Review Article Photosensitivity in children: An approach to diagnosis and management Arun C. Inamadar, Aparna Palit Department of Dermatology, Venereology and Leprosy, BLDEA’S SBMP Medical College, Hospital and Research Centre, Bijapur, Karnataka, India Address for correspondence: Dr. Arun C. Inamadar, Department of Dermatology, BLDEA’s SBMP Medical College, Hospital and Research Centre, Bijapur - 586103, Karnataka, India. E-mail: [email protected] ABSTRACT Photosensitivity disorders in children include a wide array of conditions, many of which are unique to this age group. Prompt diagnosis of these disorders becomes difficult at times because of the overlapping clinical pictures. Genodermatoses and metabolic disorders may have associated systemic involvement, which may lead to these children presenting to pediatricians who may overlook the photosensitivity. A dermatologist’s consultation is essential in such cases for specific instructions and counseling of parents regarding photoprotection in these children. This intervention may improve the quality of life by reducing the morbidity and chances of early mortality. This review includes a comprehensive discussion of the distinguishing clinical features of childhood photodermatoses along with general guidelines regarding their investigation and treatment. Key Words: Photosensitivity, Genodermatoses, Idiopathic photodermatoses INTRODUCTION onset, the chronological order of appearance of symptoms associated with clinical examination Photosensitivity is defined as an abnormal response to and investigations are helpful in such cases. “ordinary” light exposure.[1] The prevalent skin types in India are Fitzpatrick skin types IV and V. The HISTORY incidence of photosensitivity disorders in the pediatric age group is much lower than in adults. In contrast to What was the age of onset? adults, the bulk of photosensitive children are included The age of onset of photosensitivity and related skin under the group of metabolic and genetic disorders[1] lesions helps in diagnosing different disorders (Table and pose a diagnostic challenge to the treating 2). Most of the genodermatoses and a few rare types physician. The causes of photosensitivity in children of porphyrias manifest during infancy. Idiopathic are summarized in Table 1. photodermatoses usually affect older children. Solar urticaria (SU) is uncommon in children and affects DIAGNOSTIC APPROACH adolescents or adults. Elicitation of adequate history is vital. A systematic Is there any seasonal variation? approach with a detailed history of the age of Patients with Hartnup disease show an exacerbation How to cite this article: Inamadar AC, Palit A. Photosensitivity in children: An approach to diagnosis and management. Indian J Dermatol Venereol Leprol 2005;71:73-9. Received: June, 2004. Accepted: August, 2004. Source of Support: Nil. Conflict of interest: None declared. 73 Indian J Dermatol Venereol Leprol March-April 2005 Vol 71 Issue 2 Inamadar AC, et al: Photosensitivity in children Table 1: Causes of photosensitivity in children disorders like PLE, SU, actinic prurigo (AP) and HV may Genetic disorders: • Xeroderma pigmentosum also have an intermittent course with complete clearing • Rothmund Thomson syndrome between episodes. A chronic course with acute • Bloom’s syndrome • Cockayne syndrome exacerbations and a tendency for skin changes to • Ataxia telangiectasia persist between exacerbations is suggestive of Metabolic disorders: • Hartnup disease • Phenylketonuria genodermatoses and metabolic disorders. Conditions • Albinism. like collagen vascular disorders are aggravated by • Erythropoietic proto porphyria. • Congenital erythropoietic porphyria exposure to light. In patients with Hartnup disease the • Hepato-erythrocytic porphyria photosensitivity and the dermatitis become milder with Nutritional disorder: • Pellagra [2] [4] Idiopathic, acquired • Polymorphous light eruption age. HV resolves or improves in adolescence. photodermatoses: • Solar urticaria • Actinic prurigo • Hydroa vaccineforme Are any other family members affected? Collagen vascular • Neonatal lupus erythematosus A family history of photosensitivity is available in cases disorders: • Childhood systemic lupus erythematosus • Juvenile dermatomyositis of photodermatoses with a genetic basis. A family Exogenous agents: • Drug-induced photosensitivity history is positive in 50% cases of actinic prurigo.[5] • Phytophotodermatitis Clinical evidence of any collagen vascular disease is seen Table 2: Age of onset of disorders with photosensitivity in nearly 40% of mothers of children with neonatal lupus [6] Age of Disorder with photosensitivity erythematosus (LE). Sixty per cent of them suffer from presentation Sjögren’s syndrome, systemic lupus erythematosus At birth • Neonatal lupus erythematosus (2/3rd cases) (SLE) or rheumatoid arthritis.[7] However, the mother’s • Congenital erythropoietic porphyria and hepatoerythrocytic porphyria (rarely) disease is subclinical in 60% cases at the time of ≤ 1 year • Rothmund Thomson syndrome, Bloom’s childbirth.[6] If such cases are followed up, a majority of syndrome, Cockayne syndrome (3-6 months) • Neonatal lupus erythematosus (1/3rd cases) these mothers develop collagen vascular disorders • Childhood systemic lupus erythematosus (rarely) subsequently.[6,7] Multiple affected family members are • Congenital erythropoietic porphyria and hepatoerythrocytic porphyria seen in albinism and phenylketonuria (PKU). • Phenylketonuria 1-5 years • Ataxia telangiectasia • Hartnup disease (average 3 years of age) Is there any history suggestive of systemic • Erythropoietic protoporphyria (mean age 4 yrs.) involvement? School- • Polymorphous light eruption (commonest going children photodermatosis in children) Idiopathic photodermatoses like PLE, SU, AP and HV • Actinic prurigo (average 9 years) usually present with only cutaneous lesions. • Hydroa vacciniforme (average 6 years of age) • Childhood systemic lupus erythematosus Occasionally, patients with SU may develop systemic • Juvenile dermatomyositis features like headache, nausea, bronchospasm and syncope if exposed inadvertently to sunlight for a of skin lesions in the spring or early summer.[2] Lesions prolonged period.[8] Various acute or chronic systemic of polymorphous light eruption (PLE) and SU also features involving different organ systems accompany aggravate in the spring and early summer but gradually the photosensitivity in genodermatoses, and in improve through the rest of the summer (hardening metabolic and nutritional disorders. effect).[1,3] Actinic prurigo worsens in summer and persists through winter.[1] Hydroa vacciniforme (HV), a Is there any history of exposure to very rare condition, occurs mostly during summer. photosensitizing agents? Exposure to photosensitisers is unusual in very young What is the course of the disease? children. Citrus fruits, mango and extracts of a few An acute single episode disease is indicative of common weeds containing furocoumarins are common idiopathic photodermatoses like PLE, nutritional sensitizing agents in school-going children.[9] Lime juice deficiency disorders like pellagra, and exogenous (which contains ten times the oil of bergamot as other factor-related photodermatoses like drug-induced citrus fruits) is the commonest offending agent.[5] photosensitivity or phytophotodermatitis. Idiopathic Handling flowers of the Compositae group of plants (e.g. Indian J Dermatol Venereol Leprol March-April 2005 Vol 71 Issue 2 74 Inamadar AC, et al: Photosensitivity in children chrysanthemum, marigold, dahlia and sunflower), significant sensitivity to sunlight. Following symptoms which contain oleoresins, may give rise to and signs may be present in several of the disorders photosensitivity. Photoallergic reactions are relatively rare in children.[5] However, several therapeutic agents Butterfly erythema: This is the presenting feature in may give rise to both phototoxic and photoallergic Bloom’s syndrome (BS), Rothmund Thomson syndrome reactions.[5] Topical antimicrobials included in soaps, (RTS), Cockayne syndrome (CS), SLE, pellagra and cosmetics and medicaments like halogenated Hartnup disease (HD). The edema and violaceous salicylanilides, clioquinol and sulfonamide derivatives discoloration involving the periorbital and malar area are common photosensitizers and may go unnoticed. of the face in juvenile diabetes mellitus (DM) is Some systemically administered drugs such as accentuated by sun exposure. In longstanding disease, sulfonamides, nalidixic acid, chlorpromazine, there is a persistent edematous plaque associated with ceftazidime, griseofulvin, ibuprofen, furosemide and scaling in the involved area.[12] dapsone can cause photosensitivity.[5] Blisters: Vesiculo-bullous lesions on photo-exposed CLINICAL FEATURES areas that heal by scarring and pigmentation resembling discoid LE are seen in RTS, BS (Figure 1) and porphyrias. Intolerance to sunlight of varying degrees is the In addition, porphyrias are associated with milia presenting complaint in all the conditions. Infants and formation and mutilation of the affected parts. children with porphyrias experience burning and stinging pain on sun exposure and present with Dermatitis: A sharply demarcated, erythematous, dry, incessant crying even at night.[2] A characteristic feature scaly dermatitis over the face, neck and other photo- of ataxia telangiectasia (AT) is a high sensitivity to exposed areas is seen in pellagra and Hartnup disease[2] ionizing radiation (such