Review article Photodermatoses in children Siti Nurani Fauziah, Wresti Indriatmi, Lili Legiawati Department of Dermatology & Venereology Faculty of Medicine, Universitas Indonesia, dr. Cipto Mangunkusumo Hospital, Jakarta, Indonesia E-mail: [email protected] Abstract Photodermatoses cover the skin’s abnormal reactions to sunlight, usually to its ultraviolet (UV) component or visible light. Etiologically, photodermatoses can be classified into 4 categories: (1) immunologically mediated photodermatoses (idiopathic photodermatoses); (2) drug- or chemical-induced photosensitivity; (3) hereditary photodermatoses; and (4) photoaggravated dermatoses. The incidence of photodermatoses in the pediatric population is much lower than in adults. Polymorphous light eruption (PMLE) is the most common form of photodermatoses in children, followed by erythropoietic protoporphyria. Early diagnosis and investigations should be performed to avoid long-term complications. Photoprotection is the mainstay of photodermatoses management, including use of physical protection and sunscreen. Keywords: children, photodermatoses, photoprotection, polymorphous light eruption. Background usually on the face, neck, extensor forearms, and hands. Early recognition of symptoms and early Photodermatoses is a term used to describe diagnosis is important to prevent complications 1 abnormal reactions of the skin to light, especially due to inadequate photoprotection. to ultraviolet (UV) radiation or visible light.1-3 Incidence of photodermatoses in children is lower This paper will explore the more commonly compared to in adults. Jansen reported 82% out encountered photodermatoses: immunologically- of 95 children with photodermatoses were mediated and drug- or chemical-induced diagnosed with polymorphous light eruption photodermatoses. (PMLE).4 Studies conducted by Horkay et al. and Ten Berge et al. found similar results, where Classification PMLE is the most commonly found Photodermatoses in children comprise several photodermatosis in children.5,6 Photodermatoses groups of diseases, and the classifications induced by systemic or topical agents are rare in available are made to assist clinical evaluation of children, but the number of cases has been photobiological processes.1,3 According to their increasing in the last few years due to the rise in etiology, photodermatoses in children can be photosensitizer use.1 classified as:1 1. Immunologically-mediated According to the data on patient visits in the photodermatoses pediatric dermatology outpatient clinic in dr. Cipto - Polymorphous light eruption (PMLE) Mangunkusumo General Hospital (CMGH), - Juvenile spring eruption (JSE) Jakarta, Indonesia, in the 2014-2015 period there - Actinic prurigo (AP) was 1 patient with endogenous chemical-induced - Solar urticaria (SU) photodermatosis and 176 patients with - Hydroa vacciniforme (HV) photoaggravated dermatoses, out of a total of 1150 patient visits.7 Photodermatoses can be 2. Drug- or chemical-induced suspected if reactions such as sunburn appear on a. Exogenous: the exposed areas shortly after sun exposure, Phototoxicity: systemic and topical J Gen Proced Dermatol Venereol Indones. 2017;2(2):77-88 77 Photoallergy: systemic and topical unclear, immune responses between b. Endogenous: cutaneous porphyrias photoantigens and UV rays are thought to play a role.1,2 3. Hereditary Hereditary photodermatoses includes xeroderma 1. Polymorphous light eruption (PMLE) pigmentosum, Cockayne syndrome, PMLE can be found in children and adults, trichothiodystrophy, Rothmund-Thomson especially in young women. Horkay et al. reported syndrome, Bloom syndrome, Smith-Lemli-Opitz 4% of 398 PMLE cases has the age of onset under syndrome, Hartnup disease, and Kindler 5 years old, and 10% is between the age of 6-10.5 syndrome. In Finland, 25% of all PMLE cases appeared before the age of 15.4 PMLE is the most common 4. Photoaggravated photodermatosis in children.1,4-6 It is more often Photoadggravated photodermatoses includes found in Fitzpatrick type 1 skin.2 Family history of atopic dermatitis (AD), Darier-White disease, PMLE is positive in 3-56% cases.8 dermatitis herpetiformis, herpes simplex infection, lupus erythematosus (LE), dermatomyositis, PMLE is speculated to develop due to UV pellagra, psoriasis, and seborrheic dermatitis. irradiation on the skin’s photoantigens, leading to delayed hypersensitivity responses. The Diagnosis “hardening” phenomenon, where severe clinical Photodermatoses in children are diagnosed from manifestations are found on first exposure and history and physical findings. History includes age milder symptoms will develop on subsequent on onset, climate during eruption, duration exposures.1-3 between sun exposure and lesion onset, duration and evolution of skin lesions, exposure to Clinical manifestations photosensitizers (topical or systemic), Clinical signs of PMLE can be polymorphous, involvement of other organs or systemic which includes papules, papulovesicular involvement, and family history of eruptions, vesicobullous eruptions, plaques, photodermatoses and consanguinity.1 insect bite-like eruptions, urticaria, and lesions similar to erythema multiforme. Usually, PMLE Lesion distribution is a vital clinical feature in eruptions present as confluent erythematous children with suspected photodermatoses; lesions papules forming symmetrical plaques on the face. generally appear in sun-exposed areas, such as Subjective symptoms can include mild pruritus the face, the neck (V area), dorsal forearms, and and a burning sensation. Lesions develop 30 hands. Locations that are relatively lesion-free minutes to 48 hours after sun exposure, and fade also need to be examined, which are the upper in several days. Systemic symptoms are rarely eyelid, behind the ears (Wilkinson triangle), found, but cases with fever, headache, and submental, nasolabial folds, and neck folds. nausea have been reported.1,8-9 Lesion morphology can vary during: 1) the acute phase, which consists of vesicles, papules, Differential diagnosis excoriations, and crusts; 2) the chronic phase Differential diagnosis of PMLE in children include which consists of lichenification. Furthermore, AD, LE, SU, HV, and erythropoietic protoporphyria other forms of skin lesions can be observed in (EPP).1 specific diseases, such as urticaria in solar urticaria, acute phase of erythropoietic Investigations protoporphyria (EPP), and atrophic scars in HV.1 Histopathology Workup includes laboratory examination, skin Edema, focal spongiosis, and vesicles can be biopsy, and phototests. Evaluation of growth and found in the epidermis. Moreover, acanthosis, development, systemic involvement, and focal parakeratosis, and basal vacuolization can neurological deficits are important to help identify be present. Perivascular infiltrate of lymphocytes specific genodermatosis related to (dominant), neutrophils, and eosinophils can be photodermatoses.1 seen.1,2 Immunologically-Mediated Photodermatoses Phototesting Immunologically-mediated photodermatoses, The minimal erythema doses (MEDs) to UVA and previously known as idiopathic photodermatoses UVB in most PMLE patients are normal. or primary photodermatoses, comprise PMLE, Provocative phototesting can be performed to JSE, SU, HV, and AP. The pathophysiology is confirm diagnosis by irradiating the skin with UVA J Gen Proced Dermatol Venereol Indones. 2017;2(2):77-88 78 or UVB, beginning with twice the MEDs, continued JSE management. Oral antihistamines can be with 20-40% dosage increase per day for 3-4 given to manage pruritus, if present.1 days, and the exposed area is observed for 1-2 days for PMLE lesions.1 Phototesting is only 3. Actinic prurigo (AP) useful to differentiate PMLE from other similar AP is most frequently found in Mexico, Central and clinical diseases, because PMLE’s clinical South America, especially in individuals living at manifestations are nonspecific.3 altitudes over 1.000 m above sea level, but cases in Asia and in Caucasians have also been Management reported. The human leukocyte antigen (HLA) is The most effective approach to managing PMLE involved in AP; the subtype HLADRB1*0407 is the is to avoid sun exposure. Topical corticosteroids most prevalent, found in 60-70% of cases. are effective when lesions are newly developed. Generally AP develops in children younger than Systemic corticosteroids can be administered to 10 years old, but can be found in people aged 2- patients with severe and acute PMLE, usually 43 years.14,15 prednisone 0.6-1 mg/kg body weight/day, tapered down according to clinical improvement.10 In Clinical manifestations patients with recurrent PMLE, prophylactic Clinical findings of AP can include highly pruritic phototherapy with narrowband UVB (NB UVB) or papules, plaques, and nodules, together with psoralen with UVA (PUVA) with similar dosing to excoriations and scars in sun exposed areas such phototherapy for psoriasis, three times a week for as the face, neck, extensor forearms, and dorsal 5 weeks, can be considered.9 Other treatments surfaces of the hands. Eczematous lesions and such as antimalaria agents, betacarotene, and lichenification can be seen. Exacerbations usually nicotinamide can help manage PMLE, but their happen during the summer and the spring. The mechanisms of action are unclear.1 lips and conjunctiva may be involved, presenting as cheilitis, conjunctivitis, and pseudopterigium. 2. Juvenile spring eruption (JSE) Cases of alopecia of the eyebrows related to AP Studies of children in New Zealand reported