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Hydroa Vacciniforme and Solar Urticaria 351 Hydroa Vacciniforme and CrossMark E'= -~ - at o l 2004 ;43( 12). Solar Urticaria ==_ - ~ 3 1, d om id e inhi bits Iln::~ ct on and an tigen ,'::'0 s J Inves t Derma­ Rattanavala i Nltiyarom, MOd, Chanisada Wongpraparut, MD b,* ;:: - '~a t m e nt of actin ic ~ -=,:) 326- 8. KEYWORDS ~ - a Th alidomide • Hydroa vacciniforme • Epstein-Barr virus • Photosensitivity • Photodermatoses -_a.- :" aching hospi taL • Lymphoproliferative disorder. Solar urticaria • Phototherapy • Plasmaplleresis - :: - 278- 84 _ ,: F a! Thalidomidein ) - ;' : 3 108(4)467- 71 KEY POINTS ' -,: -".. th alidomid e for _ ~"- atol 200 1;42(4) • Hydroa vacciniforme (HV) is characterized by a vesiculopapular eruption and necrotic lesions that heal with varioliform scars. - .~ -- ~ u ez - So t o L Tra­ • HV has been reported to be associated with latent Epstein-Barr virus infection, raising the possibil­ ,:;-- !ah domida. Estu ­ ity of increased risk of Iymphoproliferative malignancy, ~:: Rev Mex 1993 • The mainstay of therapy in HV is adequate photoprotection, • Solar urticaria (SU) is characterized by skin erythema, swelling, and whealing immediately after sun -,-~:;. ' ~ s JP, Moncada8 exposure. • Treatments for SU include photoprotection, medical therapy, phototherapy, photochemotherapy, and plasmapheresis. ,:;- ~en VV R. Use of ,e manifestations - - a eno l 2000;130(6) HYDROA VACCINIFORME eggs," a reference to the vesicular eruption. "Vacci­ =-'~ _ei s A, De-Lu cas­ niforme" means "poxlike" scar, which is character­ Hydroa vacciniforme (HV) is a rare photosensitivity --;:c -, 3 inthe trea tment istic of this condition when the lesions heal, disorder predominantly affecting children , It is - .r - S:cc Esp Oftal mo characterized by recurrent vesiculopapular eruptions that evolve into necrotic crusts and var­ Epidemiology ',~ j", Brigard D, Hu­ ; ::':; ,- c pruri go, J Am ioliform scars on sun-exposed areas, Latent The rarity of HV and lack of universally diagnostic >. - : 10-2, Epstein-Barr virus (EBV) infection has been sug­ criteria make the precise prevalence difficult to ., ical characterlS­ gested to have a role in the underlying pathogen­ establish, The estimated prevalence of HV is 0,34 esis, HV has clinically been classified into classic -:;~ i1 As ians : a case cases per 100,000 population,1 Although well -;;;-; ' 301 Lep rol 2013; HV and severe HV-like eruption , The latter, recognized globally, it predominantly affects Cau­ described in adults, may be associated with sys­ casians,2.3 A bimodal distribution has been temic symptoms and an increased risk of Iympho­ described with peaks presenting at ages 1 to 7 proliferative disorders, Classic HV tends to remit and 12 to 16 years, Several cases of adult-onset by adolescence or early adulthood, This disease classic HV have also been reported.3-5 The pro­ significantly affects quality of life, causing both portion of female to male patients varies depend­ psychosocial and emotional morbidity, ing on the studies, ranging from 1: 1 to 1:2,1-3 Male patients tend to have a later onset, longer History duration, and more severe symptoms than female HV was first reported by Bazin in 1862,1 The term patients, 1 Although HV is usually sporadic, familial "hydroa" possibly derives from the Greek for "water cases have also been noted 6 Discl osure Statement: No conflict of Interest, E " Department of Pediatrics, Faculty of Medicine, Siriraj Hospital, Ma hidol University, 2 Wang lang Road, Bang­ CI ~ koknoi, Bangkok 10700, Thailand, b Department of Dermatology, Faculty of Medici ne, Si riraj Hospital, Mahi­ en .:! dol University, 2 Wanglang Road , Bangkoknoi, Bangkok 10700, Thailand c ~ Corresponding author. c:; E-mail address: [email protected] Q,) ~-: Dermatol Clin 32 (2014) 345-353 ....E http://dx, doi, org/ 10, 1016/j,det,20 14,03,0 13 Q,) 0733 -8635/14/$ - see front matter © 2014 El sevier Inc. All rights reserved, "'C 346 Nitiyarom & Wongpraparut Pathogenesis itching or stinging sensation within hours or days af­ eruptions may 2. .0::_ ter sun exposure. Subsequently, the lesions prog­ as fever, malaise The precise pathophysiology of HV remains un­ ress to ulceration with necrotic crusts, and mosquito bites, I, - :: known. Sunlight, especially ultraviolet A (UVA), is eventually heal over a period of 1 to 6 weeks with splenomegaly, __ _ known to play an important role, because the char­ varioliform atrophic scars (Fig. 2). The distribution abnormal liver 'l_ - ::­ acteristic lesions of HV can be reproduced after 7 10 tends to be symmetric. The eruptions typically thrombocytoper <' artificial UVA exposure - EBV infections may develop on sun-exposed areas, such as the face and colon have 22:: also playa role in the pathogenesis of the disease, and dorsal hands, in the summer. Unlike classic patients with S8. ,,-:: as EBV has been detected in the lymphocytic infil­ HV, the lesions of severe HV-like eruption are larger EBV-associateo -2 trate of HV lesions from both pediatric and adult and deeper; this variant occurs more commonly ir' and/or natural ~ ~ :­ patients. Elevated levels of EBV DNA copies in adults. The lesions are distributed extensively. the peripheral blood and EBV-encoded small nu­ including on sun-protected areas, and may not al­ Histology clear ribonucleic acid (EBER) in cutaneous lesions ways be associated with photosensitivity, 19,2.< have been fou nd in both classic HV and severe HV­ Histology of ea __ 11 13 Facial swelling is also common. Seasonal variatior like eruptions. - Thus, both conditions have is not observed in the severe form. Disfigurement of giosis, focal ke c: - _:: been suggested to be variants within the same dis­ the ears and nose, in addition to contracture of the '/ascular .1 - ease spectrum. Higher copies of EBV are associ­ fingers, has been reported.22 Heat may provoke the Intraepidermal .2 :; ­ ated with symptom severity and worse prognosis symptoms in some of these patients.3 crosis, and u lce~ ' .: for patients. 1<l .1 5 In addition to HV, chronic EBV Ocular and oral mucosal involvement has beer esions. Both c 2SS :: infection is also known to be related to other het­ reported in HV. Ophthalmic complications include share similar s' - ­ erogeneous disorders including Iymphoprolifera­ mild photophobia, chemosis, keratoconjunctivitis. form usually reI, 82 ': :: tive disorders, hemophagocytic syndrome, and comeal ulcer and erosion, iritis, uveitis, and scler­ '.vith few atypica ::: = hypersensitivity to mosquito bites. 16-18 Therefore, itis25; these were observed in 6.3 % of patients in a cutaneous fat .­ patients with the severe variant of HV may be at 25 26 :he infiltrates _~ study from Japan. The same study also re­ risk of progression to various EBV-associated ma­ ported oral lesions, including aphthous stomatitis oressing cytotox :: ~ lignant lymphomas.2,19-2& and ulcerative gingivitis, in 17.5% of the patients. cellular antige - i Oral lesions were seen predominantly in severe mmunofluoresce- === Clinical Manifestations HV-like eruptions rather than in classic HV. Classic HV patients experience various clinical signs and HV spontaneously resolves during adolescence o' symptoms related to their disease. HV is classified young adulthood, whereas severe HV-like erup­ Several condli into two types: classic or typical HV, and severe tions tend to have a relatively longer clinical course 22 :; i erential d l af "- ':~ =­ HV-like eruption.'2 and usually become more severe with age. - 2 and porph r 2 __-J e:ed by their - ~ ~ ~ Mucocutaneous manifestations Systemic manifestations --9 vesicular 'C'- :­ Classic HV usually presents with recurrent erythem­ Classic HV is considered a benign disease wit ' :: solves witho_ - - = atous papules and vesicles (Fig. 1) associated with rare complications. However, severe HV-like ;0 most c or-~ :­ - estizos (indi . :: ~:: :'-'d European :; '-=.1 .:. A) panel anc ", . l ::'_llIous syste ­ -erpes simplex __ ::-. viral culture. 2-_:: '.)ry of exposure :c .:-: _a boratory Fin d ~ __ - '1 8 diagnosis is ~ =-. -gs and pathC£ -::­ ~ _. s picious cases :: .: - "S may be ce o;;. :..crphyria. AUt02"- ' :- : o9ss ed to excluce --:: _::Jus erythema: c 5_~ Fig, 1. A 5-year-old boy with hydroa vacciniforme pre­ Fig , 2. Atrophic poxlike scars on the right cheek of a - ay be checkec ,.- 3= senting with papules, vesicles, and necrotic lesions of 5-year-old boy with hydroa vacc iniforme after - :; e proposed :r:::- -­ the left cheek. (Courtesy of Tor Shwayder, MD, Henry 10 days of treatment. (Courtesy of Tor Shwayder, c: ,tiate the two 2"? Ford Hospita l, Detroit, MI.) MD, Henry Ford Hospita l, Detroit, MI.) -- e T-cell recep!(; - ;7: Hydroa Vaccin iforme and So lar Urticaria 347 '": - --:ours or days aI­ eruptions may have systemic manifestations, such Photobiological Evaluation I me lesions prog­ as fever, malaise, weig ht loss, hypersensitivity to Although most patients show increased sensitivity :: -.:-~ c crusts, and mosquito bites, lymphadenopathy, hepatomegaly, to UVA radiation, they may demonstrate normal . to 6 weeks with splenomegaly, abdominal pain, headache, minimal erythema dose (MED) to UVA. Repetitive 2 . - he distribution abnormal liver function testing, leukopenia, and UVA doses have been found to provoke character­ ?"'~p t ions typicall y thrombocytopenia.,g Erosions in the esophagus istic lesions of HV 9 The action spectrum for induc­ E ~_c h as the face and colon have also been noted.26 Furthermore, tion of skin lesions ranges from 320 to 390 nm.B - ,,' Unlike classic patients with severe disease may progress to :: =ruption are
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