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Psoriasis in childhood and adolescence Pediatric psoriasis (Diaper area) • • • Psoriasis vulgaris represents a rare dermatosis in childhood and corresponds to about 4% of all dermatosis observed in patients below the age of 16 years . Psoriasis that starts in childhood has high family incidence. The most common type presented in infants is characterized by welldelimited erythematous plaques involving the genitals, gluteal and peri-umbilical region, which tends to be persistent and resistant to treatment . Facial affection is rare. As time goes by, new erythematous- squamous plaques show up, affecting primarily the trunk and the limbs Relative frequency of clinical types of psoriasis and the clinical presentations of the disease differ among adults and children. Plaque psoriasis is the most frequent clinical variant in children and adolescents (34-84%), apart from the form that affects the diaper region (psoriatic diaper rash) The characteristic topography in children is affection of diaper area (psoriatic diaper rash), which occurs in children up to the age of two years. Differently from diaper dermatitis (contact dermatitis), lesions have clearer and brighter erythema, well-delimited margins, and involve inguinal folds, with variable pruritus. Classically, these signs and symptoms respond poorly to conventional therapeutic approach to diaper dermatitis. After one or two weeks from onset of diaper erythema, some children develop the classical lesions of psoriasis on the face, scalp, trunk and limbs Pediatric psoriasis (Plaque) • Psoriasis is a chronic skin condition that occurs in children and adults. The typical appearance is of red, thickened, scaly patches on the skin (plaques). These plaques can vary in size and distribution from person to person. In some people it may affect small areas of skin while others may have large areas covering their body Pediatric psoriasis ( Plaque) • • • • • • The diagnosis of psoriasis is usually made clinically. This involves a doctor examining the skin and making the diagnosis based on the appearance of the affected areas. The plaques tend to be distributed symmetrically. They favour certain sites such as scalp, elbows and knees; or; skin folds such as behind ears, armpits and groin. They are well circumscribed, red and scaly. There is often a family history of psoriasis. Occasionally, a skin biopsy may be necessary to distinguish psoriasis from other skin conditions that may appear similar. Plaque type psoriasis Clinical Science Jan 01, 2011,120 • • • Clinical presentation of severe plaque psoriasis (A) and histochemical staining of a plaque biopsy (B) (A) Clinical presentation of a patient with severe plaque psoriasis, the commonest form of psoriasis. Multiple erythematosquamous sharply demarcated plaques are evident on the back and on extensory surface of the upper limbs. Obesity is commonly found in psoriatic patients. (B) Haematoxylin and eosin staining of a psoriasis plaque biopsy in which all key features are present: hyperproliferation and parakeratosis of the epidermis, neutrophil accumulation in stratum corneum, elongation of rete-ridges, vasodilation of venules and capillaries, and intense inflammatory infiltrates in the dermis. Clinical Science Jan 01, 2011,120 • Schematic view of some factors/pathways involved in the formation of psoriatic plaques Both environmental triggers and genetic defects (e.g. LCE3B/LCE3C1), which alter the skin barrier, may contribute to the molecular events that, through the formation of self-DNA/RNA and LL37 complexes, lead to the synthesis of IFN-α by plasmacytoid DCs (pDC) and maturation of myeloid DCs (mDC) into mature DCs. Mature DCs in turn produce multiple cytokines that promote differentiation and expansion of Th1 (i.e. IL-12), Th17 (i.e. IL-6, TGF-β1 and IL-23) and Th22 (i.e. TNF-α and IL-6) cells. Both Th1 and Th17 cytokines induce keratinocytes to produce CCL20, a chemoattractant for CCR6-expressing DCs and T-cells, thus promoting the accumulation of these cells in the psoriatic skin. Th17-related cytokines stimulate DCs and proliferating keratinocytes to make IL-20, a cytokine that promotes keratinocyte proliferation. Keratinocytes produce inflammatory cytokines, such as IL-1β, IL-6 and TNF-α, thus contributing to enhance DC activation and expand the local inflammation. Guttate psoriasis Guttate psoriasis • • • • Guttate psoriasis - is frequent in children and young adults. These lesions are round, up to 3 cm in diameter, and are found in a symmetric distribution on the trunk and proximal extremities. -in a majority of patients, guttate psoriasis appears abruptly 1-3 weeks after an upper respiratory tract infection with Streptococcus. Therefore obtaining a throat or perianal culture for Strep is recommended. Pediatric psoriasis • • • • • • Pediatric psoriasis consists of three age groups of psoriatic patients like @ infantile psoriasis, a self-limited disease of infancy, @ psoriasis with early onset, and @ pediatric psoriasis with psoriatic arthritis. The varied clinical presentations in childhood include plaque-type, guttate, erythrodermic, napkin, and nail-based disease. Like all forms of auto-immunity, susceptibility is likely genetic, but environmental triggers are required to initiate disease activity. The disease in children is more pruritic, common in girls, and the lesions are relatively thinner, softer, and less scaly. Plaque type is the most common form of disease, but certain clinical variants are rare in children like erythroderma, arthropathy, and localized and generalized pustular psoriasis. Psoriasis in children is more frequently precipitated by infections and manifests as acute guttate psoriasis. The co-morbidities of childhood psoriasis include allergic contact dermatitis, eczema, vitiligo and alopecia areata. Psoriasis is sometimes misdiagnosed as dermatitis seborrheica, neurodermatitis and balanitis Infantile psoriasis ( Mis-diagnosed as seborrheic dermatitis) • The congenital form, defined as occurrence of any of the clinical variants of psoriasis at birth or during the first days of life, is extremely rare. It is normally expressed in the forms of plaque psoriasis. Cases of congenital or neonatal erythrodermic psoriasis are rare, severe and demand immediate intervention. Differential diagnosis in these cases include staphylococcal scalded skin syndrome, toxic shock syndrome, candidiasis, congenital ichthyosis, immunodeficiencies, such as Omenn syndrome, metabolic disorders, atopical and seborrheic dermatitis, pityriasis rubra pilaris and generalized mastocytosis Infant showing dry skin with well-defi ned erythematous scaly papules and plaques and the father's hand showing psoriatic plaques • • 2013 : 56 : 1 : 72 Pediatric psoriasis (Different patterns) • • • Guttate psoriasis • • • • • • • Each of the patterns of psoriasis described in adults can be seen in children. These include: Flexural psoriasis (red areas between skin folds) Scalp psoriasis (thick scales found on areas of the scalp) Nail psoriasis (nail dystrophy related to psoriasis) Acute guttate psoriasis (small red plaques occurring after an infection) Chronic plaque psoriasis (red plaques with scaling occurring anywhere on the body) Erythrodermic psoriasis (severe reddening covering most or all of the body) Pustular psoriasis (severe pustules that arise acutely) Photosensitive psoriasis (affecting areas of sun exposure) Guttate, facial and flexural psoria sis are particularly common in children. Childhood psoriasis - Clinical manifestations Ind. j. pediatric Derm. 2012 : 13 : 1 : 3-8 • • • • • • Clinical features are based on age of onset, family history and associated genetic markers. It is classified into two types. Type I psoriasis, with an early onset, positive family history and association with HLA-Cw6, -B57 and DR7. Patients in this category tend to have more severe disease with large body areas involved and frequent recurrences Type II psoriasis, with late onset (after 40 years), negative family history, and association with HLA-Cw2. Congenital and Infantile Psoriasis Psoriasis can rarely present in infancy. In infants, there are 2 patterns of clinical presentation: Psoriatic diaper rashes - Skin lesions in the diaper area has 2 distinctive patterns: Localized psoriatic diaper rash with well demarcated bright red rashes and psoriatic diaper rash with dissemination where the initial presentation is well defined erythematous plaques localized to the diaper area slowly progressing to involve other areas. The differential diagnosis includes seborrheic dermatitis, irritant contact dermatitis and candida albicans infection. More sharply demarcated lesions, positive family history, presence of lesions in other areas and nail involvement favors the diagnosis of psoriasis. Also, regular follow up over a period of time to note the progression helps in making an accurate diagnosis. Psoriatic erythroderma - Psoriasis in infancy can rarely present as a nonspecific erythroderma . Clinically, scalp hyperkeratosis and nail involvement favors the diagnosis of psoriasis. However, other causes of erythroderma have to be differentiated which includes congenital non-bullous ichthyosiform erythroderma, atopic dermatitis, combined variable immunodeficiency and Nethertons syndrome. A skin biopsy may be needed to confirm the diagnosis. Childhood and Adolescence Psoriasis It is similar to adulthood psoriasis in many ways. As in adults, classical plaque psoriasis represents the most frequent clinical form in children. The initial manifestation of psoriasis if often triggered through an infection. The typical presentation in these cases is guttate psoriasis. Plaque Psoriasis Psoriasis in children may present in the typical adult form of the classic plaque type of psoriasis. However, lesions are smaller, scales are often finer in children . Areas of distribution are like in the adult form with a predilection to the extensor surfaces, knees, buttocks, elbows, and scalp. Acute Guttate Psoriasis It is an eruption of small papules in a widespread distribution, often preceded by an intercurrent illness, usually a pharyngitis or tonsillitis due to group A β-hemolytic streptococci. Guttate psoriasis may later evolve into the classic plaque form or completely resolve in 3-4 months after elimination of triggering factors. This form of psoriasis has to be differentiated from pityriasis lichenoides chronic which may require a biopsy in some cases. Micropapular or Follicular psoriasis Small follicular papules of 1 to 2 mm size over the extensor aspects of the limbs are found particularly in the dark-skinned children . Scratching these papules reveals white scale. This form is quiet common and needs to be differentiated from atypical pityriasis rubra pilaris (PRP). Facial Involvement Psoriatic involvement of the face is more common in children than in adults,. Lesions are erythematous, well defined and less itchy than eczematous patches . Presence of lesions under the eye is common and often annular forms exist. Childhood psoriasis - Clinical manifestations (Continue) Ind. j. pediatric Derm. 2012 : 13 : 1 : 3-8 • Scalp Psoriasis The scalp is a common site of disease involvement at the onset and throughout the course of psoriasis. The clinical presentations are highly variable, ranging from mild to severe disease. It is characterized by thick silvery white scale on patches of erythema. It may extend beyond the hairline. Isolated scalp involvement is also common in children. Pityriasis amiantacea is a condition of the scalp characterized by thick yellow-white scales densely coating the scalp and adhering to the scalp hairs as they exit the scalp. They resemble flakes of asbestos and depending on the underlying disorder, the scalp skin may appear normal, or may be deeply erythematous. The condition is associated with hair loss and sometimes it is difficult to comb the hair due to the adherent thick scale at the base of hair shafts. Complications such as secondary bacterial infection can occur and hair loss may be associated with scarring. Tinea capitis is an important differential diagnosis, especially in children. Inverse Flexural Psoriasis Inverse psoriasis is located on genital, perianal, axillary, inguinal, or periumbilical areas. It usually appears as well-demarcated glazed erythematous lesions lacking the psoriatic scale. Local factors such maceration, bacterial or fungal infections may modify or aggravate psoriasis in these locations. Palmoplantar Psoriasis . Presentation varies from thick scaling with fissuring to a glazed erythema. Important differential diagnosis includes PRP, tinea pedis and dyshidrotic eczema. Lack of symptoms, nail changes and well demarcated lesions helps in the diagnosis. PRP can be differentiated on a biopsy. Psoriatic Acral Dermatitis . It is characterized by cutaneous involvement of the digits without the typical nail dystrophy of psoriasis. Features are erythema, scaling and fissures of the distal phalanges associated with shortening of the nail bed. Nail Involvement Psoriatic nail changes have been reported :. Nail pitting , onycholysis , subungual hyperkeratosis , and nail discoloration. It can be an isolated finding or associated with other forms of psoriasis Pustular Psoriasis Pustular psoriasis is rare and occurs more frequently in the adult population. Four forms have been described: Generalized pustular psoriasis , sub acute annular pustular psoriasis , palmoplantar pustular psoriasis and pustular acrodermatitis of Hallopeau. The sub acute annular form is the most common form in children, and is characterized by annular plaques with a pustular margin. Generalized pustular psoriasis of von Zumbusch is a type of acute erythroderma with diffuse pustules all over the body associated with high grade fever and constitutional symptoms. It resolves spontaneously with frequent recurrences as waves of pustules or the more classical plaque forms. It may be triggered by streptococcal infection. Localized pustular psoriasis of the palms and soles (acropustulosis) has been noted to occur rarely in children. • Psoriatic Arthropathy It is a rare condition in children. The peak age of onset is 9-12 years, and there is a slight female predominance. The pattern of arthritis and course is similar to adults. The International League Against Rheumatism has proposed the following diagnostic criteria for psoriatic arthropathy in children: Arthritis and psoriasis or, Arthritis and – Family history of medically confirmed psoriasis in parents or siblings – Dactylitis – Nail abnormalities (pitting or onycholysis) Exclusions include positive rheumatoid test and presence of systemic arthritis. In general, skin lesions precede the onset of arthritis in 80% of cases. In children, psoriatic arthritis is initially oligoarticular involving mainly the proximal and distal interphalangeal joints of the feet and proximal interphalangeal joints of the hands and the knees and ankles. In the late stages, it tends to be polyarticular. Presence of blue discoloration over the affected joints is an important clinical clue to diagnosis. Juvenile idiopathic arthritis is an important differential diagnosis. The course of psoriatic • • • • US Pharm. 2007;32(4):49-55. Pediatric psoriasis (Peringual) • Childhood psoriasis variants include periungual affection with different levels of onycodystrophy present Palmoplantar psoriasis Nail involvement in psoriasis Pediatric psoriasis (Face) • Lesions are characterized by papules and well-delimited erythematous plaques of varied sizes and silver desquamation, frequently organized in a symmetric fashion. In childhood, psoriasis may present atypical characteristics, that is, single or few erythematous plaques that are slightly desquamative, affecting uncommon regions such as the face - including periorbital, perioral and nasal regions many times hindering the correct diagnosis Psoriatic diaper rash with dissemination Ind. j. pediatric Derm. 2012 : 13 : 1 : 3-8 Pediatric psoriasis (Face) Follicular Psoriasis • • What is characteristic in childhood psoriasis is follicular affection with variable pruritus, better detected in limb lesions . There may also be alternate forms that mimic pityriasis alba. Follicular psoriasis Pediatric psoriasis (Extremities) • Psoriasis lesions frequently affect the scalp, followed by extremities and the trunk. There is frequent symmetric distribution of lesions and absence of pruritus . Affection of hands, feet, genitals and flexion areas , including the periumbilical area, is also common among children Thumb sign in psoriatic affection Scalp affection by psoriasis • Scalp affection, with presence of white scales, adhered and thickened placed around hair follicles with mild erythema (pseudotinea amiantacea) may lead to temporary hair loss or even psoriatic alopecia. There may be single plaque lesion or poorly delimited and desquamative lesions, clinically indistinguishable from seborrheic dermatitis Pediatric psoriasis Generalized pustular psoriasis with erythroderma Pediatric psoriasis • Annular pustular psoriasis Although disease develops in most patients between the ages of 15 and 35, in 10% to 15% of patients, findings develop before 10 years of age. Certain aspects of pediatric psoriasis are under-appreciated. These include a predilection for involvement of the diaper area, frequent involvement of the face , the "thumb sign" (erythema and scaling of the dorsal aspects of the thumbs;, alopecia (Figure 3), and mucositis . Traditional therapies for children have been similar to those for adult disease, although safety and efficacy have not been established for any pediatric psoriasis treatment. Options for topical therapy include corticosteroids, calcipotriene, and tazarotene. Systemic alternatives include methotrexate, cyclosporine, and oral retinoids. Phototherapy is not a practical consideration for infants and children. Sequential therapies that have been advocated for significant disease include topical corticosteroids used in conjunction with calcipotriene or tazarotene. The alternation of systemic therapies has also been used in an effort to minimize exposure to any 1 drug, produce synergistic responses, and minimize possible side effects. Pediatric psoriasis • , Pediatric psoriasis -- facial involvement Psoriatic alopecia Childhood psoriasis Childhood psoriasis The immune response and psoriasis Diagnosis of psoriasis • • • Diagnosis of psoriasis is mainly clinical. Through methodic curettage of Brocq, we may find the two typical clinical findings of this dermatosis: sign of stratification of scales and sign of bleeding points or Auspitz sign (small points of bleeding when the scale is removed) . Woronoff hale or ring (perilesional light zone) is highly characteristic of the disease, but it is rarely observed . Isomorphic phenomenon of Köbner manifests the onset of the dermatosis in healthy skin areas after different types of local trauma in patients genetically predisposed and affected by the disease. Psoriasis is characterized by the classical example of Köbner phenomenon, which occurs in 1/3 of the patients with psoriasis. The lesions appear between 10 and 14 days after the trauma. However, the onset of lesions after few days or even years has also been reported. The pathogenesis of this phenomenon remains controversial, focusing mainly on immune and vascular affections. The phenomenon may be evidenced in 50% of the children with psoriasis, and in 39% of the affected adults. Köbner-positive patients may become Köbnernegative and vice-versa, regardless of any therapeutic strategies used . Another phenomenon that was recently described, named Renbök phenomenon, and also called reverse Köbner, expresses the situation in which any local trauma posed on the psoriatic plaque leads to the disappearance of the lesion and replacement by apparently healthy skin on the site. Classically, Köbner-positive patients do not have Renbök phenomenon, because they seem to be mutually exclusive . There is no specific laboratory exam to diagnose psoriasis . Histological presentation is not specific, but it is highly suggestive. The first modifications evidenced are vasodilation and perivascular inflammatory infiltrate. The infiltrate invades the epidermis, in which there is mild spongiosis, invasion of neutrophils and parakeratosis. In a defined lesion there is elongation of regular epithelial cones, with thinning of supra-papillary portion; papillae are enlarged and swollen, showing dilated and tortuous capillaries. In the epidermis, there is parakeratosis, disappearance of granular layer and presence of neutrophil groups (Munro microabscesses). Especially in pustular psoriasis, there may be the presence of cavities containing neutrophils, named spongioform pustules of Kogoj. Inflammatory infiltrate is mild and comprised by mononuclear cells, especially lymphocytes . • Differential diagnosis • to be considered in childhood and adolescence include seborrheic dermatitis, eczemas, superficial mycosis, secondary syphilis, pityriasis rubra pilaris, lichen planus, lupus erythematous, chronic lichenoid pityriasis, ILVEN, enteropathic acrodermatitis, erythrodermic pemphigus foliaceus, drug erythrodermia, Sneddon-Wilkinson subcorneum pustulosis, generalized acute exanthematic pustulosis and impetigo bullous Signs of psoriasis • If the scales are scratched away from the lesion, they fall of as tiny flakes (Candle sign). If the scale is removed totally, a moist, thin, translucent layer of skin is revealed. The lesion remains dry until this last level is reached (sign of the last Häutchen). If scratching is continued bleeding points appear (Auspitz sign). These bleeding points are thought to be the tips of the dermal papillae. • • The early papular lesions start to expand and form different shapes. Some of the patches undergo involution in the center so curved patterns appear. These different types are named as serpiginous, annular, gyrate, guttate (like water drop) or rupial (like oyster shell). Koebner phenomenon Seborrheic dermatitis Eczema of the face Diaper rash Differential diagnosis of diaper dermatitis Differential diagnosis of diaper dermatitis Treatment of psoriasis • Treatment of psoriasis intends to control the disease and improve quality of life of the patients. To determine the best therapeutic regimen, one should consider gender, age, clinical presentation, disease severity, associated signs and symptoms, co-morbidities, concomitant modification, previous treatment, adverse events and participation of parents or guardians in treatment. Initially one should clarify the patients and parents about the characteristics of the treatment and its course, as well as to guide them about the importance of sun exposure. To some patients, psychotherapic follow-up may be necessary . For most pediatric patients, psoriasis may be treated with topical medication. Phototherapy is an option to more extensive and refractory cases. Systemic therapy is reserved to more severe and extensive cases ( that cannot be controlled with topical treatment and/or phototherapy . Therefore, specific therapies depend on form and extension of the disease . • Topical Treatment • As monotherapy or combined regimen, the use of topical medications is normally enough to control mild forms of psoriasis. In the moderate to severe cases, topical treatment, when associated with phototherapy and/or systemic therapy, provides more comfort to patients, speeds up improvement and minimizes pruritus. Emollients and/or humectants (ammonia lactate, Vaseline, ceramides or mineral oil) and, in hyperkeratosic lesions, keratolytic agents (salicylic acid - 3 to 6%, urea - 5 to 20%), should be included in all therapeutic regimen, be it supportive or alternated with active products, or even in asymptomatic stages. The options for topical use are the following described below. Topical corticosteroids: they have antiinflammatory actions, anti-proliferative (anti-mitotic), immunosuppresant, vasoconstrictor and anti-pruriginous action. It is the most widely used topical therapy in cases of childhood psoriasis. Efficacy of response to topical corticosteroids range according to its clinical form, and it is high in inverted psoriasis, moderate in body psoriasis, and mild in palmarplantar and ungual psoriasis. The location of the psoriasis lesion determines the potency of topical corticosteroids to be used owing to the risk of adverse events. Medium and high potency corticosteroids are indicated in scalp, limb and trunk lesions. Less powerful corticosteroids are indicated in lesions located on the face, periauricular regions, folds and genitals. After clinical improvement, we should try to replace them by low potency corticosteroids to avoid development of atrophy, strias, hypertrichosis and inhibition of hypothalamicpituitary-adrenal axis, especially in children. Tachyphylaxis, that is, loss of efficacy as a result of continuous use of the medication and the need to use stronger and stronger drugs to control the disease, is a constant factor in this infirmity. Coaltar (2-10%): the vehicle is Vaseline, cold cream or ointments. When used in isolation, it has moderate action on plaque psoriasis, but when associated with phototherapy, its action is maximized. It may be combined with salicylic acid 2-5% in hyperkeratosic lesions. It represents a very effective and very low cost therapeutic option. On the scalp, it is used as liquor carbonis detergens (coaltar 20% in alcohol 95o, emulsified with quillaja extract, diluted in creams or emulsions), or as shampoo. Folliculitis is the most frequent adverse event in the use of coaltar. Among the inconveniences of its use: low cosmetic acceptance. There is controversy about the carcinogenic potential of coaltar. Despite in vitro studies and animal models clearly showing its carcinogenic potential, epidemiological studies with coaltar in human beings have not demonstrated increase in incidence of neoplasms in the studied group • • • • Topical Treatment OF PSORIASIS • • • • • • Göckerman Method: indicated for disseminated plaque psoriasis, not erythrodermic. It is the association of coaltar with UVB radiation. Coaltar ointment is applied on the patient, and it should remain in place as long as possible. UVB application is made in increasing doses, daily or in alternate days, without removing the ointment. After irradiation, a shower should be taken to remove the scales and reapply the ointment. Total of 20-30 applications until lightening of lesions. Anthralin (or ditranol): It is believed that its effect is cytostatic, reducing the mitotic activity of psoriatic epidermal cells. It may be used in low concentrations (0.1% to 0.5%) during 24h or in high concentrations (1 to 3%) in applications of only 15 to 30 minutes. Prepared as creams, pastes or ointments. Skin lightening takes place 3 to 4 weeks after application. Irritating substance, it should be avoided in intertriginous areas, close to the eyes and mucosa and on healthy perilesional skin, where there may be erosion and blisters. It stains clothes, tiles and the skin around the lesions. There is practically no risk of systemic toxicity, presenting excellent safety profile in children. It is considered highly effective medication for psoriasis, leading to prolonged periods of remission and no tachyphylaxis . Calcipotriol: analog of vitamin D3 that reduces proliferation and induces differentiation of keratinocytes, in addition to modifying immune response. It is safe and, in monotherapy, it had moderate efficacy to treat mild and moderate episodes of psoriasis in adults. When used in combined regimens or in sequence with topical corticosteroids, they offer prolonged periods of remission, without rebound effect that is induced by corticosteroids in monotherapy. It should be applied at night and washed off in the morning. Efficacy and safety of calcipotriol in treatment of pediatric patients are not fully defined yet. In different literature reports, calcipotriol ointment has been proved to be effective, well tolerated and safe in children with psoriasis, and local irritation is the most commonly reported adverse effect . Even though there are no formal guidelines for its use in children, the use of up to 45 g/week/m2 in children does not seem to influence calcium serum levels . It may cause skin irritation, especially on the face, where it should be avoided. In addition to pruritus, erythema and ardor, there may be folliculitis and pigmentation abnormalities on the applied sites. Topical Immunomodulators: pimecrolimus and tacrolimus may be indicated to localized forms on the face, folds and mucosa, because they cause fewer adverse events than corticosteroids and analogs of vitamin D and they have better absorption in these areas. Efficacy is extremely variable. Topical retinoids: The retinoid used in psoriasis patients is tazarotene, available in gel and concentrations at 0.01% and 0.05%. With mild to moderate efficacy, tazarotene is indicated to chronic plaque psoriasis. It is not approved for use in children with psoriasis, but there is indication for its use in acne for children older than 12 years. It may cause irritation, burning sensation and local PHOTOTHERAPY & SYSTEMIC TREATMENT • PHOTOTHERAPY & SYSTEMIC TREATMENT • PUVA, broad band UVB (290-320 nm) and narrow band UVB (311 nm) phototherapy. It is a therapeutic option used in isolation or combined with other therapeutic modalities, either topical or systemic. The action mechanism of phototherapy is through anti-proliferative, antiinflammatory and immunosuppresant activity. Different forms of psoriasis may be treated with this method, but the best indication is to moderate psoriasis, with predominance of fine plaques. Patients with pustular or erythrodermic psoriasis should not be submitted to phototherapy and even sun exposure, owing to the risk of worsening and vasodilation. In children, treatment should be reserved to those that can understand and accept this therapeutic modality. The necessary frequency for satisfactory treatment is three times a week. UVB radiation is highly effective also for the treatment of plaque and guttate psoriasis. It is used in isolation or associated with the use of coaltar (Göckerman method). The most common adverse effect is burns, and it has low risk for skin cancer. Contraindications to the method are photosensitivity and melanoma history. Protection glasses should be worn during the exposure. The anti-psoriatic effect is greater when using the 311nm range, which allows less time of exposure to narrow band. Satisfactory outcomes can normally be seen after 8 weeks of treatment. PUVA method tends to be more effective and quick in inducing improvement when compared to UVB. Associated with 8-MOP systemic, topical or added by sun exposure. Systemic medication should be taken every two hours before light exposure and it has the disadvantage of requiring eye protection for 24 hours. The advocated dose is 20mg below 50Kg of weight, 30mg between 51-65Kg, 40mg between 66-80Kg and 50mg over 80kg. There are no studies showing safety of oral PUVA in children below the age of 8 years, but the method may be used in adolescents . Antibiotics: Even though there are evidences that antibiotic therapy modifies the natural progression of guttate psoriasis triggered by infection, children with this form of the disease and documented streptococcus infection should receive penicillin or erythromycin for seven to 14 days . • • • • • • Methotrexate (amethopterin): Antagonist of folic acid, with which it has structural similarity. It may be administered by oral, intramuscular or intravenous route, and it is essentially excreted by renal route. Bioavailability of the medication reduces with the intake of some foods, especially dairy foods; however, the drug does not have to be taken in fast. Methotrexate should be used in extensive and resistant cases of psoriasis in childhood, or in cases of arthropathic, erythordermic and generalized pustular psoriasis. The used dose for pediatric cases is 0.2-0.4 mg/kg/week, up to the weekly total dose of 12.5 to 20mg. It may be associated with folic acid (1 to 5 mg per os/ day). It has quick action. Recently, low doses of methotrexate have been associated with use of biologicals, especially infliximab, based on its inhibitory action in the production of antibodies , . Hematological controls and periodical liver and renal function tests are mandatory. Clinically, one of the early signs of intolerance is the onset of aphthoid lesions on the oral mucosa, signaling significant leucopenia. The most frequent adverse effect is gastric intolerance. It has multiple drug interactions, Absolute contraindications are pregnancy and breastfeeding, liver cirrhosis, active liver infection and liver failure . Live or attenuated virus vaccine should be avoided. Acitretin: derived from vitamin A (retinol), it is employed in the dose 0.5 to 1.0 mg/kg/d. Especially indicated in generalized pustular psoriasis, also used in generalized plaque psoriasis, and erythrodermic psoriasis (improvement is expected within 3-4 months from treatment). It represents a systemic therapeutic option most used in children with disseminated and resistant presentations to topical treatment and phototherapy. Adverse effects include mild cheilitis (dose-dependent), epistaxis, conjunctivitis, paronychia, alopecia, pruritus, dyslipidemia and teratogenia (etretinate persists in the body for two years, and it should be contraindicated in childbearing age women). Prolonged therapy with acitretin should be carefully considered in children because there are reports of premature closure of bone epiphysis, tendon and ligaments calcifications and delay in bon growth. Radiological exams should be performed annually. Efficacy of acitretin tends to be moderate and it is high when associated with phototherapy. Clinical response is delayed. Among absolute contraindications are pregnancy and willingness to get pregnant in near future, liver and renal failure and allergy to paraben contained in capsules . The use of vaccines to the specific age range is not contraindicated. Acitetrin PHOTOTHERAPY & SYSTEMIC TREATMENT • • • Cyclosporine A: acts by inhibiting activated T-CD4 lymphocytes preventing IL-2 release. Even though it has been widely studied in patients with atopical dermatitis, there are no safety and efficacy studies for psoriasis in children. It should be reserved for severe cases, such as erythrodermic psoriasis and in cases rapidly progressive and without response to other therapeutic methods. Cyclosporine dose is 2-5 mg/Kg, daily, for 3-4 months, after which it should be gradually discontinued. Recurrences tend to happen as a result of dose tapering. Adverse effects include nephrotoxicity, hypertension, nausea, paresthesia feelings, gingival hyperplasia, hypertrichosis and increase in risk of neoplasms, but they do not seem to be more frequent in children than in adults with psoriasis. The drug requires renal, hepatic and hematological monitoring every 2-4 weeks. Contraindications to the use of cyclosporine are renal function abnormalities, uncontrolled systemic hypertension, malignancy and breastfeeding. Immunization with live or attenuated virus vaccine should be avoided during the period and between 3-12 months after its completion, depending on the dose . Cyclosporine has multiple drug interactions, but it is one of the few treatments for psoriasis that may be used in pregnant women . Immunobiological agents: Immunobiological or simply biological agents represent a group of drugs that interfere in specific and timely manner over the immune system. They act by blocking or stimulating one or more immune response pathways. Despite their high complexity and structural variability, all biological agents represent proteins obtained from modern biotechnology techniques. The goal of these therapeutic agents includes the traffic of lymphocytes in skin microcirculation, antigenic presentation of antigen-presenting cells to lymphocytes and, finally, different cytokines They are extremely expensive drugs. To present, etanercept is the biological agent most carefully studied to be used in children with psoriasis. It represents the soluble form of totally human tumor necrosis factor (TNF) receptor . The drug was approved for the first time in 1998 for moderate to severe rheumatoid arthritis, and later it was approved for treatment of children and adolescents with juvenile rheumatoid arthritis (1999). In 2004, etanercept was approved to treat moderate to severe plaque psoriasis in adults . In a recent study published in the literature, children aged 4 to 17 years with moderate to severe psoriasis responded favorably to the mediation at a dose of 0.8 mg/Kg/week (maximum of 50mg), cutaneous route, within a total of 48 weeks, which included serious adverse events (including pneumonia, gastroenteritis, dehydration and surgical removal of ovarian cyst), which were solved without sequels . PROGNOSIS • PROGNOSIS • Most children present the mild form of psoriasis with favorable response to topical treatment. Even though regression of the picture may be followed by prolonged remission, a chronic and recurrent course is the most common progression. In many cases, there are changes to the psoriasis pattern. Some children get worse as they become older, requiring more aggressive treatments. 56 Patients with guttate psoriasis tend to progress to remission of the disease or even progress to plaque psoriasis . •