Psoriasis is a chronic, inflammatory, Prescribing in children

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1 Psoriasis in children: current approaches to management Laura Proudfoot BSc, MRCP, Elisabeth Higgins MA, FRCP and Judy Davids RGN Our series Prescribing in children gives practical advice for successful management of childhood problems in general practice. Here, the authors describe the diagnosis and treatment options for psoriasis. Figure 1. Psoriasis presenting in an infant as nappy dermatitis; at this early stage significant scale is often lacking, making a confident diagnosis difficult Psoriasis is a chronic, inflammatory, immune-mediated skin disease with considerable variation in its morphology, severity and duration, affecting 2 per cent of the population in Europe and the USA. Thirty per cent of patients describe disease onset prior to the age of 16, 10 per cent before the age of 10 and up to 2 per cent in the under twos. 1 However, misclassification and natural reluctance to perform potentially diagnostic but also invasive investigations in children may result in underestimation of childhood psoriasis as a whole. There is evidence for a complex, multifactorial, genetic predisposition underlined by twin studies and the increased frequency of certain human leukocyte antigen (HLA) haplotypes. The risk for first-degree relatives of an isolated case is 10 per cent; children of two affected parents have a 50 per cent disease risk. This article describes diagnostic and therapeutic approaches to the management of psoriasis in children. Clinical features of psoriasis in children Psoriasis in children is similar to that seen in adults, although lesions are often less thick and scaly and pruritus is more of a feature. In addition, lesions tend to be located more often on the scalp and face and in the flexures. 2 A pattern of nappy rash with sharply demarcated, often glazed erythematous papules and plaques (see Figure 1) is often seen in infants who later present with more typical psoriasis. Significant scale is often lacking due to the moisture present and a confident diagnosis, with differentiation from seborrhoeic dermatitis at this early stage, can be difficult. In older children flexural, guttate and chronic plaque psoriasis are all common presentations. Guttate psoriasis is characterised by small, drop-like erythematosquamous papules and plaques symmetrically and widely distributed and frequently preceded by a streptococcal pharyngitis by two to three Prescriber 5 June

2 weeks. Lesions generally persist for several months and then resolve spontaneously, but many experience a subsequent recurrence. Flexural psoriasis occurs at the intertriginous areas with fissuring and scant scale. Chronic plaque lesions consist of sharply demarcated, erythematous plaques covered with a silvery scale, most commonly affecting the extensor sites, scalp, umbilicus and lumbar area (see Figures 2 and 3). Generalised and localised pustular psoriasis and erythrodermic varieties are less commonly encountered in the paediatric population but are described. Severe nail changes are relatively uncommon in children and most often seen as onycholysis, pitting and discoloration in longstanding psoriasis. Nail bed involvement is usually absent or mild. However, isolated single-nail disease can occur in young children (see Figure 4) and may present a diagnostic conundrum. Table 1 outlines the differential diagnosis of psoriasis in childhood. Management General principles Childhood psoriasis is a therapeutic challenge. Treatment regimens largely follow those used in adults. However, comparative trials or guidelines are lacking in this age group and regimens are frequently a result of extrapolation from adult studies, adapted according to age, sites affected and accessibility of treatment. For purposes of treatment planning, patients may be grouped into mild-moderate, ie <10 per cent body surface area (BSA) affected (where the palm approximates to 1 per cent BSA), and moderatesevere, ie >10 per cent BSA categories. Mild-moderate disease generally requires only topical agents, whereas moderate-severe Figure 2. A psoriatic plaque on the extensor aspect of a child s arm; topical steroids are useful in flexural sites and can be used in combination preparations disease may warrant systemic treatments along with adjuvant topical management, as attempts to treat extensive disease with only topical agents are often impractical and met with failure and frustration. The chronic, recurrent nature of the skin disease, the physical appearance of the lesions and treatment regimens can all be associated with marked psychosocial and physical morbidity. 3 Compliance in small children is parent dependent and can be enhanced by detailed early education. As children get older and take more responsibility for the application of their treatments, school support will be needed, and the emotional problems that can emerge in some patients and families must be addressed along with dermatological care. Dermatological nursing input can play a vital role; organisations such as the UK Psoriasis Association are excellent resources and referral to liaison psychiatric services may be warranted. Eliminating trigger factors Infections have long been recognised as a key trigger for psoriasis. Precipitation of psoriasis, especially of the guttate type, by group A betahaemolytic streptococcal pharyngitis or perianal infections is well documented, particularly in children and young adults where swabs of these sites should be obtained. A 2000 Cochrane review undertaken to assess the evidence for the effectiveness of antistreptococcal interventions in guttate and plaque psoriasis 4 found no conclusive evidence that the use of antibiotics or tonsillectomy were beneficial; however, the authors would advocate consideration of such interventions if there was evidence of recurrent streptococcal infection. Physical trauma to the skin commonly results in a psoriatic lesion developing at the site of injury (Koebner phenomenon), and in children a patch may appear at an immunisation site. Drug-induced flares are incompletely understood but the most relevant in children include the use of antimalarials and antiepileptic medications and following systemic steroids, with withdrawal resulting in a rebound psoriatic flare. Topical therapy Local topical agents are often sufficient to control disease. Emollients, corticosteroids, dithranol, coal tar preparations and calcipotriol Prescriber 5 June

3 Figure 3. Infections can precipitate chronic plaque psoriasis in children; however, a Cochrane review found no conclusive evidence of benefit with antibiotics or tonsillectomy provide the mainstay of treatment, chosen according to age, morphology and extent of disease, sites affected and practical considerations. 5 Skin hydration and emollients are valuable, inexpensive adjuncts to psoriasis treatments, having an antiproliferative effect and helping to minimise pruritus and irritation. Petroleum jelly ointments or thick creams should be applied immediately after a bath/shower and throughout the day. These are also useful for softening the thick adherent scale of scalp psoriasis prior to, and often in combination with, salicylic acid as a keratolytic. Availability, acceptance and ease of application mean that topical steroids are often the first treatment prescribed for paediatric chronic plaque psoriasis and a number of different vehicles are available to choose from. They can be used as a combination preparation to decrease the irritability of other treatments such as calcipotriol and are useful in flexural sites and on the face and hairline. Mild or moderately potent steroids with a twice-daily application regimen are preferred, although some may require intermittent or rotational therapy with potent corticosteroids, particularly in stubborn areas such as the scalp. Side-effects such as skin atrophy and striae must be considered and application should be slowly tapered to avoid a rebound flare. Coal tar is underutilised and useful in all ages in scalp, chronic plaque and guttate types and, if sufficiently dilute, is less irritating than calcipotriol and dithranol. Preparations containing up to 6 per cent coal tar can be used in children aged one month to two years, and 10 per cent preparations in children over two years. Shampoos, creams and compounded ointments are available and can be used once daily; however, coal tar is not suitable for use in the scrotal area or on acutely inflamed skin, and the smell, staining and phototoxicity may limit its tolerability, especially in older children. Dithranol reduces cell turnover, and in chronic plaque psoriasis short contact therapy with per cent cream applied for minutes in the evenings is safe and effective, 6 although not suitable for use on the face or in the flexures. The dithranol concentration is increased every three days to maintain slight irritation of the skin. This regimen avoids the need for treatments during school hours and the associated staining of clothes, but parents may need to be instructed in the technique by a specialist dermatology nurse before feeling confident to undertake this at home. Calcipotriol is a well-established therapy for mild-moderate plaque psoriasis affecting <30 per cent BSA. Its effectiveness and safety have been evaluated in children, 7 with local skin reactions the most common side-effect. It is often used in sequential and rotational combination with topical steroids but is not suitable for use on the face, scalp or genital areas 38 Prescriber 5 June

4 in children. Calcipotriol 50µg per g applied twice daily is licensed in children to a maximum dose of 50g per week for those aged 6-12 years and 75g per week for those over 12 years. There is very limited evidence for the use of topical tacrolimus (unlicensed use) in childhood psoriasis and it is not used as widely as in eczema. 8 Primary systemic treatments In recalcitrant psoriasis, severe evolving disease and in the rare cases of generalised pustular psoriasis or pustular erythroderma, aggressive treatment with phototherapy or systemic agents may be warranted under specialist supervision. 9 Ciclosporin, methotrexate, retinoids and biological therapies have not been systematically evaluated or licensed for use in childhood psoriasis, but their use has been described and clinical experience allows specialist treatment in certain severe cases. Ciclosporin has been extensively studied in severe childhood atopic eczema, where it has been shown to be safe and well tolerated. But there is limited experience of its use in psoriatic children, where it is reserved for severe recalcitrant cases and for short-term control in difficult situations such as stressrelated flares during school examinations. The recommended initial dose is 3-4mg per kg per day with a maximum treatment period of two years. The major side-effects, nephrotoxicity and hypertension, appear to be dose dependent and reversible after discontinuation of treatment. 10 Methotrexate is widely used to treat adult psoriasis but controlled studies in the paediatric population are lacking. Good clinical response has been obtained in children at doses of mg per kg once per week 11 with frequent monitoring for hepatotoxicity and bone marrow suppression. Acitretin is a retinoid effective in severe psoriasis including pustular and erythrodermic forms and is especially suited for combination therapy. It can be used for intermittent rescue therapy in children but its teratogenicity and the long-term effects on bone growth particularly limit its use in this age group. A baseline skeletal survey followed by repeated radiological monitoring every months has been recommended for children on longerterm retinoids and doses should not exceed 1mg per kg per day. Biological agents Recent advances have allowed the development of biological agents and immune response modifiers targeting key mechanisms of psoriasis pathogenesis. These are licensed in adults for severe psoriasis refractory to at least two systemic agents and phototherapy or if other treatments are not tolerated. Etanercept (Enbrel) is widely used to treat adult psoriasis and is indicated for the treatment of polyarticular juvenile rheumatoid arthritis in children as young as four years. It has been shown to significantly reduce disease severity in children and adolescents with moderate-severe plaque psoriasis 12 and has recently been licensed in children from the age of eight years, under specialist supervision, for severe plaque psoriasis when standard treatment has failed or the patient is intolerant of, or has a contraindication to, these treatments. Phototherapy Ultraviolet light is not commonly utilised in children, but UVB phototherapy is an effective treatment for chronic plaque psoriasis, often in combination with coal tar, and may be one method of obtaining longer-term disease control in Figure 4. Severe nail involvement is relatively uncommon in children and single-nail psoriasis can present a diagnostic conundrum older children who are able to comply. 14 PUVA is not licensed in children but can also be used in older adolescents. Tolerability, cumulative adverse effects and carcinogenicity are concerns, and the Age at onset Infancy/early childhood Older children Nail changes Differential diagnosis irritant nappy rash seborrhoeic dermatitis nummular (atopic) eczema candidosis seborrhoeic dermatitis atopic eczema tinea corporis/capitis pityriasis rubra pilaris pityriasis rosea pityriasis lichenoides chronica tinea unguium 20-nail dystrophy lichen planus Table 1. Differential diagnosis of psoriasis in childhood Prescriber 5 June

5 if confirmation of the diagnosis is needed when a patient is not responding adequately to topical therapies for consideration of systemic therapy widespread and severe disease psoriatic arthritis for collaboration with a rheumatologist for nursing education and/or family support significant psychological impact from the disease Table 2. When to refer a child with psoriasis to a dermatologist educational problems, given the need for multiple treatments each week, may be prohibitive. Conclusion Childhood psoriasis provides a special challenge. Evidence is lacking in this age group and management requires a holistic therapeutic approach. In most cases intermittent combination and rotational topical therapy in the outpatient setting is sufficient to control disease, but some children who worsen with age or fail to respond may require more aggressive therapy under specialist dermatological care (see Table 2). References 1. Farber EM, et al. Cutis 1999;64: Leman J, et al. Paediatr Drugs 2001; 3(9): Fox FE, et al. Dev Neurorehabil 2007;10(2): Owen CM, et al. Br J Dermatol 2000;143(suppl 57): Cordoro KM. Skin Therapy Lett 2008;13(3): Zvulunov A, et al. Int J Dermatol 1994;33: Oranje AP, et al. J Am Acad Dermatol 1997;36: Brune A, et al. Pediatr Dermatol 2007;24(1): Cordoro KM. Skin Therapy Lett 2008;13(4): Pereira TM, et al. J Eur Acad Dermatol Venereol 2006;20: Collin B, et al. Clin Exp Derm 2009;34(3): Paller AS, et al. N Engl J Med 2008;358(3); Enbrel SPC. Enbrel. 14. Jury CS, et al. Clin Exp Dermatol 2006;31: Dr Proudfoot is a specialist registrar in dermatology, Dr Higgins is a consultant dermatologist and Sister Judy Davids is a dermatology specialist nurse in the Department of Dermatology, King s College Hospital NHS Trust, London Prescriber 5 June

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