Clinical, Demographic and Treatment Characteristics of Pediatric Psoriasis: A Multicenter Study of 150 Patients

BACKGROUND/AIMS: Many studies have focused on the epidemiological features of adult and childhood psoriasis. However, only a few studies have been conducted to demonstrate the clinical and demographic characteristics of pediatric psoriasis in Turkey. This study aimed to determine clinical, demographic, and treatment characteristics of childhood psoriasis in a multicenter series.MATERIALS and METHODS: This study was conducted in four different centers that are located in four cities of Turkey between June 2016 and June 2020. The demographic parameters, possible triggering factors (emotional stress, physical trauma, infection, and medication), and clinical characteristics (psoriasis type, psoriasis area severity index, involved areas, nail involvement, joint involvement, subjective symptoms, disease duration, last treatments and duration of use, and history of accompanying diseases) of pediatric patients with psoriasis were retrospectively analyzed.RESULTS: A total of 150 patients from four different centers were enrolled in the study, of whom 71 (47.30%) were males and 79 (52.70%) were females, with a mean age of 13.71 +/- 42 years (age range: 1-18 years). A family history of psoriasis was determined in 20 (13.33%) patients. Possible triggering factors included emotional stress (n=90, 60%), physical trauma (n=21, 14%), infection (n=14, 9.33%), and medication (n=1, 1.67%). The most common area of involvement was the trunk (n=69, 46%) followed by the scalp (n=42, 28%), hand (n=20, 13.33%), and face (n=19, 12.67%). The prevalence of clinical types was as follows: plaque (n=125,83.33%), guttate (n=10, 6.67%), palmoplantar (n=7, 4.67%), inverse (n=6, 4%), and pustular (n=2, 1.33%) psoriasis. Nail and joint involvement were observed in 30 (20%) and 15 (10%) patients, respectively. The last treatments received included topical treatment (n=101, 67.33%), phototherapy (n=23, 15.33%), acitretin (n=16,10.67%), methotrexate (n=9, 6%), and cyclosporine (n=1, 0.67%).CONCLUSION: In our cohort, the clinical types and treatments used for childhood psoriasis were similar to those of other studies, but the rate of family history was lower, whereas the incidence of emotional stress was higher. Addressing the psychological impacts of psoriasis along with its physical aspects may provide better treatment outcomes

Ichthyosis, follicular atrophoderma, and hypotrichosis caused by mutations in ST14 Is associated with impaired profilaggrin processing

Congenital ichthyosis encompasses a heterogeneous group of disorders of cornification. Isolated forms and syndromic ichthyosis can be differentiated. We have analyzed two consanguineous families from the United Arab Emirates and Turkey with an autosomal recessive syndrome of diffuse congenital ichthyosis, patchy follicular atrophoderma, generalized and diffuse nonscarring hypotrichosis, marked hypohidrosis, and woolly hair (OMIM 602400). By genome-wide analysis, we found a homozygous interval on chromosome 11q24–q25 and obtained a LOD score of 4.0 at D11S910. We identified a homozygous splice-site mutation in the Arab patients and a frame-shift deletion in the Turkish patient in the gene suppression of tumorigenicity-14 (ST14). The product of ST14, matriptase, is a type II transmembrane serine protease synthesized in most human epithelia. Two missense mutations in ST14 were recently described in patients with a phenotype of ichthyosis and hypotrichosis, indicating diverse activities of matriptase in the epidermis and hair follicles. Here we have further demonstrated the loss of matriptase in differentiated patient keratinocytes, reduced proteolytic activation of prostasin, and disturbed processing of profilaggrin. As filaggrin monomers play a pivotal role in epidermal barrier formation, these findings reveal the link between congenital disorders of keratinization and filaggrin processing in the human skin