi.org/10.1111/phpp.12018Explains how to differentiate PMLE reaction from true LE. Discusses pathogenesis of LE in regard to UV exposure.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  15. Tzaneva S, Volc-Platzer B, Kittler H, Honigsmann H, Tanew A. Antinuclear antibodies in patients with polymorphic light eruption: a long-term follow-up study. Br J Dermatol. 2008;158:1050–4. https://doi.org/10.1111/j.1365-2133.2008.08500.x.

    Article  CAS  PubMed  Google Scholar 

  16. Hasan T, Nyberg F, Stephansson E, Puska P, Hakkinen P, Sarna S, et al. Photosensitivity in lupus erythematosus, UV photoprovocation results compared with history of photosensitivity and clinical findings. Br J Dermatol. 1997;136:699–705 Doi:9205502.

    Article  CAS  Google Scholar 

  17. Sanders CJ, Van Weelden H, Kazzaz GA, Sigurdsson V, Toonstra J, Bruijnzeel-Koomen CA. Photosensitivity in patients with lupus erythematosus: a clinical and photobiological study of 100 patients using a prolonged phototest protocol. Br J Dermatol. 2003;149:131–7. https://doi.org/10.1046/j.1365-2133.2003.05379.x.

    Article  CAS  PubMed  Google Scholar 

  18. Krause I, Shraga I, Molad Y, Guedj D, Weinberger A. Seasons of the year and activity of SLE and Behcet’s disease. Scand J Rheumatol. 1997;26:435–9. https://doi.org/10.3109/03009749709065715.

    Article  CAS  PubMed  Google Scholar 

  19. Leone J, Pennaforte JL, Delhinger V, Detour J, Lefondre K, Eschard JP, et al. Influence of seasons on risk of flare-up of systemic lupus: retrospective study of 66 patients. Rev Med Interne. 1997;18:286–91. https://doi.org/10.1016/s0248-8663(97)84013-1.

    Article  CAS  PubMed  Google Scholar 

  20. Furukawa F, Itoh T, Wakita H, Yagi H, Tokura Y, Norris DA, et al. Keratinocytes from patients with lupus erythematosus show enhanced cytotoxicity to ultraviolet radiation and to antibody-mediated cytotoxicity. Clin Exp Immunol. 1999;118:164–70. https://doi.org/10.1046/j.1365-2249.1999.01026.x.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  21. Kock A, Schwarz T, Kirnbauer R, Urbanski A, Perry P, Ansel JC, et al. Human keratinocytes are a source for tumor necrosis factor alpha: evidence for synthesis and release upon stimulation with endotoxin or ultraviolet light. J Exp Med. 1990;172:1609–14. https://doi.org/10.1084/jem.172.6.1609.

    Article  CAS  PubMed  Google Scholar 

  22. Casciola-Rosen L, Rosen A. Ultraviolet light-induced keratinocyte apoptosis: a potential mechanism for the induction of skin lesions and autoantibody production in LE. Lupus. 1997;6:175–80. https://doi.org/10.1177/096120339700600213.

    Article  CAS  PubMed  Google Scholar 

  23. Golan TD, Elkon KB, Gharavi AE, Krueger JG. Enhanced membrane binding of autoantibodies to cultured keratinocytes of systemic lupus erythematosus patients after ultraviolet B/ultraviolet A irradiation. J Clin Invest. 1992;90:1067–76. https://doi.org/10.1172/JCI115922.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  24. •• McGrath H. Ultraviolet-A1 irradiation therapy for systemic lupus erythematosus. Lupus. 2017;26:1239–51. https://doi.org/10.1177/0961203317707064Discusses effects of UVA2 spectrum and implication in pathogenesis of LE.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  25. Mitchell D. Revisiting the photochemistry of solar UVA in human skin. Proc Natl Acad Sci U S A. 2006;103:13567–8. https://doi.org/10.1073/pnas.0605833103.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  26. • Halliday GM, Bryne SN, Damian DL. Ultraviolet A radiation: its role in immunosuppression and carcinogenesis. Semin Cutan Med Surg. 2011;30:214–21. https://doi.org/10.1016/j.sder.2011.08.002Discusses augmentation of proinflammatory cytokines in LE as a result of UVA2 exposure.

    Article  CAS  PubMed  Google Scholar 

  27. Wolska H, Blaszczyk M, Jablonska S. Phototests in patients with various forms of lupus erythematosus. Int J Dermatol. 1989;28:98–103. https://doi.org/10.1111/j.1365-4362.1989.tb01327.x.

    Article  CAS  PubMed  Google Scholar 

  28. •• Stannard JN, Reed TJ, Meyers E, Lowe L, Sarkar MK, Xing X, et al. Lupus skin is primed for IL-6 inflammatory responses through a keratinocyte-mediated autocrine type I interferon loop. J Invest Dermatol. 2017;137:115–22. https://doi.org/10.1016/j.jid.2016.09.008Discusses effects of UV radiation on IFN-kappa expression.

    Article  CAS  PubMed  Google Scholar 

  29. •• Sarkar MK, Hile GA, Tsoi LC, Xing X, Liu J, Liang Y, et al. Photosensitivity and type I IFN responses in cutaneous lupus are driven by epidermal-derived interferon kappa. Ann Rheum Dis. 2018;77:1653–64. https://doi.org/10.1136/annrheumdis-2018-213197Discusses effects of IFN-kappa on the pathogenesis of LE.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  30. Mori M, Pimpinelli N, Romagnoli P, Bernacchi E, Fabbri P, Giannotti B. Dendritic cells in cutaneous lupus erythematosus: a clue to the pathogenesis of lesions. Histopathology. 1994;24:311–21. https://doi.org/10.1111/j.1365-2559.1994.tb00531.x.

    Article  PubMed  Google Scholar 

  31. Sontheimer RD. Photoimmunology of lupus erythematosus and dermatomyositis: a speculative review. Photochem Photobiol. 1996;63:583–94. https://doi.org/10.1111/j.1751-1097.1996.tb05660.x.

    Article  CAS  PubMed  Google Scholar 

  32. • Kuhn A, Gensch K, Haust M, Meuth AM, Boyer F, Dupuy P, et al. Photoprotective effects of a broad-spectrum sunscreen in ultraviolet-induced cutaneous lupus erythematosus: a randomized, vehicle-controlled, double-blind study. J Am Acad Dermatol. 2011;64:37–48. https://doi.org/10.1016/j.jaad.2009.12.053Discusses progression of phototesting in LE.

    Article  CAS  PubMed  Google Scholar 

  33. Wozniacka A, Lesiak A, McCauliffe DP, Sysa-Jedrzejowska A. Photoprotective properties of chloroquine phosphate. J Eur Acad Dermatol Venereol. 2007;21:1133–4. https://doi.org/10.1111/j.1468-3083.2006.02120.x.

    Article  CAS  PubMed  Google Scholar 

  34. • Reich A, Marcinow K, Bialynicki-Burla R. The lupus band test in systemic lupus erythematosus patients. Ther Clin Risk Manag. 2011;7:27–32. https://doi.org/10.2147/TCRM.S10145Discusses differential penetration of UVA spectrum into different layers of the skin. Discusses the effects of UVA spectrum after pentration in to dermal-epidermal junction.

    Article  PubMed  PubMed Central  Google Scholar 

  35. Theofilopoulos AN, Koundouris S, Kono DH, Lawson BR. The role of IFN-gamma in systemic lupus erythematosus: a challenge to the Th1/Th2 paradigm in autoimmunity. Arthritis Res. 2001;3:136–41. https://doi.org/10.1186/ar290.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  36. Harigai M, Kawamoto M, Hara M, Kubota T, Kamatani N, Miyasaka N. Excessive production of IFN-gamma in patients with systemic lupus erythematosus and its contribution to induction of B lymphocyte stimulator/B cell-activating factor/TNF ligand superfamily-13B. J Immunol. 2008;181:2211–9. https://doi.org/10.4049/jimmunol.181.3.2211.

    Article  CAS  PubMed  Google Scholar 

  37. Do RK, Chen-Kiang S. Mechanism of BLyS action in B cell immunity. Cytokine Growth Factor Rev. 2002;13:19–25. https://doi.org/10.1016/s1359-6101(01)00025-9.

  38. Collins CE, Gavin AL, Migone TS, Hilbert DM, Nemazee D, Stohl W. B lymphocyte stimulator (BLyS) isoforms in systemic lupus erythematosus: disease activity correlates better with blood leukocyte BLyS mRNA levels than with plasma BLyS protein levels. Arthritis Res Ther. 2006;8:R6. https://doi.org/10.1186/ar1855.

    Article  PubMed  Google Scholar 

  39. Alanson M, Reeve VE. Ultraviolet A (320-400 nm) modulation of ultraviolet B (290-320 nm)-induced immune suppression is mediated by carbon monoxide. J Invest Dermatol. 2005;124:644–50. https://doi.org/10.1111/j.0022-202X.2005.23614.x.

    Article  Google Scholar 

  40. Boulware DW, Hedgpeth MT. Lupus pneumonitis and anti-SSA(Ro) antibodies. J Rheumatol. 1989;16:479–81. https://doi.org/10.1111/j.0022-202X.2005.23614.x.

    Article  CAS  PubMed  Google Scholar 

  41. Deng C, Kaplan MJ, Yang J, Ray D, Zhang Z, McCune WJ, et al. Decreased Ras-mitogen-activated protein kinase signaling may cause DNA hypomethylation in T lymphocytes from lupus patients. Arthritis Rheum. 2001;44:397–407. https://doi.org/10.1002/1529-0131(200102)44:2<397::AID-ANR59>3.0.CO;2-N.

    Article  CAS  PubMed  Google Scholar 

  42. Roelandts R. The diagnosis of photosensitivity. Arch Dermatol. 2000;136:1152–7. https://doi.org/10.1001/archderm.136.9.1152.

    Article  CAS  PubMed  Google Scholar 

  43. Dourmishev L, Meffert H, Piazena H. Dermatomyositis: comparative studies of cutaneous photosensitivity in lupus erythematosus and normal subjects. Photodermatol Photoimmunol Photomed. 2004;20:230–4. https://doi.org/10.1111/j.1600-0781.2004.00115.x.

    Article  CAS  PubMed  Google Scholar 

  44. Cheong WK, Hughes GR, Norris PG, Hawk JL. Cutaneous photosensitivity in dermatomyositis. Br J Dermatol. 1994;131:205–8. https://doi.org/10.1111/j.1365-2133.1994.tb08492.x.

    Article  CAS  PubMed  Google Scholar 

  45. Nesbitt LT. Cutaneous immunofluorescence in dermatomyositis. Int J Dermatol. 1980;19:270–8. https://doi.org/10.1111/j.1365-4362.1980.tb00328.x.

    Article  PubMed  Google Scholar 

  46. Everett MA, Curtis AC. Dermatomyositis; a review of nineteen cases in adolescents and children. AMA Arch Intern Med. 1957;100:70–6. https://doi.org/10.1001/archinte.1957.00260070084009.

    Article  CAS  PubMed  Google Scholar 

  47. Euwer RL, Sontheimer RD. Amyopathic dermatomyositis (dermatomyositis siné myositis): presentation of six new cases and review of the literature. J Am Acad Dermatol. 1991;24:959–66.

  48. Quain RD, Werth VP. Management of cutaneous dermatomyositis: current therapeutic options. Am J Clin Dermatol. 2006;7:341–51. https://doi.org/10.2165/00128071-200607060-00002.

    Article  PubMed  Google Scholar 

  49. Chung JH, Kwon OS, Eun HC, Youn JI, Song YW, Kim JG, et al. Apoptosis in the pathogenesis of cutaneous lupus erythematosus. Am J Dermatopathol. 1998;20:233–41. https://doi.org/10.1097/00000372-199806000-00002.

    Article  CAS  PubMed  Google Scholar 

  50. Werth VP, Zhang W. Wavelength-specific synergy between ultraviolet radiation and interleukin-1 alpha in the regulation of matrix-related genes: mechanistic role for tumor necrosis factor-alpha. J Invest Dermatol. 1999;113:196–201. https://doi.org/10.1046/j.1523-1747.1999.00681.x.

    Article  CAS  PubMed  Google Scholar 

  51. Werth VP, Callen JP, Ang G, Sullivan KE. Associations of tumor necrosis factor alpha and HLA polymorphisms with adult dermatomyositis: implications for a unique pathogenesis. J Invest Dermatol. 2002;119:617–20. https://doi.org/10.1046/j.1523-1747.2002.01869.x.

    Article  CAS  PubMed  Google Scholar 

  52. Pachman LM, Liotta-Davis MR, Hong DK, Kinsella TR, Mendez EP, Kinder JM, et al. TNFalpha-308A allele in juvenile dermatomyositis: association with increased production of tumor necrosis factor alpha, disease duration, and pathologic calcifications. Arthritis Rheum. 2000;10:2368–77. https://doi.org/10.1002/1529-0131(200010)43:10%3C2368::AID-ANR26%3E3.0.CO;2-8.

    Article  Google Scholar 

  53. Schwarz A, Bhardwaj R, Aragane Y, Mahnke K, Riemann H, Metze D, et al. Ultraviolet-B-induced apoptosis of keratinocytes: evidence for partial involvement of tumor necrosis factor-alpha in the formation of sunburn cells. J Invest Dermatol. 1995;104:922–7. https://doi.org/10.1111/1523-1747.ep12606202.

    Article  CAS  PubMed  Google Scholar 

  54. •• Ogawa-Momohara M, Muro Y, Akiyama M. Anti-Mi-2 antibody titers and cutaneous manifestations in dermatomyositis. J of Cutan Immunol and Allergy. 2019;2(2):49–52. https://doi.org/10.1002/cia2.12048Describes association of anti-Mi-2 antibody and increased UV exposure in DM patients.

  55. •• Matsuda T, Ueda-Hayakawa I, Kambe N, Son Y, Ozaki Y, Hamaguchi Y, et al. Four cases of anti-Mi-2 antibody-positive dermatomyositis: relationship between anti-Mi-2 antibody titre and disease severity and activity. J Eur Acad Dermatol Venereol. 2018;32:e233–4. https://doi.org/10.1111/jdv.14754Describes association of anti-Mi-2 antibody with severity and activity of DM.

    Article  CAS  PubMed  Google Scholar 

  56. Burd CJ, Kinyamu HK, Miller FW, Archer TK. UV radiation regulates Mi-2 through protein translation and stability. J Biol Chem. 2008;283:34976–82. https://doi.org/10.1074/jbc.M805383200.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

Download references

Author information

Author notes

  1. Spandana Maddukuri and Jay Patel contributed equally to this work.

Authors and Affiliations

  1. Corporal Michael J. Crescenz VAMC, Philadelphia, PA, USA

    Spandana Maddukuri, Jay Patel & Victoria P. Werth

  2. Department of Dermatology, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, USA

    Spandana Maddukuri, Jay Patel & Victoria P. Werth

Authors
  1. Spandana Maddukuri
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Jay Patel
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Victoria P. Werth
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Victoria P. Werth.

Ethics declarations

Conflict of Interest

Dr. Victoria P. Werth is a consultant for Celgene, Medimmune, Resolve, Genetech, Idera, Janssen, Lilly, Pfizer, Biogen, BMS, Gilead, Amgen, Medscape, Nektar, Incyte, EMD Sorona, CSL Behring, Principia, Crisalis, Vielo, Argenx, Kirin, Regeneron, Principia, AstraZeneca, Kirin, Octapharma, and GSK. She has also received grants from Celgene, Janssen, Pfizer, Biogen, Gilead, Corbus Pharmaceuticals, Genentech, AstraZeneca, Viela, CSL Behring, and Syntimmune.

Human and Animal Rights and Informed Consent

This article does not contain any studies with human or animal subjects performed by any of the authors.

Additional information

Publisher’s Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

This article is part of the Topical Collection on Photodermatology

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Maddukuri, S., Patel, J. & Werth, V.P. Photosensitivity in Lupus Erythematosus and Dermatomyositis. Curr Derm Rep 9, 93–99 (2020). https://doi.org/10.1007/s13671-020-00300-7

Download citation

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s13671-020-00300-7

Keywords

Search

Navigation