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CME ARTICLE
Year : 2019  |  Volume : 20  |  Issue : 3  |  Page : 191-198

Childhood psoriasis: Disease spectrum, comorbidities, and challenges


1 Department of Medicine, Division of Rheumatology, Allergy and Clinical Immunology, School of Medicine, University of California, Davis; VA Medical Center, Sacramento, California, USA
2 Department of Pediatric Dermatology, Institute of Child Health, Kolkata, West Bengal, India

Date of Web Publication28-Jun-2019

Correspondence Address:
Dr. Siba P Raychaudhuri
Department of Medicine and Dermatology, Division of Rheumatology, Allergy and Clinical Immunology, School of Medicine, University of California, Davis, USA. VA Medical Center, 10535 Hospital Way, Bldg#650, Research Service, Mather, Sacramento 95655, California
USA
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijpd.IJPD_111_18

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  Abstract 


Psoriasis is a chronic, inflammatory, papulosquamous disorder with a variable clinical spectrum. Although it affects population of all age groups, the significance and negative impact of childhood psoriasis is often neglected worldwide. In nearly one-third of the psoriatic population, psoriasis appears during the childhood. In this article, we have focused on different issues and challenges faced during the management of childhood psoriasis. In addition to the varied clinical spectrum and associated comorbidities, in this article, we have given emphasis on the following real needs for childhood psoriasis: (i) psychological and social effects on the affected children, (ii) impact on their family members, and (iii) early diagnosis and management of psoriasis on children with psoriasis. We have concluded the article with the “concept of total care,” which is a third dimension for the management of psoriasis.

Keywords: Challenges, childhood, comorbidities, psoriasis, total care


How to cite this article:
Sarkar S, Dhar S, Raychaudhuri SP. Childhood psoriasis: Disease spectrum, comorbidities, and challenges. Indian J Paediatr Dermatol 2019;20:191-8

How to cite this URL:
Sarkar S, Dhar S, Raychaudhuri SP. Childhood psoriasis: Disease spectrum, comorbidities, and challenges. Indian J Paediatr Dermatol [serial online] 2019 [cited 2019 Jul 20];20:191-8. Available from: http://www.ijpd.in/text.asp?2019/20/3/191/261863




  Introduction Top


Psoriasis is a chronic immune-mediated inflammatory disorder affecting the skin, nails, and joints in both children and adults, which has been estimated to affect 1%–3% of the population. Psoriasis begins in childhood in almost one-third of the cases.[1],[2],[3] It tends to have a chronic relapsing and remitting course. It can have a significant impact on the quality of life (QoL) by interfering with self-esteem, familial and societal relationships, and school life.[4],[5] There is a higher prevalence of comorbidities in children with psoriasis as compared to those without psoriasis.[3],[6],[7],[8] As guidelines are lacking and most (systemic) treatments are not approved for use in children, the treatment of pediatric psoriasis remains a challenge.


  Epidemiology Top


Worldwide, the prevalence estimates range from 0.51% to 11.3% of adults and 0% to 1.37% of children.[9] The prevalence of childhood psoriasis in Europe ranges between 0.5% and 2.0%, with a roughly linear increase from birth to adolescence.[2],[3],[10] Childhood psoriasis is more common in girl child. Tollefson et al. showed that the number of children diagnosed with psoriasis had increased over a 30-year period in the USA from 29.6 to 62.7/100,000 patient-years.[11] In an epidemiological study of various dermatoses in schoolchildren aged 6–14 years from North India, Dogra and Kumar reported that the point prevalence of psoriasis was found to be 0.02%.[12] Another study from North India reported that, out of 419 patients of childhood psoriasis, it constituted 0.3% of all the dermatology outpatients and 12.5% of the total psoriasis patients at a tertiary care hospital.[13] Karthikeyan et al. reported that psoriasis comprised 1.4% of all pediatric dermatoses seen in patients <14 years of age at a referral hospital in South India.[14] Manoharan et al. reported that the incidence of childhood psoriasis was observed to be 17.15% in a total of 344 psoriasis patients during 8 months.[15]


  Clinical Types Top


Plaque psoriasis

Plaque psoriasis is the most common type which affects about 35%–70% in children.[16],[17] The typical plaque is a sharply demarcated, red plaque, covered by silvery white scales. In younger children, the lesion can be atypical, smaller, pinker, less demarcated, and less scaly. It affects typical locations of psoriasis such as the elbows, knees, scalp, and umbilicus, but can be generalized and the distribution is often symmetric. The most common sites affected are elbows, knees, scalp, and lower back [Figure 1].[1]
Figure 1: (a) Classical plaque psoriasis. (b) Plaque-type psoriasis affecting the extensor aspect of the elbow

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Guttate psoriasis

Guttate psoriasis was derived from the Latin “gutta” (a drop), meaning “drop-like” appearance of cutaneous lesions. It is acute in onset, often a week after a streptococcal pharyngitis[18] and less often with an association with perianal streptococcal dermatitis.[19],[20] Characterized by round and slightly oval papules and plaques; varying from 2 to 3 mm to 1 cm in diameter.. The small papular lesions have characteristic overlying silvery-white scales and are scattered more or less symmetrically mainly on the trunk, abdomen, and back. The lesions occur less frequently on the face, ears, and scalp, and are unusual on the palms and soles. It usually persists for 3–4 months and resolves spontaneously and may continue for more than a year. Most patients may experience a recurrence within the next 3–5 years after an attack of guttate psoriasis [Figure 2].[21]
Figure 2: Guttate psoriasis: erythematous, scaly, monomorphic lesions of guttate papules

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Pustular psoriasis

Pustular psoriasis is characterized by localized or generalized superficial sterile pustules over an erythematous base. This variant of psoriasis is rare in this age group.[22],[23] In a review of 1262 cases of childhood psoriasis, Morris et al. reported a 0.6% rate of pustular variants.[16] Four clinical patterns of pustular psoriasis have been described in children: generalized pustular psoriasis (Von Zumbusch), annular pustular psoriasis, exanthematic pustular psoriasis, and localized pustular psoriasis.[24],[25],[26] They are not necessarily mutually exclusive and mixed variants are also possible.[24],[25],[26] The Zumbusch pattern is characterized by waves of widespread eruption of sterile pustules, associated with constitutional symptoms such as high fever, malaise, anorexia, and pain. Most patients with this pattern tend to develop psoriasis vulgaris. The annular pattern is a more frequent, subacute eruption and is characterized by erythema and pustules in a circinate pattern. This form can follow or precede the generalized form of pustular psoriasis. The mixed type has both Zumbusch and annular patterns. Various triggering factors have been identified including infection, vaccination, and steroids. Juvenile generalized pustular psoriasis can occur at any age, but the onset of the disease is often during the 1st year of life.[27]

Scalp psoriasis

It is a type of psoriasis which is very common in children. The scalp is the first site to be involved in many children [Figure 3]. The hairline and occipital area of the scalp are often the first sites with typical plaque form. In mild forms, it appears as seborrheic dermatitis, but in severe forms, it can present with pityriasis amiantacea, i.e., thick fixed, silver scales.
Figure 3: Well-demarcated erythematous, scaly plaque affecting the scalp

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Nail psoriasis

Nearly 25-50% of children with psoriasis have associated nail involvement.[3] The most common feature is pitting; other features include discoloration, onycholysis, subungual hyperkeratosis, “oil drop” sign or salmon patch, and transverse lines and ridges.

Inverse psoriasis

This type of psoriasis develops in flexural and intertriginous areas, such as retroauricular, axillae, groin, genital, or perianal areas. Because of moisture, the typical scale is absent in these sites and presents as a glazed erythema leading to absence of Auspitz's sign. [Figure 4].[23]
Figure 4: Inverse psoriasis. (a) Involvement of groin (b) perianal involvement

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Napkin psoriasis

Napkin psoriasis presents as erythematous, bright, and well-demarcated plaques on the skin covered by the diaper. Most of the times, scales may not be visible due to high moisture content in the diaper area. It may be the initial manifestation of psoriasis in young children, with or without psoriasis present elsewhere on the body [Figure 5].[16],[28]
Figure 5: Napkin psoriasis. (a and b) Involvement of the skin covered by diaper

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Facial involvement

Sometimes, it can be the sole manifestation of psoriasis in 4%–5% of children. It presents as erythematous, scaly patches or plaques on the eyebrows, nasolabial folds, perioral skin, or other facial areas.[29]

Psoriatic arthritis

Juvenile psoriatic arthritis is a rare condition in children and affects 0.7%–10% of children with psoriasis.[17],[30] The peak age of onset for arthritis is around puberty. PsA may precede or follow the onset of cutaneous manifestations of psoriasis. Joint involvement is often asymmetric and may be monoarticular or polyarticular. Younger children tend to have dactylitis and small joint involvement, while older children more often have enthesitis and axial joint disease.[31],[32]

Oral psoriasis

Tongue involvement affects 5%–10% of children. In adults, fissured tongue is more frequently seen, but in children, migratory glossitis is mostly observed.

Erythrodermic psoriasis

Erythrodermic psoriasis is very rare in children. It is characterized by widespread cutaneous erythema and associated scale and exfoliation.


  Rarer Presentations In Children Top


Linear, annular, and palmoplantar psoriasis are rare types of psoriasis in children. Some cases of congenital psoriasis have also been reported.[33]


  Differential Diagnosis Top


The differential diagnosis of childhood psoriasis is eenumerated in [Table 1].
Table 1: Common differential diagnosis of psoriasis in the pediatric population

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  Comorbidities Top


Childhood psoriasis is linked to several comorbidities[34] with adult equivalence [Table 2].[35],[36] The comorbidities are divided into metabolic and nonmetabolic comorbidities. The metabolic comorbidities include abdominal obesity, Obese habitus, metabolic syndrome,[37] dyslipidemia, diabetes, and hypertension.[38] The nonmetabolic comorbidities include atopic dermatitis,[39] vitiligo, alopecia areata, lichen planus,[40] celiac disease, rheumatoid arthritis,[38] epilepsy, valvular cardiomyopathy,[37] and ischemic heart disease.[38] Dermatologists should consider the assessment of comorbidities in children with psoriasis, especially when proposing a treatment that can have an impact on these comorbidities, such as corticosteroids (which can induce hyperlipidemia, hyperglycemia, or body weight increment), acitretin (which can induce hyperlipidemia), cyclosporine (which can induce hypertension), or antitumor necrosis factor (TNF)-α (which can induce body weight increment).[41]
Table 2: Comorbidities in pediatric psoriasis and adult psoriasis

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  Quality of Life Top


Psoriasis has a major influence on the children's QoL and is a challenging problem. Kimball et al. have reviewed the associated comorbidities and correlation with the age of onset of psoriasis. This retrospective study demonstrated that children who were diagnosed at early stages in life were more likely to be depressed, and consider psoriasis to be the causal factor for their depression, experience a decline in quality of life, have sleep problems, use recreational drugs, and experience more discrimination in social settings in comparison to others.[8] Manzoni et al. have shown that psoriasis had the most negative impact on one's QoL. A regression analysis was performed using the Infant Dermatitis Quality of Life Index score, which determined that patients with diagnosed psoriasis had 2.7 times more impaired QoL in comparison to the healthy controls.[42] Furthermore, a recent study by Crosta et al.[43] has shown a prevalence of childhood trauma related to psoriasis. It was seen that children with psoriasis experience traumatic events early in their life and are associated with a lower resilience. In this study, it was observed that traumatic experiences and a low resilience adversely affect the QoL of an individual.[43] Psoriasis, not only has a negative impact on QoL of patients, but also negatively impacts the quality of life of their caregivers.


  Psychological Effects Top


Psoriasis is one of the common childhood diseases that causes a lot of psychological stress. Being a disfiguring and debilitating skin disorder, it becomes an independent risk factor for psychiatric comorbidities in children. They have an increased risk of depression and anxiety. Visible lesions may create social discomfort and affects self-esteem. Itching, pain, and appearance of psoriatic lesions affect the daily and social activities, which may in turn cause embarrassment. All these factors result in significant psychiatric burden. About 60% of pediatric psoriasis patients report clinically significant psychiatric symptoms and are likely to receive psychiatric diagnoses.[44] They have approximately 18%–28% greater risk of being diagnosed with psychiatric disorders such as depression or anxiety.[8] Psychiatric conditions developing due to psoriasis lead to the addition of antidepressants and/or anxiolytic medication along with therapies for psoriasis, which further increases the management burden not only for the children but also for the parents. Younger children with psoriasis are more vulnerable to psychosocial problems than adolescents with psoriasis.[45]


  Social Effects Top


As it is a visible skin disease, school, peer groups, and the society come into play. Large-scale studies have shown impairment of QoL in children with psoriasis.[8],[45] Children with psoriasis most often face negative peer and public opinions. They are more likely to be alienated, teased, bullied, and discriminated, which traumatizes them emotionally, leading them to depression and anxiety.[8] Limited involvement in outdoor activities including sports negatively impacts the social well being of these children.


  Impact on Family Members Top


Psoriasis has a negative impact on the quality of life of patients as well as that of the family members. Most parents have fatigue due to sleep disruption, lack of concentration, and neglect personal care. It also affects their emotional well-being. Parents experience considerable amount of stress, frustration, and sadness over the child's illness. Finance is also a matter of concern because of the cost of treatment, travel costs for multiple visits and as well as doctor fees. Care of the psoriatic child entails additional responsibility which allows little time to self care or career enhancement and can compromise the care of the other family members.


  Challenges in Treatment Top


Treatment of psoriasis in pediatric population possesses a major challenge. Factors such as body metabolism, physical development, cutaneous absorption, pharmacodynamics, and pharmacokinetics of drugs, which are different from that of adults, make safe and effective treatment of psoriasis a great challenge. All these factors are well developed in adults, and they have higher tolerability of various treatments than children. Treatment of childhood psoriasis is not only a challenge limited to the child, but also to the parent and the doctor. Proper counseling of the patient and the parent is the first and most effective part of management, explaining to them the nature of the disease, i.e., the chronicity of the disease and the recurring nature, treatment options available, and their pros and cons. This segment will review the challenges encountered and limitations of topical therapy, phototherapy, and systemic therapy.

Challenges in topical therapy

Topical therapy is generally prescribed for mild-to-moderate and some severe cases of psoriasis. The choice of topical therapy should be prescribed taking into consideration the long-term side effects, with particular reference to steroids and topical immunosuppressive agents. Due to the altered ratio of skin surface area to body weight, there is higher penetration capacity.

Keratolytics such as salicylic acid are one of those topical agents used in thick plaque psoriasis capable of reducing scaling. However, it should be used with caution as even small amounts of salicylic acid can lead to central nervous system side effects or renal damage that can be fatal. As there is a potential risk of salicylism resulting from percutaneous salicylate absorption, it is best to use in children >6 years of age and sites limited to scalp, palms, and soles.

Coal tar which has both antipruritic and anti-inflammatory effects is one of those topical agents used alone or with combinations. However, tar causes irritation, also known to induce chromosomal aberration in peripheral lymphocytes, and bring out the release of heat shock protein.[46] It is not preferred to use in children <12 years of age.

Topical corticosteroids (TCs) are the most frequently employed topical agents. It is suitable for treating childhood psoriasis among all age groups (>2 months of age). The major problem faced by the dermatologists with TC use is the rapid recurrence after discontinuation of therapy. Abrupt stoppage of TCs can develop pustular psoriasis. Long-term use of TCs can lead to local infections, skin atrophy, telangiectasia, striae, acneiform eruption, purpura, and contact dermatitis. Rebound and tachyphylaxis should be kept in mind while using topical steroids for a prolonged period. Due to the higher body surface area to body mass ratio compared to adults, systemic side effects of topical applications are more common in children. Even small doses of potent topical steroids can produce systemic side effects such as suppression of hypothalamic–pituitary–adrenal axis, iatrogenic Cushing's syndrome, and growth retardation in children, especially in infants.[47]

Topical Vitamin D analogs such as calcipotriol, calcitriol, maxacalcitol, and tacalcitol have been found useful in treating plaque psoriasis in children, but overuse of topical Vitamin D analogs can lead to hypocalcemia.

Topical calcineurin inhibitors (TCIs) such as tacrolimus and pimecrolimus act as nonsteroidal immunomodulatory drugs, but the use of TCI in children <2 years of age is not approved by the US Food and Drug Administration.

Topical retinoids such as tazarotene are used in treating plaque psoriasis in older children, but skin irritation is the most common side effect, and thus its use is restricted to thicker plaques in the nonintertriginous sites.

Challenges in phototherapy

The three ultraviolet (UV) spectra used in the treatment of psoriasis are UVA 320–400 nm, broadband UVB (BB-UVB, 290–320 nm), and narrowband UVB (NBUVB, 311 ± 2 nm). Choice of phototherapy is appropriate for children with psoriasis involving 15%–20% of body surface area (BSA) having refractory course. However, UVA or psoralens UVA therapy is not advisable below 12 years of age because of gastrointestinal side effects. The most common challenges in phototherapy are requirement of trained and skillful staff in an appropriate environment, with all protection and constant supervision. Another drawback is the patient's poor adherence to phototherapy as one has to come to the phototherapy center twice or thrice a week for prolonged period for therapy. Moreover, the cost of these units limits light-based therapies to very few centers in India. One has to keep in mind that any form of light therapy has to be commenced in children with enough considerations and thoughtfulness of the child being exposed to UV light-containing sunlight for many more years to come in his/her lifetime.

Challenges in systemic therapies

Systemic agents include retinoid, methotrexate, cyclosporine, and biologics. These agents are used only in severe forms of psoriasis or extensive plaque psoriasis with or without impending erythroderma. The indication for systemic therapy should be one or more of the following: involvement of BSA ≥20%; Psoriasis Area and Severity Index ≥10; erythrodermic psoriasis, with or without metabolic complication; generalized pustular psoriasis; psoriatic arthropathy; and localized disease not responding to topical therapy alone or with significant psychological morbidity, putting at risk the routine day-to-day work.

Acitretin (second-generation retinoid) is the most commonly used systemic retinoid in children. Common side effects are xerosis, cheilitis, and epistaxis. Premature closure of epiphysis and diffuse idiopathic skeletal hyperostosis limit the use of retinoids in children.

Methotrexate is the most commonly used antimetabolite agent and systemic drug in children. Although well tolerated, nausea and vomiting are the common side effects. Relative contraindications include obesity, renal dysfunction, hepatitis, cirrhosis and abnormal liver function tests, significant pulmonary disease, blood dyscrasias (such as anemia, leukopenia, and thrombocytopenia), and active and chronic infectious diseases (such as tuberculosis).

Cyclosporine is an effective drug and is used in the situations of crisis, such as in erythrodermic psoriasis. Hypertension, nephrotoxicity, and immunosuppression are the major side effects, and hence the drug is reserved only for severe cases.[48],[49]

Biologics are various protein molecules used in the management of refractory pediatric psoriasis, where conventional systemic agents cannot be used or failed to treat. In 2009, the European Commission approved etanercept for the treatment of children ≥6 years old with chronic severe plaque psoriasis intolerant or resistant to nonbiologic systemic therapies. The most common adverse events reported with etanercept include minor infections such as upper respiratory tract infections and pharyngitis, injection-site reactions, and headaches.[50] Infliximab is associated with a higher risk of infections, tuberculosis reactivation, infusion reactions, and congestive heart failure than other TNF-α inhibitors, and caution is required with regard to the risk of hepatotoxicity based on experience from its administration in adult psoriatic patients,[51] but data evaluating infliximab use in pediatric psoriasis are lacking. In a recent retrospective review on the safety of systemic therapy in pediatric psoriasis by Bronckers et al., 38.7% of patients reported one or more adverse effects with the use of biologic agents. Injection-site reactions were the most commonly reported adverse effect, seen in 18.9%, followed by infections (primarily of airway) in 11.3%. Infection rate was higher for adalimumab (15.8%) than etanercept (8.8%). Only 2.8% of patients reported adverse events requiring discontinuation of therapy.[52] Another safety concern is the increased risk of malignancies including lymphoproliferative disorders which may be especially of interest in children since they may continue to receive biologics through their adolescence into adulthood. The risk of TB reactivation (pulmonary and extrapulmonary) is significant especially in developing countries; the risk–benefit ratio should be individualized when indicated for potentially life-altering or life-threatening situations in children such as severe pustular psoriasis. Parents should be cautioned about the side effects and potential life-threatening complications, which can be associated with the treatment. There are anecdotal case reports of apremilast being used with success in childhood psoriasis. The observed benefits were most likely a direct result of apremilast, as it was used in the absence of any concomitant medications or moisturizers.


  Concept of Total Care Top


Psoriasis is a multisystem disorder. Given the increased prevalence of comorbidities in pediatric patients, it is critical to have a multidisciplinary approach for the treatment of childhood of psoriasis. A treatment regimen should not only include the management of psoriasis but also the comorbidities associated with it. It is understandable for a dermatologist that it is not possible to provide the full spectrum of health care like a primary health-care provider. It needs a multidisciplinary approach and should include the following subspecialists on a case-to-case basis: pediatrics, cardiology, psychiatry, endocrinology, rheumatology, physical therapy, and dietary/lifestyle modification programs. A dermatologist needs to identify the potential comorbidities, work closely with other specialists, and provide appropriate referrals. An ideal approach would be a comprehensive and an effective care as proposed for psoriasis as a “total care program.”[53]

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
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Introduction
Epidemiology
Clinical Types
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