Short clinical s tu dy lnfanti le psoriasis K E Y WORDS psoriasis, infantile, napkin psoriasis, erythrodennic, pustular lnf antik psoriasis A short clinical study E. Kassay, A. Saringer, E. Torok and Z. Szalai SUMM ARY Psoriasis is a lifelong disease. The signs are exacerbating and waning without any apparent reason. lnfants with psoriasis have high incidence of the disease at 5-13 years of age, but it is also possible that they will be free of symptoms during their entire lite. In the treatment a conservative approach is advo- cated, there are few cases where retinoids are needed. It is important for the parents to be familiar with the nature and management of the disease. We hope that gene therapy will play a positive role in the treatment in near future. Introduction Psoriasis is a chronic disease with accelerated epider- mal turnover ancl epiclermal hyperplasia. It has a preva- lence of 1-3 % in the general population, but represents 4.1 % of ali clermatoses encountered in children younger than 16 years of age (1,2). In about 2-6 % of ali psoriasis cases, the first signs appear before the age of two years (3.4). The pathogenesis of the clisease is multifactorial: multiple genetic and environmental factors play a role in infantile psoriasis. The data on the HLA antigens pre- disposing to psoriasis are somewhat conflicting: I-ILA B-13, B-17, B-27, Bw 57, Cw 2, Cw6 and DR-7 (5,6,7). The triggering factor in infantile psoriasis is usually an infection. Associatecl cliarrhoea, along with the urine in the napkin dermatitis may also contribute to the deve- lopment of the disease . Infections also play a major role in exacerbating the disease (7). The importance of family history in psoriasis bas been stressed years ago (1 ,7). Interestingly, 60 % of our patients as observed last year had a previous infection. Children suffering from psoriasis have higher serum total IgE than the normal population (5). Atopic derma- titis and psoriasis often occur together, but the reports on their concordance are contradictory (8, 9). Clinical manifestations Napkin psoriasis is the most frequent form of pso- riasis in infants. Last year 80% of our patients suffering Acta Dermatoven APA Vol 10, 2001, No 2 - --------- ---------- ---- - - --- --- - - 63 Infantile psoriasis Short clinical study Figure 1 . Napkin psoriasis, beginning lesions in the anal and gluteal area in an infant. Figure 2. Napkin psoriasis, developed lesions in an infant. Figure 3. Napkin psoriasis spreading to the body. Figure 4. Erythrodermic psoriasis in an infant 64 - - - - - - - --------------------- ----Acta Dermatoven APA Vol 10, 2001, No 2 Short clinical study from infantile psoriasis presented the first signs in tbe gluteal region (Figure 1,2). The first lesions appeared in the perianal and perigenital area as sharply demar- cated red, shiny papules. In warm and humid environ- ment (e.g. areas coverecl by napkins) the papules were tbin and not scaly. Later they spread to the whole re- gion, and a confluent, erythematous, not scaling area of the skin was involved (Figure 3). Some weeks later secondaty lesions , scaly white plaques and papules ap- pearecl all over the bocly. Lesions tenclecl to be sym- metrical. Other commonly affected areas included tbe scalp as cradle cap, tbe face, tbe flexural regions (neck folds , antecubital and popliteal creases). Histopathological examination usually reveals the diagnosis of psoriasis. Only a long-term follow up can however confirm whether the patient bas trne psoriasis or a psoriasiform dermatitis. Patients witb infantile pso- riasis have a high incidence of psoriasis at 5-13 years of age (10). Severe erythrodermic/generalized pustular type of psoriasis is rare in infants (Figure 4). Pustular psoria- sis may follow seborrboeic dermatitis. Psoriatic e1ytb- roderma develops from pustular psoriasis or from gen- lnfantile psoriasis applied. As these therapies failecl isotretinon was intro- clucecl at a close of 20 mg/clay. She bas been taking it for two years and she is in a relative goocl ancl stable condition. As we triecl to cliscontinue the isotretinoin, the ectropium worsened. Sunlight was beneficial, while infections worsened tbe course of the clisease. In a few instances the first lesions appearecl on other regions, as recurrent intertrigo, or as a single plaque resembling nummular eczema, but this was ve1y rare. Tbe diagnosis was difficult, because the single lesions may persist for a long tirne. Only a few cases of congenital psoriasis have been publishecl. Differential diagnosis. Napkin psoriasis, cradle cap ancl wiclespreacl lesions must be differentiated from candicla infection, sebor- rhoeic or nummular eczema. Psoriatic erythroderma might resemble Leiner's clisease or non-bullosus ichtyo- siform e1ythroderma (2). eralizecl napkin psoriasis or it can be the initial sign of the disease. Complications are hypalbuminemia, sicl- Therapy eropenia, ancl ectropium. Erythroderma may persist for years (2). One of our patients is 22 years old ancl sbe has hacl e1ythroclerma since she was 1.5 year old. Her familiar history is negative. She hacl cl1y skin from birth ancl she had seborrhoeic eczema severa! times. The first pustular psoriasis attack occurred at one and a half-year of age, following an upper respiratory tract infection. Later on the pustular psoriasis evolvecl into psoriatic e1ythroclerma. This condition has persisted for years: occasionally her condition improved, but the girl has never been free of symptoms. During this time she has receivecl clifferent treatments. Systemic preclnisolon, metbotrexat, etretinate, PUVA ancl plasmapheresis were 'Rl J" F· E Jl E 'N1 ('' ]"' s . . ~ : ~ . __ \ ~ _l ' __i . ~ l Topical therapy. Moisturizers ancl emollients are helpful in preventing attacks. Napkin psoriasis and sec- oncla1y widespread lesions usually responded well to the steroid creams or /ancl antifungals. The lesions dis- appeared in 2-3 months. Milcl keratolytics are the first choice for the craclle cap. Coal tar was well toleratecl. Systemic therapy. The actual infection must be treatecl. In severe erythrodermic/pustular psoriasis retinoicls (0.01-0.1 mg/kg) are recommendecl during a fewweeks but sometimes they neecl to be administratecl for years. Sicle effects are rare. l. Farber EM, Mullen RH, Jacobs AH, et al. Infantile psoriasis: a follow up study. Ped Derm 1986; 33: 237-43. 2. Beylot C, Puissant A, Boulac P, at al. Particular clinical features of psoriasis in infants and children. Acta Derm Venereol Supp (Stockh) 1979; 87: 95-97. 3. Neville EA, Finn OA. Psoriasiform napkin dermatitis - a follow up study. Br J Dermatol 1976; 92: 279- 2~. . 4. Meszaros Cs, Debreczeni M, Ladanyi E, Szilagyi E. Gyermekkori psoriasis sajatossagai. B6rgy6gy es Venereol Szemle, 1990; 66: 15-19. 5. Beylot C, Boisseau-Garsand AM, Beylot-Barry M, Doutre MS. Psoriasis before two years of age. Vol of Abstr 8t1, Internati ona! congress of Ped Derm. Paris. 1998. Acta Dermatoven APA Vol 10, 2001, No 2 --------------------- -------------- -- 65 Infimtile psoriasis AUTHORS' ADDRESSES Short clinical study 6. Farber EM. }uvenile psoriasis (Early interventions can reduce risks for problems later). Postgraduate Medicine. 1998; 103: 89-99. 7. Braun-Falco O, Plewig G, Wolff HH, Burgdorf WHC. Dermatology, 2nd Compl. revis. Ed., Springer Berlin, 2000; 585-606. 8. Krulig Lat al. Histocompatibility HLA antigens in psoriasis. Arch Dermatol 1975; 111: 875. 9. Nyfors A, Lemholt K. Psoriasis in children. Br J Derm 1975; 92: 437-442. 10. Torčik E, Rutkai K. Gyermekborgy6gyaszat. Budapest. Medicina, 1995; 257. 11. Christophers E, Henseler T. Zum gemeinsamen Auftreten von Neuroderntitis und Psoriasis. Hautartzt 1992; 43: 664. ErzsebetJ(assay, MD, HeimPdl Children's Hospital, Department oj Dermatology, Ulloi ut 86, 1089 Budapest, Hungary Attila Saringer, MD, Heim Pal Children's Hospital, Department oj Internal Medicine, same address Eva Toro k, MD, PhD, projessor qf dermatology, same address Zsuzsanna Szalai, MD,PhD, same address 66 - - ----- - -------------- ------ - - --Acta Dermatoven APA Vol 10, 2001, No 2