Polymorphous Light Eruption 

  • Author: Noah S Scheinfeld, MD, JD, FAAD; Chief Editor: Dirk M Elston, MD   more...
 
Updated: Jul 27, 2011
 

Background

Polymorphous light eruption (PMLE) is an acquired disease and is the most common of the idiopathic photodermatoses. PMLE is characterized by recurrent, abnormal, delayed reactions to sunlight, ranging from erythematous papules, papulovesicles, and plaques to erythema multiforme –like lesions on sunlight-exposed surfaces. Within any 1 patient, only 1 clinical form is consistently manifested.

The possibility that a subset of PMLE called benign summer light eruption or BSLE which might be milder and might be more UVA driven has been suggested by an Italian group.[1]

Richards et al found that PMLE engenders a substantial psychosocial impact on patients who have the condition.[2] Based on results from the Illness Perception Questionnaire sent to 302 patients and returned by 150 patients, 40% experienced emotional distress linked to PMLE. The psychological impact was related to the predicted consequences of PMLE, whereas health-related variables played a lesser role. Women associated more severe consequences linked to PMLE and were more emotionally distressed than men.

Note the images below.

The face of a patient with polymorphous light erupThe face of a patient with polymorphous light eruption. Note the erythema and an indurated plaque. Close-up view of polymorphous light eruption plaquClose-up view of polymorphous light eruption plaque.
Next

Pathophysiology

The etiology of polymorphous light eruption (PMLE) is not fully known, and it is likely to be multifactorial. The immunologic pathogenesis of PMLE is supported by the study of timed biopsy samples of PMLE lesions. The CD4 subtype of T cells seen very early after exposure is replaced by CD8 lymphocytes 72 hours after irradiation. In general, findings conform to type IV delayed-type hypersensitivity mechanism.

It is possible that the use of tobacco makes PMPLE worse.[3]

In some PMLE lesions induced by UV-A, keratinocytes were found to express intercellular adhesion molecule 1 (ICAM-1).[4, 5] ICAM-1 is absent from normal keratinocytes, but it is known to be strongly induced by interferon gamma. The induction of ICAM-1 on keratinocytes results either from direct effects of UV on the promoter region of the ICAM-1 gene or from indirect effects of interferon gamma produced by activated lymphocytes aggregating in an underlying PMLE.

Intravascular and focal perivascular deposits of fibrin were detected in biopsy samples of PMLE papules. Vascular deposits of C3 and immunoglobulin M (IgM) were noted in a few patients. These findings may suggest that vascular injury with activation of a clotting cascade may play a role in the pathogenesis of PMLE. Repair of ultraviolet-damaged DNA is normal.

The demonstration that the female hormone 17beta-estradiol prevents UV radiation–induced suppression of the contact hypersensitivity response caused by the release of immunosuppressive cytokines (interleukin [IL]–10) from keratinocytes might thus explain why the risk of PMLE is higher in females than in males and why the risk decreases in women after menopause.[6]

Neutrophils may play a role in the development of PMLE. Immunohistochemical analysis by Schornagel et al in 2004 showed a significant decreased neutrophil infiltration in PMLE skin after UV-B irradiation compared with healthy case control subjects (P < .05).[7] ICAM-1 and E-selectin expression on endothelial cells increased in both healthy controls and in the PMLE patients after UV-B irradiation. Chemotactic response towards IL-8 and C5a was not different between PMLE patients and healthy controls. The authors concluded that PMLE is marked by an altered immune response resulting in decreased skin infiltration of neutrophils after UV-B irradiation.

Kölgen et al noted that the reduced expression of tumor necrosis factor-alpha, IL-4, and IL-10 in the UV-B–irradiated skin of patients with PMLE.[8] The reduction of these cytokines seems linked to a relative neutropenia and is a manifestation of decreased Langerhans cell migration and reduced TH2 skewing. An impairment of these mechanisms underlying UV-B–induced immunosuppression may be important in the pathogenesis of PMLE.[9]

A study by Koulu et al assessed a total of 48 subjects (24 patients with PMLE and 24 healthy sex-matched and age-matched controls).[10] The study found similar immunosuppression of contact sensitization to diphenylcyclopropenone due to earlier exposure to solar-simulating UV radiation between both groups. However, among patients with PMLE who were immunosuppressed by UV radiation, only one exhibited immunotolerance to the same allergen 10–24 months later (P=0.023). The study concluded that impaired propensity to UV-induced, allergen-specific immunotolerance may promote recurrent PMLE.

Previous
Next

Epidemiology

Frequency

United States

Polymorphous light eruption (PMLE) affects about 10% of the US population. This figure is likely to be an underestimate because many patients do not seek medical attention. Many of the photodermatoses were lumped together before their individual pathogeneses were identified. PMLE is now a distinct clinical entity, as are many of the other photodermatoses (eg, solar urticaria, photoallergic dermatitis, hydroa vacciniforme, chronic actinic dermatitis, erythropoietic porphyria, lupus erythematosus).

Reported in 2007, Kerr and Lim identified 280 patients with photodermatoses.[11] One hundred thirty-five (48%) were African Americans, 110 (40%) were white, and 35 (12%) were patients of other races. They noted a statistically significantly higher proportion of African Americans with PMLE compared with whites.

Benanni et al noted a low incidence of PMLE in renal transplant recipients,[12] and Hönigsmann reports a prevalence of 10-20% in the United States and Western Europe.[13]

International

Deng et al used a questionnaire to survey 4899 residents (49% men and 51% women) of random Chinese villages in Yuan Jiang county (Dai and Hani minorities), Kunming city (Han people and Yi minority), Lijiang county (Naxi minority), and Shangri-La county (Zang minority).[14] The altitudes of these regions were 380 meters, 1870 meters, 2410 meters, and 3280 meters, respectively. The prevalence of PMLE was 32 (0.65%) in 4899 residents and was 3.8 times higher in women compared with men. At higher elevations, the prevalence of PMLE increased. The mean time of sun exposure for PMLE was 6 h/d. The mean duration of PMLE was 5.8 years.

PMLE affects 21% of the population in Sweden.

Mortality/Morbidity

Expression of polymorphous light eruption (PMLE) may range from an insignificant, mild rash to severe disease affecting the patient's quality of life. Each case should be evaluated individually.

Richards et al found that emotional distress attributable to PMLE occurred in greater than 40% of individuals.[2] Women more than men associated more severe consequences with their PMLE and experienced more emotional distress.

Race

Polymorphous light eruption (PMLE) affects all racial skin types, but it is more common in fair-skinned individuals with skin types I-IV than in other individuals. Overall, family history is positive for PMLE in about 15% of the patients. However, Native Americans have a hereditary form of PMLE with apparent autosomal dominant inheritance; 75% reveal disease in a family member.[15]

Sex

Polymorphous light eruption (PMLE) affects females 2-3 times more often than males. However, these data may be skewed because women are more likely to seek medical attention for cosmetic problems than males.

Age

Polymorphous light eruption (PMLE) usually has an onset in the first 3 decades of life. Men seem to have later onset of the disease than women.

Naleway et al reviewed records of 124 patients diagnosed with PMLE and found most were women and that the mean age of PMLE onset was 37.8 years.[16] They noted only 4 required phototherapy treatment.

Previous
Proceed to Clinical Presentation
 
 
Contributor Information and Disclosures
Author

Noah S Scheinfeld, MD, JD, FAAD  Assistant Clinical Professor, Department of Dermatology, Columbia University College of Physicians and Surgeons; Consulting Staff, Department of Dermatology, St Luke's Roosevelt Hospital Center, Beth Israel Medical Center, and New York Eye and Ear Infirmary; Private Practice

Noah S Scheinfeld, MD, JD, FAAD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Optigenex Consulting fee Independent contractor

Coauthor(s)

Sophie Shirin, MD  Consulting Staff, Global Dermatology

Sophie Shirin, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Raul Del Rosario, MD  Consulting Staff, Dermatopathology, Mission Hospital at Laguna Beach

Raul Del Rosario, MD is a member of the following medical societies: American Society for Clinical Pathology

Disclosure: Nothing to disclose.

Specialty Editor Board

Craig A Elmets, MD  Professor and Chair, Department of Dermatology, Director, UAB Skin Diseases Research Center, University of Alabama at Birmingham School of Medicine

Craig A Elmets, MD is a member of the following medical societies: American Academy of Dermatology, American Association of Immunologists, American College of Physicians, American Federation for Medical Research, and Society for Investigative Dermatology

Disclosure: Palomar Medical Technologies Stock None; Astellas Consulting fee Review panel membership; Massachusetts Medical Society Salary Employment; Abbott Laboratories Grant/research funds Independent contractor; UpToDate Salary Employment; Biogen Grant/research funds Independent contractor; Clinuvel Independent contractor; Covan Basilea Pharmaceutical Grant/research funds Independent contractor; ISDIN None Consulting; TenX BIopharma Grant/research funds Independent contractor

Richard P Vinson, MD  Assistant Clinical Professor, Department of Dermatology, Texas Tech University Health Sciences Center, Paul L Foster School of Medicine; Consulting Staff, Mountain View Dermatology, PA

Richard P Vinson, MD is a member of the following medical societies: American Academy of Dermatology, Association of Military Dermatologists, Texas Dermatological Society, and Texas Medical Association

Disclosure: Nothing to disclose.

Jeffrey P Callen, MD  Professor of Medicine (Dermatology), Chief, Division of Dermatology, University of Louisville School of Medicine

Jeffrey P Callen, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, and American College of Rheumatology

Disclosure: Amgen Honoraria Consulting; Abbott Honoraria Consulting; Electrical Optical Sciences Consulting fee Consulting; Celgene Honoraria Safety Monitoring Committee; GSK - Glaxo Smith Kline Consulting fee Consulting; TenXBioPharma Consulting fee Safety Monitoring Committee

Joel M Gelfand, MD, MSCE  Medical Director, Clinical Studies Unit, Assistant Professor, Department of Dermatology, Associate Scholar, Center for Clinical Epidemiology and Biostatistics, University of Pennsylvania

Joel M Gelfand, MD, MSCE is a member of the following medical societies: Society for Investigative Dermatology

Disclosure: AMGEN Consulting fee Consulting; AMGEN Grant/research funds Investigator; Genentech Grant/research funds investigator; Centocor Consulting fee Consulting; Abbott Grant/research funds investigator; Abbott Consulting fee Consulting; Novartis investigator; Pfizer Grant/research funds investigator; Celgene Consulting fee DMC Chair; NIAMS and NHLBI Grant/research funds investigator

Chief Editor

Dirk M Elston, MD  Director, Ackerman Academy of Dermatopathology, New York

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Acknowledgments

The authors and editors of eMedicine gratefully acknowledge the contributions of previous author, Dr. Ada Winkielman, to the development and writing of this article.

References

  1. Guarrera M, Cardo P, Rebora AE, Schena D, Calzavara-Pinton P, Venturini M, et al. Polymorphous light eruption and benign summer light eruption in Italy. Photodermatol Photoimmunol Photomed. Feb 2011;1:35-9. [Medline].

  2. Richards HL, Ling TC, Evangelou G, et al. Psychologic distress in polymorphous light eruption and its relationship to patients' beliefs about their condition. J Am Acad Dermatol. Mar 2007;56(3):426-31. [Medline].

  3. Metelitsa AI, Lauzon GJ. Tobacco and the skin. Clin Dermatol. Jul-Aug 2010;4:384-90. [Medline].

  4. Norris PG, Barker JN, Allen MH, et al. Adhesion molecule expression in polymorphic light eruption. J Invest Dermatol. Oct 1992;99(4):504-8. [Medline].

  5. Stephansson E, Ros AM. Expression of intercellular adhesion molecule-1 (ICAM-1) and OKM5 in UVA- and UVB-induced lesions in patients with lupus erythematosus and polymorphous light eruption. Arch Dermatol Res. 1993;285(6):328-33. [Medline].

  6. Aubin F. Why is polymorphous light eruption so common in young women?. Arch Dermatol Res. Oct 2004;296(5):240-1. [Medline].

  7. Schornagel IJ, Sigurdsson V, Nijhuis EH, Bruijnzeel-Koomen CA, Knol EF. Decreased neutrophil skin infiltration after UVB exposure in patients with polymorphous light eruption. J Invest Dermatol. Jul 2004;123(1):202-6. [Medline].

  8. Kolgen W, van Meurs M, Jongsma M, et al. Differential expression of cytokines in UV-B-exposed skin of patients with polymorphous light eruption: correlation with Langerhans cell migration and immunosuppression. Arch Dermatol. Mar 2004;140(3):295-302. [Medline].

  9. Wackernagel A, Back B, Quehenberger F, Cerroni L, Kerl H, Wolf P. Langerhans cell resistance, CD11b+ cell influx, and cytokine mRNA expression in skin after UV exposure in patients with polymorphous light eruption as compared with healthy control subjects. J Invest Dermatol. May 2004;122(5):1342-4. [Medline].

  10. Koulu LM, Laihia JK, Peltoniemi HH, Jansén CT. UV-induced tolerance to a contact allergen is impaired in polymorphic light eruption. J Invest Dermatol. Nov 2010;130(11):2578-82. [Medline].

  11. Kerr HA, Lim HW. Photodermatoses in African Americans: a retrospective analysis of 135 patients over a 7-year period. J Am Acad Dermatol. Oct 2007;57(4):638-43. [Medline].

  12. Benanni B, Bruckner T, Bock M, et al. Low incidence of polymorphous light eruption in renal transplant recipients. Acta Derm Venereol. 2007;87(4):372-4. [Medline].

  13. Hönigsmann H. Polymorphous light eruption. Photodermatol Photoimmunol Photomed. Jun 2008;24(3):155-61. [Medline].

  14. Deng D, Hang Y, Chen H, Li H. Prevalence of photodermatosis in four regions at different altitudes in Yunnan province, China. J Dermatol. Aug 2006;33(8):537-40. [Medline].

  15. Draelos ZK, Hansen RC. Polymorphic light eruption in pediatric patients with American Indian ancestry. Pediatr Dermatol. Nov 1986;3(5):384-9. [Medline].

  16. Naleway AL, Greenlee RT, Melski JW. Characteristics of diagnosed polymorphous light eruption. Photodermatol Photoimmunol Photomed. Aug 2006;22(4):205-7. [Medline].

  17. Lugovic Mihic L, Bulat V, Situm M, Cavka V, Krolo I. Allergic hypersensitivity skin reactions following sun exposure. Coll Antropol. Oct 2008;32 Suppl 2:153-7. [Medline].

  18. Jansen CT. The natural history of polymorphous light eruptions. Arch Dermatol. Feb 1979;115(2):165-9. [Medline].

  19. Majoie IM, van Weelden H, Sybesma IM, Coenraads PJ, Sigurdsson V. Polymorphous light eruption-like skin lesions in welders caused by ultraviolet C light. J Am Acad Dermatol. Jan 2010;62(1):150-1. [Medline].

  20. Kontos AP, Cusack CA, Chaffins M, Lim HW. Polymorphous light eruption in African Americans: pinpoint papular variant. Photodermatol Photoimmunol Photomed. Dec 2002;18(6):303-6. [Medline].

  21. Chiam LY, Chong WS. Pinpoint papular polymorphous light eruption in Asian skin: a variant in darker-skinned individuals. Photodermatol Photoimmunol Photomed. 2009;25:71-4. [Medline].

  22. Gronhagen CM, Gunnarsson I, Svenungsson E, Nyberg F. Cutaneous manifestations and serological findings in 260 patients with systemic lupus erythematosus. Lupus. Sep 2010;19(10):1187-94. [Medline].

  23. Popovic K, Nyberg F, Wahren-Herlenius M, Nyberg F. A serology-based approach combined with clinical examination of 125 Ro/SSA-positive patients to define incidence and prevalence of subacute cutaneous lupus erythematosus. Arthritis Rheum. Jan 2007;56(1):255-64. [Medline].

  24. Schornagel IJ, Guikers KL, Van Weelden H, Brijnzeel-Koomen CA, Sigurdsson V. The polymorphous light eruption-severity assessment score does not reliably predict the results of phototesting. J Eur Acad Dermatol Venereol. Jun 2008;22(6):675-80. [Medline].

  25. Leroy D, Dompmartin A, Verneuil L, Michel M, Faguer K. [Polychromatic phototest sensibility is superior to UVA phototest in polymorphic light eruptions]. Ann Dermatol Venereol. Jun-Jul 2002;129(6-7):860-4. [Medline].

  26. Hadshiew IM, Treder-Conrad C, v Bülow R, et al. Polymorphous light eruption (PLE) and a new potent antioxidant and UVA-protective formulation as prophylaxis. Photodermatol Photoimmunol Photomed. Aug 2004;20(4):200-4. [Medline].

  27. Gruber-Wackernagel A, Bambach I, Legat FJ, et al. Randomized double-blinded placebo-controlled intra-individual trial on topical treatment with a 1,25-dihydroxyvitamin D(3) analogue in polymorphic light eruption. Br J Dermatol. Jul 2011;165(1):152-63. [Medline].

  28. Jeanmougin M, Peyron JL, Thomas P, Beani JC, Guez E, Bachot N. [Polymorphic light eruption: prophylaxis using a topical combination of antioxidants and UVA protection formulations]. Ann Dermatol Venereol. May 2006;133(5 Pt 1):425-8. [Medline].

  29. DeLeo VA, Clark S, Fowler J, Poncet M, Loesche C, Soto P. A new ecamsule-containing SPF 40 sunscreen cream for the prevention of polymorphous light eruption: a double-blind, randomized, controlled study in maximized outdoor conditions. Cutis. Feb 2009;83(2):95-103. [Medline].

  30. Fourtanier A, Moyal D, Seite S. Sunscreens containing the broad-spectrum UVA absorber, Mexoryl SX, prevent the cutaneous detrimental effects of UV exposure: a review of clinical study results. Photodermatol Photoimmunol Photomed. Aug 2008;24(4):164-74. [Medline].

  31. Murphy GM, Logan RA, Lovell CR, Morris RW, Hawk JL, Magnus IA. Prophylactic PUVA and UVB therapy in polymorphic light eruption--a controlled trial. Br J Dermatol. Apr 1987;116(4):531-8. [Medline].

  32. Bilsland D, George SA, Gibbs NK, Aitchison T, Johnson BE, Ferguson J. A comparison of narrow band phototherapy (TL-01) and photochemotherapy (PUVA) in the management of polymorphic light eruption. Br J Dermatol. Dec 1993;129(6):708-12. [Medline].

  33. Barolet D, Boucher A. LED photoprevention: reduced MED response following multiple LED exposures. Lasers Surg Med. Feb 2008;40(2):106-12. [Medline].

  34. Jansen CT. Oral carotenoid treatment in polymorphous light eruption: a cross-over comparison with oxychloroquine and placebo. Photodermatol. Jun 1985;2(3):166-9. [Medline].

  35. Neumann R, Rappold E, Pohl-Markl H. Treatment of polymorphous light eruption with nicotinamide: a pilot study. Br J Dermatol. Jul 1986;115(1):77-80. [Medline].

  36. Ahmed RS, Suke SG, Seth V, Jain A, Bhattacharya SN, Banerjee BD. Impact of oral vitamin E supplementation on oxidative stress & lipid peroxidation in patients with polymorphous light eruption. Indian J Med Res. Jun 2006;123(6):781-7. [Medline].

  37. Norris PG, Hawk JL. Successful treatment of severe polymorphous light eruption with azathioprine. Arch Dermatol. Oct 1989;125(10):1377-9. [Medline].

  38. Boonstra HE, van Weelden H, Toonstra J, van Vloten WA. Polymorphous light eruption: A clinical, photobiologic, and follow-up study of 110 patients. J Am Acad Dermatol. Feb 2000;42(2 Pt 1):199-207. [Medline].

  39. Calzavara Pinton PG, Venturini M, Capezzera R, Zane C, Facchetti F. Photosensitive erythema multiforme and erythema multiforme-like polymorphous light eruption. Photodermatol Photoimmunol Photomed. Jun 2003;19(3):157-9. [Medline].

  40. Dummer R, Ivanova K, Scheidegger EP, Burg G. Clinical and therapeutic aspects of polymorphous light eruption. Dermatology. 2003;207(1):93-5. [Medline].

  41. Epstein JH. Polymorphous light eruption. J Am Acad Dermatol. Oct 1980;3(4):329-43. [Medline].

  42. Epstein JH. Polymorphous light eruption. Photodermatol Photoimmunol Photomed. Jun 1997;13(3):89-90. [Medline].

  43. Fesq H, Ring J, Abeck D. Management of polymorphous light eruption : clinical course, pathogenesis, diagnosis and intervention. Am J Clin Dermatol. 2003;4(6):399-406. [Medline].

  44. Jansen CT. The morphologic features of polymorphous light eruptions. Cutis. Aug 1980;26(2):164-7, 169-70. [Medline].

  45. McFadden N, Larsen TE. Polymorphous light eruption: the properties of a UVA-induced PLME patient group. Photodermatol. Feb 1986;3(1):36-40. [Medline].

  46. Muhlbauer JE, Mihm MC Jr, Harrist TJ. Papular polymorphous light eruption. Fibrin, complement, and immunoglobulin deposition. Arch Dermatol. Jul 1984;120(7):866-8. [Medline].

  47. Schornagel IJ, Knol EF, van Weelden H, Guikers CL, Bruijnzeel-Koomen CA, Sigurdsson V. Diagnostic phototesting in polymorphous light eruption: the optimal number of irradiations. Br J Dermatol. Dec 2005;153(6):1234-6. [Medline].

 
Previous
Next
 
The face of a patient with polymorphous light eruption. Note the erythema and an indurated plaque.
Close-up view of polymorphous light eruption plaque.
Papular variant of polymorphous light eruption.
Large facial plaques.
Histopathologic features of polymorphous light eruption. Note the tight perivascular lymphocytic infiltrate in the upper dermis.
Close-up view of the perivascular infiltrate.
Photograph of a springtime eruption of polymorphous light eruption in a 6-year-old boy. The eruption has occurred in the spring since the patient was aged 5 years, and it resolves completely with no scarring by mid to late summer. High block sunscreens attenuate but do not prevent the eruption. No itching or pain occurs. Courtesy of Jeremy F. Harrison, FRCA, FFAEM.
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2012 by WebMD LLC.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.