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Home , Hydroa vacciniforme, Photodermatitis, Photodermatosis, Prurigo, Solar urticaria

Ashley Feneran, DO Jenifer Lloyd, DO

University Hospitals Regional Hospitals

AMERICAN OSTEOPATHIC COLLEGE OF DERMATOLOGY Objectives

 Review key points of several photodermatoses  Update knowledge and treatment of photodermatoses  Discuss vitamin D levels in photodermatoses

Types of photodermatoses

 Immunologically mediated disorders  Defective DNA repair disorders  Photoaggravated dermatoses  Chemical- and drug-induced Types of photodermatoses

 Immunologically mediated disorders  Polymorphous light eruption  Actinic  Chronic actinic Polymorphous light eruption (PMLE)

 Most common form of idiopathic  Possibly due to delayed-type reaction to an endogenous cutaneous photo- induced  Presents within minutes to hours of UV exposure and lasts several days Pathology

 Superficial and deep lymphocytic infiltrate  Marked papillary dermal PMLE Treatment

 Topical or oral corticosteroids  High SPF  Restriction of UV exposure  Hardening – natural, NBUVB, PUVA  Antimalarial PMLE updates

 Study suggests topical vitamin D analogue used prophylactically may provide therapeutic benefit in PMLE

Gruber-Wackernagel A, Bambach FJ, Legat A, et al. Br J Dermatol, 2011. PMLE updates

 Study seeks to further elucidate the pathogenesis of PMLE  Found a decrease in Langerhans cells and an increase in density in lesional skin

Wolf P, Gruber-Wackernagel A, Bambach I, et al. Exp Dermatol, 2014.

 Similar to PMLE  Common in native American children  Strong association with HLA DR4 subtype DRB1*0407  Heals with scarring Pathology

 Shallow ulcer, neutrophils, & fibrin deposits  Acanthosis, spongiosis, & superficial vessel telangiectasias  Follicular chelitis Actinic prurigo treatment

 Photoprotection  UV protective film on windows  Topical and oral steroids  NBUVB  Thalidomide  Pentoxifylline  Cyclosporine  Azathioprine  TNF-a inhibitors Actinic prurigo updates

 Study describes successful use of thalidomide in 6 total adult and pediatric patients with actinic prurigo  Regular nerve conduction studies performed

Crouch R, Foley P, Ng J, et al. Australas J Dermatol, 2002. Hydroa vacciniforme (HV)

 Rare, scarring seen in early childhood  Some cases are associated with latent EBV infection  Hemorrhagic bullae heal with varioliform scars Pathology

 Spongiosis & epidermal vesicles  Epidermal and upper dermis necrosis  Lymphocytic infiltrate of dermis Hydroa vacciniforme treatment

 Refractory to treatment  Azathioprine  Close monitoring for atypical  Thalidomide features  Cyclosporine  Photoprotection  Beta carotene  NBUVB  Fish oil supplements  Oral antimalarials Hydroa vacciniforme updates

 Recent case report illustrates adult- onset HV with T-cell monoclonality with a favorable course

Nomura H, Suzuki H, Egami S, et al. Br J Dermatol, 2015. Chronic actinic dermatitis

 Chronic eczema caused by UV radiation and visible light  Tends to affect older men  Likely a delayed-type hypersensitivity reaction  Often a pre-existing allergic or photoallergic Pathology

 Spongiosis, acanthosis, superficial perivascular infiltrate  Occasional apoptotic keratinocytes  Exocytosis of inflammatory cells Chronic actinic dermatitis treatment

 Photoprotection  Topical steroids  Topical calcineurin inhibitors  NBUVB  PUVA  Azathioprine  Cyclosporine  Mycophenolate mofetil Chronic actinic dermatitis updates

 This study disproved the hypothesis that the filaggrin gene plays a strong role in the pathogenesis of chronic actinic dermatitis

Harkins C, Waters A, Kerr A, et al. J Invest Dermatol, 2015. Solar urticaria

 Urticaria induced by sun exposure  Appears within 5-10 minutes of sun exposure & resolve over 1-2 hours  Most cases do not resolve  Risk of anaphylactic-type response Pathology

 Mild perivascular lymphocytic, mast cell, & eosinophilic infiltrate  Mild dermal edema  Can see intraluminal neutrophils and eosinophils Solar urticaria treatment

 Photoprotection  Sunscreen is not particularly helpful for those sensitive to visible light  Anti-histamines  Topical steroids  Graduated UV exposure  IVIG   Plasmapheresis Solar urticaria updates

 This study showed promising results with IVIG to treat solar urticaria, however the duration of response was not prolonged and adverse effects were frequent

Aubin F, Porcher R, Jeanmougin M. J Am Acad Dermatol, 2014. Solar urticaria updates

 Case studies are conflicting on efficacy of omalizumab in solar urticaria Baliu-Piqué C, Aquilera Peiró P. J Eur Acad Dermatol Venereol, 2015. Müller S, Schempp C, Jakob T. J Eur Acad Dermatol Venereol, 2014.

Vitamin D levels

 All of the discussed photodermatoses have the recommendation for photoprotection, but what effect, if any, does this have on vitamin D levels?  This prospective, longitudinal study compared sunlight exposure, photoprotective behavior, and vitamin D levels in patients with photosensitivity vs. healthy adults

Rhodes L, Webb A, Berry J, et al. Br J Dermatol, 2014.  Compared to healthy adults, photosensitive patients had:  Lower weekend UVB doses  Smaller skin area exposure  Greater sunscreen use

Rhodes L, Webb A, Berry J, et al. Br J Dermatol, 2014.  Compared to healthy adults, photosensitive patients had:  Lower vitamin D levels year-round  Year-round lows

Rhodes L, Webb A, Berry J, et al. Br J Dermatol, 2014.  Recommendation is to educate patients on potential for year-round decreased vitamin D levels and supplementation of 400IU daily Program Director: Jenifer Lloyd, DO

PGY-3 PGY-2 PGY-4 Rosanne Paul, DO Mathew Loesch, DO, PhD Aziza Wahby, DO Madeline Tarrillion, DO Miesha Merati, DO

AMERICAN OSTEOPATHIC COLLEGE OF DERMATOLOGY AMERICAN OSTEOPATHIC COLLEGE OF DERMATOLOGY References 1. Aubin F, Porcher R, Jeanmougin M. Severe and refractory solar urticarial treated with intravenous immunoglobulins: A phase II multicenter study. J Am Acad Dermatol. 2014; 71(5): 948-953. 2. Baliu-Piqué C, Aquilera Peiró P. Three cases of solar urticaria successfully treated with omalizumab. J Eur Acad Dermatol Venereol. 2015. Epub ahead of print. 3. Bruderer P, Shahabpour M, Christoffersen S, Andre J, Ledoux M. Hydroa vacciniforme treated by a combination of beta-carotene and canthaxanthin. Dermatology. 1995;190:343-345. 4. Collins P, Ferguson J. Narrow-band UVB (TL-01) phototherapy: an effective preventative treatment for the photodermatoses. Br J Dermatol. 1995;132:956-963. 5. Crouch R, Foley P, Ng J, et al. Thalidomide experience of a major Australian teaching hospital. Australas J Dermatol. 2002; 43(4): 278-284. 6. Durbec F, Regulai Z, Leonard F, Pluot M, Bernard P. Efficacy of v-3 polyunsaturated fatty acids for the treatment of refractory hydroa vacciniforme. Pediatr Dermatol. 2012;29: 118-119. 7. Gruber-Wackernagel A, Bambach I, Legat FJ. Randomized double-blinded placebo-controlled intra-individual trial on topical treatment with a 1,25-dihydroxyvitamin D₃ analogue in polymorphic light eruption. Br J Dermatol 2011; 165(1): 152-63. 8. Hall L, Eminger L, Hesterman K. Epstein-Barr virus: Dermatologic associations and implications: part I. Mucocutaneous manifestations of Epstein-Barr virus and nonmalignant disorders. J Am Acad Dermatol. 2015; 72(1): 1-19. 9. Harkins C, Waters A, Kerr A, et al. Loss-of-function mutations in the gene encoding filaggrin are not strongly associated with chronic actinic dermatitis. J Invest Dermatol. 2015; 135(7): 1919-1921. 10. Kutlubay Z, Sevim A, Engin B, et al. Photodermatoses, including phototoxic and photoallergic reactions (internal and external). Clin Dermatol. 2014; 32: 73-79. 11. Lim H, Snauwaert J. Vitamin D and photodermatoses. Br J Dermatol. 2014; 171(6): 1297-1298. 12. Müller S, Schempp C, Jakob T. Failure of omalizumab in the treatment of solar urticarial. J Eur Acad Dermatol Venereol. 2014. Epub ahead of print. 13. Nitiyarom R, Wongpraparut C. Hydroa vacciniforme and solar urticaria. Dermatol Clin. 2014; 32(3): 345-353. 14. Nomura H, Suzuki H, Egami S, et al. A patient with elderly-onset atypical hydroa vacciniforme with an indolent clinical course. Br J Dermatol. 2015. Epub ahead of print. 15. Rhodes L, Durham B, Fraser W, et al. Dietary fish oil reduces basal and B-generated PGE2 levels in skin and increases the threshold to provocation of polymorphic light eruption. J Invest Dermatol. 1995; 105: 532-535. 16. Rhodes L, Webb A, Berry J, et al. Sunlight exposure behaviour and vitamin D status in photosensitive patients: Longitudinal comparative study with healthy individuals at U.K. latitude. Br J Dermatol. 2014; 171(6): 1478-1486. 17. Rhodes L, White S. Dietary fish oil as a photoprotective agent in hydroa vacciniforme. Br J Dermatol. 1998; 138: 173-178. 18. Valbuena M, Muvdi S, Lim H. Actinic prurigo. Dermatol Clin. 2014; 32(3): 335-344. 19. Wolf P, Gruber-Wackernagel A, Bambach I, et al. Photohardening of polymorphic light eruption patients decreases baseline epidermal Langerhans cell density while increasing mast cell numbers in the papillary dermis. Exp Dermatol. 2014; 23: 424-448.