Autoimmune Photodermatoses Part II - Clinical Manifestations and Treatment

  • Rita Ramos Pinheiro Serviço de Dermatologia, Hospital Santo António dos Capuchos, Centro Hospitalar de Lisboa Central, Lisboa, Portugal
  • Virgínia Coelho de Sousa Serviço de Dermatologia, Hospital Santo António dos Capuchos, Centro Hospitalar de Lisboa Central, Lisboa, Portugal
  • Filipa Rocha Páris Serviço de Dermatologia, Hospital Santo António dos Capuchos, Centro Hospitalar de Lisboa Central, Lisboa, Portugal
  • Margarida Apetato Serviço de Dermatologia, Hospital Santo António dos Capuchos, Centro Hospitalar de Lisboa Central, Lisboa, Portugal
  • Gabriela Marques Pinto Serviço de Dermatologia, Hospital Santo António dos Capuchos, Centro Hospitalar de Lisboa Central, Lisboa, Portugal
Keywords: Dermatitis, Photoallergic/diagnosis, Photoallergic/therapy, Immune System, Photosensitivity Disorders/ diagnosis, Photosensitivity Disorders/therapy, Ultraviolet Rays


The autoimmune photodermatoses are a group of heterogeneous idiopathic dermatoses including five different clinical entities some potentially severe with great impact in patients’ quality of life. General photoprotection measures are sufficient for the treatment of patients with mild forms of disease. In severe disease the therapeutic options are still limited, reflecting the ill-defined physiopathological mechanisms. However recent advances in photoprotection field are contributing to a higher therapeutic success. In an era where photodermatology has been losing its importance, we propose to revise the clinical manifestations and therapeutic options of autoimmune photodermatoses. These are rare and challenging disorders concerning diagnostic and treatment that need a specialized approach by dermatologists.


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Gruber-Wackernagel A, Byrne SN, Wolf P. Pathogenic

mechanisms of polymorphic light eruption. Front Biosci.

; 1:341-54.

Wolf P, Byrne SN, Gruber-Wackernagel A. New insights

into the mechanisms of polymorphic light eruption: resistance

to ultraviolet radiationinduced immune suppression

as an aetiological factor. Exp Dermatol. 2009;


Gruber-Wackernagel A, Byrne SN, Wolf P. Polymorphous

light eruption: clinic aspects and pathogenesis. Dermatol

Clin. 2014; 32:315-34.

Gonzalez E, Gonzalez S. Drug photosensitivity, idiopathic

photodermatoses, and sunscreens. J Am Acad Dermatol.

; 35:871-85.

Jansen CT, Karvonen J. Polymorphous light eruption. A

seven-year follow-up evaluation of 114 patients. Arch

Dermatol. 1984; 120:862-5.

Richards HL, Ling TC, Evangelou G, Brooke RC, Fortune

DG, Rhodes LE. Evidence of high levels of anxiety and

depression in polymorphic light eruption and their association

with clinical and demographic variables. Br J

Dermatol. 2008; 159:439-44.

Lava SA, Simonetti GD, Ragazzi M, Guarino Gubler S,

Bianchetti MG. Juvenile spring eruption: an outbreak report

and systematic review of the literature. Br J Dermatol.

; 168:1066-72.

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. 2011; 27:35-9.

Molina-Ruiz AM, Sanmartin O, Santonja C, Kutzner H,

Requena L. Spring and summer eruption of the elbows:

a peculiar localized variant of polymorphous light eruption.

J Am Acad Dermatol. 2013; 68:306-12.

Hjorth N, Sjolin KE, Sylvest B, Thomsen K. Acne aestivalis

— Mallorca acne. Acta Derm Venereol. 1972; 52:61-3.

Waters AJ, Sandhu DR, Green CM, Ferguson J. Solar capillaritis

as a cause of solar purpura. Clin Exp Dermatol.

; 34:821-4.

Ling TC, Richards HL, Janssens AS, Anastassopoulou L,

Antoniou C, Aubin F, et al. Seasonal and latitudinal impact

of polymorphic light eruption on quality of life. J

Invest Dermatol. 2006; 126:1648-51.

Nyberg F, Hasan T, Puska P, Stephansson E, Häkkinen

M, Ranki A, et al. Occurrence of polymorphous light

eruption in lupus erythematosus. Br J Dermatol. 1997;


Millard TP, Lewis CM, Khamashta MA, Hughes GR, Hawk

JL, McGregor JM. Familial clustering of polymorphic light

eruption in relatives of patients with lupus erythematosus:

evidence of a shared pathogenesis. Br J Dermatol.


Grabczynska SA, McGregor JM, Kondeatis E, Vaughan

RW, Hawk JL. Actinic prurigo and polymorphic light eruption:

common pathogenesis and the importance of HLA-

-DR4/DRB1*0407. Br J Dermatol. 1999; 140:232-6.

Hojyo-Tomoka T, Vega-Memije E, Granados J, Flores

O, Cortés-Franco R, Teixeira F, et al. Actinic prurigo: an

update. Int J Dermatol. 1995; 34:380-4.

Crouch R, Foley P, Baker C. Actinic prurigo: a retrospective

analysis of 21 cases referred to an Australian photobiology

clinic. Australas J Dermatol. 2002; 43:128-32.

Valbuena MC. Actinic prurigo. Dermatol Clin. 2014;


Lane PR, Hogan DJ, Martel MJ, Reeder B, Irvine J. Actinic

prurigo: clinical features and prognosis. J Am Acad Dermatol.

; 26:683-92.

Addo HA. Frain-Bell W. Actinic prurigo - a specific photodermatosis?

Photodermatol. 1984; 1:119-28.

Herrera-Geopfert R, Magaña M. Follicular cheilitis. A distinctive

histopathologic finding in actinic prurigo. Am J

Dermatopathol. 1995; 17:357-61.

Jong CT, Finlay AY, Pearse AD, Kerr AC, Ferguson J, Benton

EC, et al. The quality of life of 790 patients with photodermatoses.

Br J Dermatol. 2008;159:192-7.

Nitiyarom R, Wongpraparut. Hydro vacciniforme and

Solar Urticaria. Dermatol Clin. 2014; 32:425-53.

Trikha S, Turnbull A, Srikantha N, Krishnan R, Tappin A.

Anterior keratouveitis secondary to hydroa vacciniforme:

a role for ophthalmic slit-lamp examination in this condition?

BMJ Case Rep. 2011.

Iwatsuki K, Satoh M, Yamamoto T, Oono T, Morizane S,

Ohtsuka M, et al. Pathogenic link between hydroa vacciniforme

and Epstein-Barr virus-associated hematologic

disorders. Arch Dermatol. 2006; 142:587-95.

Cho KH, Lee SH, Kim CW, Jeon YK, Kwon IH, Cho YJ, et

al. Epstein-Barr virus associated lymphoproliferative lesions

presenting as a hydroa vacciniforme-like eruption:

an analysis of six cases. Br J Dermatol. 2004; 151:372-

Yamamoto T, Hirai Y, Miyake T, Yamasaki O, Morizane S,

Iwatsuki K. Oculomucosal and gastrointestinal involvement

in Epstein-Barr virus- associated hydroa vacciniforme.

Eur J Dermatol. 2012; 22:380-3.

Paek SY, Lim HW. Chronic actinic dermatitis. Dermatol

Clin. 2014; 32:355-61.

Forsyth EL, Millard TP. Diagnosis and pharmacological

treatment of chronic actinic dermatitis in the elderly: an

update. Drugs Aging. 2010; 27:451-6.

Lim HW, Cohen D, Soter NA. Chronic actinic dermatitis:

results of patch and photopatch tests with Compositae,

fragrances, and pesticides. J Am Acad Dermatol. 1998;


Russell SC, Dawe RS, Collins P, Man I, Ferguson J. The

photosensitivity dermatitis and actinic reticuloid syndrome

(chronic actinic dermatitis) occurring in seven

young atopic dermatitis patients. Br J Dermatol. 1998;


Meola T, Sanchez M, Lim HW, Buchness MR, Soter NA.

Chronic actinic dermatitis associated with human immunodeficiency

virus infection. Br J Dermatol. 1997;


Somani VK. Chronic actinic dermatitis — a study of clinical

features. Indian J Dermatol Venereol Leprol. 2005;


Dawe RS, Crombie IK, Ferguson J. The natural history

of chronic actinic dermatitis. Arch Dermatol. 2000;


Norris PG, Morris J, Smith NP, Chu AC, Hawk JL. Chronic

actinic dermatitis: an immunohistologic and photobiologic

study. J Am Acad Dermatol. 1989; 21:966-71.

Agar N, Morris S, Russell-Jones R, Hawk J, Whittaker

S. Case report of four patients with erythrodermic cutaneous

T-cell lymphoma and severe photosensitivity mimicking

chronic actinic dermatitis. Br J Dermatol. 2009;


Beattie PE, Dawe RS, Ibbotson SH, Ferguson J. Characteristics

and prognosis of idiopathic solar urticaria: a cohort

of 87 cases. Arch Dermatol. 2003;139:1149-54.

Horio T. Solar urticaria — idiopathic? Photodermatol

Photoimmunol Photomed. 2003;19:147-54.

Tuchinda C, Leenutaphong V, Sudtim S, Lim HW. Fixed

solar urticaria induced by UVA and visible light: a report

of a case. Photodermatol Photoimmunol Photomed.

; 21:97-9.

Wessendorf U, Hanneken S, Haust M, Neumann NJ.

Fixed solar urticaria with delayed onset. J Am Acad Dermatol.

; 60:695-7.

Kurumaji Y, Shono M. Drug-induced solar urticaria due

to repirinast. Dermatology. 1994; 188:117-21.

Yap LM, Foley PA, Crouch RB, Baker CS. Drug-induced

solar urticaria due to tetracycline. Australas J Dermatol.

; 41:181-4.

Faurschou A. Evidence-Based Guidelines for the Classification

and Management of the Photodermatoses. Section

Photoprotection. Paris: European Dermatology

Forum Guidelines; 2009.

Bissonnette R. Prevention of polymorphous light eruption

and solar urticaria. Skin Ther Lett. 2002; 7:3-5.

Azurdia RM, Pagliaro JA, Diffey BL, Rhodes LE. Sunscreen

application by photosensitive patients is inadequate for

protection. Br J Dermatol. 1999; 140:255-58.

Lademann J, Schanzer S, Jacobi U, Schaefer H, Pflücker

F, Driller H, et al. Synergy effects between organic and

inorganic UV filters in sunscreens. J Biomed Opt. 2005;


Bissonnette R, Nigen S, Bolduc C. Influence of the quantity

of sunscreen applied on the ability to protect against

ultraviolet-induced polymorphous light eruption. Photodermatol

Photoimmunol Photomed. 2012; 28:240-3.

Hoffmann K, Laperre J, Avermaete A, Altmeyer P, Gambichler

T. Defined UV protection by apparel textiles. Arch

Dermatol. 2001; 137:1089-94.

Gambichler T, Avermaete A, Bader A, Altmeyer P, Hoffmann

K. Ultraviolet protection by summer textiles.

Ultraviolet transmission measurements verified by determination

of the minimal erythema dose with solar-simulated

radiation. Br J Dermatol. 2001; 144:484-89.

Bech-Thomsen N, Ravnborg L, Wulf HC. Quantitative

study of the melanogenic effect of multiple suberythemal

doses of different ultraviolet radiation sources. Photodermatol

Photoimmunol Photomed. 1994; 10:53-6.

Hönigsmann H. Mechanism of phototherapy and photochemotherapy

for photodermatoses. Dermatol Ther.

; 16:23-7.

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.

; 116:531-8.

George SA, Gibbs NK, Aitchison T, Johnson BE, Ferguson

JA. Comparison of narrow-band phototherapy

(Tl-01) and photochemotherapy (PUVA) in the management

of polymorphic light eruption. Br Dermatol. 1993;


SCENIHR (Scientific Committee on Emerging and Newly

Identified Health Risks). Health effects of artificial light.

Brussels: European Comission; 2012.

Moise AF, Aynsley R. Ambient ultraviolet radiation levels

in public shade settings. Int J Biometeorol. 1999;


Murphy GM, Hawk JL, Magnus IA. Hydroxychloroquine

in polymorphic light eruption: a controlled trial with drug

and visual sensitivity monitoring. Br J Dermatol. 1987;


Marini A, Jaenicke T, Grether-Beck S, Le Floc'h C, Cheniti

A, Piccardi N, et al. Prevention of polymorphic light

eruption by oral administration of a nutritional supplement

containing lycopene, ss-carotene and Lactobacillus

johnsonii: results from a randomized, placebo-controlled,

double-blinded study. Photodermatol Photoimmunol

Photomed. 2013; 30:189-94.

Caccialanza M, Percivalle S, Piccinno R, Brambilla R. Photoprotective

activity of oral polypodium leucotomos extract

in 25 patients with idiopathic photodermatoses. Photodermatol

Photoimmunol Photomed. 2007; 23:46-7.

Tanew A, Radakovic S, Gonzalez S, Venturini M, Calzavara-

Pinton P. Oral administration of a hydrophilic extract

of Polypodium leucotomos for the prevention of polymorphic

light eruption. J Am Acad Dermatol. 2012; 66:58-

60. Kohli I, Shafi R, Isedeth P, Griffith JL, Al-Jamal MS,

Silpa-Archa N, et al. The impact of oral Polypodium leucotomos

extract on ultraviolet B response: a human clinical

study. J Am Acad Dermatol. 2017; 77:33-41.

Dam TN, Moller B, Hindkjaer J, Kragballe K. The vitamin

D3 analog calcipotriol suppresses the number and

antigen-presenting function of Langerhans cells in normal

human skin. J Investig Dermatol Symp Proc. 1996;


Yarosh DB, O’Connor A, Alas L, Potten C, Wolf P. Photoprotection

by topical DNA repair enzymes: molecular

correlates of clinical studies. Photochem Photobiol.

; 69:136-40.

Yarosh D, Klein J, O’Connor A, Hawk J, Rafal E, Wolf P.

Effect of topically applied T4 endonuclease V in liposomes

on skin cancer in xeroderma pigmentosum: a randomized

study. Xeroderma Pigmentosum Study Group.

Lancet. 2001; 357:926-9.

Fabrikant J, Touloei K, Brown SM. A review and update

on melanocyte stimulating hormone therapy: afamelanotide.

J Drugs Dermatol. 2013; 12:775-9.

Millard TP. Treatment of polymorphic light eruption. J

Dermatol Treat. 2000; 11:195-9.

Molin L, Volden G. Treatment of polymorphous light

eruption with PUVA and prednisolone. Photodermatol.

; 4:107-8.

Norris PG, Hawk JL. Successful treatment of severe polymorphous

light eruption with azathioprine. Arch Dermatol.

; 125:1377-9.

McCoombes JA, Hirst LW, Green WR. Use of topical

cyclosporin for conjunctival manifestations of actinic prurigo.

Am J Ophthalmol. 2000; 130:830-1.

Ferguson J, Ibbotson S. The idiopathic photodermatoses.

Semin Cutan Med Surg. 1999; 18:257-73.

Jacobson J. Thalidomide: a remarkable comeback. Expert

Opin Pharmacother. 2000;1:849-63.

Sampaio E, Sarno E, Galilly R, Cohn ZA, Kaplan G. Thalidomide

selectively inhibits tumor necrosis alpha production

by stimulated human monocytes. J Exp Med.


Torres-Alvarez B, Castanedo-Cazares JP, Moncada B.

Pentoxifylline in the treatment of actinic prurigo. A preliminary

report of 10 patients. Dermatology. 2004;


Hawk J. Evidence-Based Guidelines for the Classification

and Management of the Photodermatoses. Section I. The

Auto-Immune Photodermatoses. Paris: European Dermatology

Forum Guidelines; 2009.

Suga Y, Hashimoto Y, Matsuba S, Mizoguchi M, Ogawa

H. Topical tacrolimus for chronic actinic dermatitis. J Am

Acad Dermatol. 2002; 46:321-3.

Gardeazabal J, Arregui MA, Gil N, Landa N, Ratón JA,

Diáz-Pérez JL. Successful treatment of musk ketone-induced

chronic actinic dermatitis with cyclosporine and

PUVA. J Am Acad Dermatol. 1992; 27:838-42.

Murphy GM, Maurice PD, Norris PG, Morris RW, Hawk

JL. Azathioprine treatment in chronic actinic dermatitis: a

double-blind controlled trial with monitoring of exposure

to ultraviolet radiation. Br J Dermatol. 1989; 121:639-46.

Thomson MA, Stewart DG, Lewis HM. Chronic actinic

dermatitis treated with mycophenolate mofetil. Br J Dermatol.

; 152:784-6.

Adamski H, Bedane C, Bonnevalle A, Thomas P, Peyron

JL, Rouchouse B, et al. Solar urticaria treated with intravenous

immunoglobulins. J Am Acad Dermatol. 2011;


Waibel KH, Reese DA, Hamilton RG, Devillez RL. Partial

improvement of solar urticaria after omalizumab. J Allergy

Clin Immunol. 2010; 125:490-1.

Haylett AK, Nie Z, Brownrigg M, Taylor R, Rhodes LE.

Systemic photoprotection in solar urticaria with alphamelanocyte-

stimulating hormone analogue [Nle4- D-

-Phe7]-alpha-MSH. Br J Dermatol 2011; 164:407-14.

Collins P, Ahamat R, Green C, Ferguson J. Plasma exchange

therapy for solar urticaria. Br J Dermatol.1996;


How to Cite
Ramos Pinheiro, R., Coelho de Sousa, V., Rocha Páris, F., Apetato, M., & Marques Pinto, G. (2018). Autoimmune Photodermatoses Part II - Clinical Manifestations and Treatment. Journal of the Portuguese Society of Dermatology and Venereology, 75(4), 371-382.
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