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Update on Photodermatoses Frank A Update on Photodermatoses Frank A. Santoro, MD, and Henry W. Lim, MD Interactions with ultraviolet radiation (UVR) and chromophores in the skin happen on a daily basis. Photodermatoses, which are abnormal responses to UV exposure, can be classified into subgroups based on pathogenesis. This review will discuss the clinical features, pathogenesis, photobiologic evaluation, prognosis and therapies of the most common photodermatoses. Semin Cutan Med Surg 30:229-238 © 2011 Elsevier Inc. All rights reserved. KEYWORDS photodermatoses, polymorphous light eruption, chronic actinic dermatitis, solar urticaria, erythropoietic protoporphyria, porphyria cutanea tarda, phototoxicity, photoallergy hotodermatoses is a broad term referring to skin diseases family history, age of onset, seasonal variation, and systemic Pprovoked by exposure to ultraviolet (UV) radiation. It symptoms are important elements to consider. A skin exam- can be classified into 4 groups: immunologically mediated ination in which one focuses on the morphology and distri- photodermatoses, chemical- and drug-induced photosensi- bution, specifically involvement of the head, face, neck, tivity, photoaggravated dermatoses, and inherited disorders arms, legs, and torso, and absence underneath the chin, un- with defective DNA repair or with chromosomal instability derneath the lips, nasolabial folds, and postauricular area can (Table 1).1 In this review we will discuss the clinical features, provide further diagnostic clues. Further investigations, such pathogenesis, photobiologic evaluation, prognosis, and ther- as antinuclear antibody titers, skin biopsies, phototesting, apies of the more commonly encountered photodermatoses: photopatch testing, and porphyrin levels, might support di- polymorphous light eruption, chronic actinic dermatitis, so- agnoses. lar urticarial, phototoxicity, photoallergy, porphyria cutanea tarda, and erythropoietic protoporphyria. (Table 2)2-5 lists the characteristics of less commonly seen immunologically Phototesting and mediated photodermatoses. Photopatch Testing Phototesting and occasionally photopatch testing are impor- Approach to Patient with tant parts of evaluation. Phototesting involves exposing a Suspected Photodermatoses patient’s skin to increasing doses of UVA, UVB, and visible light. An immediate assessment to evaluate for solar urticaria A detailed history, including the relationship between erup- and a delayed assessment at 24 hours are performed. The tion and sun exposure, duration of lesions, effect of window minimal erythema dose (MED) for UVA (MED-A) and UVB glass-filtered sunlight, exposure to photosensitizing agents, (MED-B), defined as the lowest dose of radiation that pro- duces perceptible erythema covering the entire irradiated Department of Dermatology, Henry Ford Hospital, Detroit, MI. area, is determined. Phototest responses in common photo- Conflicts of Interest Disclosures: Dr Lim is the President Elect of the American dermatoses are shown in Table 3. Board of Dermatology; has provided consultancy services to LaRoceh Up to 10% of patients with photosensitivity will have a Posay, Proctor & Gamble, and Clinuvel; and has received royalties for 6 Sunscreens & Photoprotection and Photodermatology. Dr Santoro has a positive photopatch test. Photopatch testing involves the pending grant application for an educational project in dermatology application of 2 sets of identical photoallergens. After 24 through Wayne State University and has received Resident funding from hours, one set will be irradiated with UVA (10 J/cm2,or50% Neutrogena to attend the annual American Academy of Dermatology of MED-A if the MED-A is decreased), whereas the other set meeting. serves as a control and remains unexposed. A reading is per- Address reprint requests to Henry W. Lim, MD, Department of Dermatol- ogy, Henry Ford Med Center – New Center One, 3031 West Grand formed at 24 hours and 7 days after irradiation. A photocon- Boulevard, Suite 800, Detroit, MI 48202. E-mail addresses: tact allergic reaction is characterized by a reaction only on the [email protected] (Dr Santoro); and [email protected] (Dr Lim) irradiated site. Positive reactions on both irradiated and unir- 1085-5629/11/$-see front matter © 2011 Elsevier Inc. All rights reserved. 229 doi:10.1016/j.sder.2011.07.007 230 F.A. Santoro and H.W. Lim Table 1 Classification of Photodermatoses* ythematous, pruritic papules, vesicles, pinpoint papules, Immunologically mediated papulovesicles, plaques, and nodules that erupt usually Actinic prurigo within a few hours of sun exposure (Fig. 1). Commonly in- Chronic actinic dermatitis volved sites include the dorsum of the hands, V-area of the Hydroa vacciniforme neck, and sometimes, malar area of the face. Patients may Polymorphous light eruption complain of mild pruritus, whereas some also present with Solar urticaria tenderness of the skin. Systemic symptoms, including chills, Chemical- and drug-induced photosensitivity headache, fever, and nausea, can be observed in some pa- Caused by exogenous agents tients. For an individual patient, the same morphology usu- Photoallergy (topical and systemic) ally is present with each eruption. Pinpoint papules are more Phototoxicity (topical and systemic) 7 Caused by endogenous agents commonly seen in patients with darker skin. In temperate Cutaneous porphyrias climates, the eruption worsens in early spring and improves Pellagra as the sunny season progresses; this is known as “hardening.” Photoaggravated dermatoses Prevalence ranges from 10% to 20%, varying by geo- Acne vulgaris graphic location.8 PMLE is mostly seen in temperate latitudes Atopic dermatitis with initial eruptions observed in the spring. In a cohort of Bullous pemphigoid 138 patients,9 females represented 61.6% of the patients. Carcinoid syndrome Sixty-six percent of patients developed PMLE before 30 years Cutaneous T-cell lymphoma old, and 11% after 50 years of age. Darier’s disease Dermatomyostitis Pathogenesis Disseminated superficial actinic porokeratosis There is increasing evidence to indicate that delayed-type Erythema multiforme hypersensitivity immune reactions to ultraviolet radiation Grover’s disease (UVR)-induced neoantigens of the skin plays a role in the Lichen planus pathogenesis of PMLE.10 It is recognized that UVR induces Lupus erythematosus Pemphigus neoantigens in the skin, although their nature is poorly char- Pityriasis rubra pilaris acterized. UVR also has an immunosuppressive effect in the Psoriasis skin. In patients with PMLE, it has been demonstrated that Reticular erythematous mucinosis they do not have the same degree of immunosuppression Rosacea after exposure to UV, and hence are more likely to react to Seborrheic dermatitis these neoantigens, resulting in the development of skin le- Viral infections sions.11 Proinflammatory markers, such as E-selectin, vascu- Inherited disorders with defective DNA repair or with lar cell adhesion molecule-1, and intercellular adhesion mol- chromosomal instability ecule-1 and cytokines (interluekin-1, interleukin-18, and Ataxia-telangiectasia tumor necrosis factor-alpha) have also been shown to be Bloom syndrome increased in the skin of patients with PMLE.12 Cockayne syndrome Hailey–Hailey disease Phototesting Hartnup disease Although many patients with PMLE have normal MEDs (Ta- Kindler syndrome ble 2), repeated exposure to UVA or UVB (ie, provocation Rothmund–Thomson syndrome Trichothiodystrophy phototesting) can elicit lesions of PMLE in more than 60% of 13 Xeroderma pigmentosum patients. Testing with UVA (320-400 nm) has been more effective than UVB (280-320 nm) and combined UVA/UVB at *Modified from Lim et al.1 provoking disease; the ease of PMLE provocation does not correlate with clinical morphology or disease severity. radiated sites with equal intensity indicate a contact allergy to Prognosis the test substance. Positive reactions on both sites with In a patient self-reported survey of 113 individuals on the 9 greater intensity at the irradiated site indicate both contact and natural course of PMLE, 39% declared sun sensitivity re- photocontact allergy. mained the same; 40%, increased, and 14%, decreased sun sensitivity. A minority of patients had intervals without erup- tions, however, the vast majority of them developed PMLE Immunologically lesions during the follow-up period. Mediated Photodermatoses When PMLE involves the malar area, cutaneous lupus er- ythematosus (LE) needs to be ruled out by appropriate sero- Polymorphous Light Eruption logic testing (antinuclear antibody, anti SSA/Ro antibodies). Clinical Features However, studies following patients with positive antinuclear Polymorphous light eruption (PMLE) is an immunologically antibodies (titer Ͼ 1:80) and PMLE have shown that PMLE mediated photodermatosis that manifests as nonscarring er- does not progress into LE.14 Update on photodermatoses 231 Table 2 Less Commonly Encountered Immunologically Mediated Photodermatoses Photodermatosis Clinical Findings Treatment Actinic prurigo ● Age of presentation: childhood ● Photoprotection ● Symptoms/timing: pruritic sensation a couple of hours ● Symptomatic relief: topical and after sun exposure. Might have chronic pruritus. The systemic corticosteroids, disease improves in the winter in temperate climates antihistamines and shows no variation in tropical climates. ● Beta-carotene, antimalarials ● Mucosal involvement is common with involvement of -low doses of PUVA and NB–UVB the lip in 30%-60% of patients. Acute chelitis has with patient-reported similar exudative,
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