Clinical and Demographic Characteristics of Pediatric Patients Diagnosed with Localized Scleroderma: A Retrospective Analysis

Öz

Objective: Localized scleroderma (LS), which is also called as morphea, is a rare skin disease with unknown etiology. LS is typically characterized by sclerosis in the dermis and the subcutaneous tissue. The number of retrospective studies examining the epidemiological, clinical and laboratory data of patients with juvenile LS in Turkey is very limited. The purpose of this study was to investigate the clinical and demographic characteristics of pediatric patients under the age of 18, who were followed up with a diagnosis LS, also to evaluate and compare these findings with available literature.

Material and Methods: The medical records of 39 patients, who had been clinically and histopathologically diagnosed with LS and followed up in our clinic between 2012-2018, were retrospectively reviewed. Demographic, clinical and laboratory findings, and treatment options of the patients were recorded.

Results: A total of 39 pediatric patients (8 boys, 31 girls, mean age 12.1 years) with LS were enrolled in the present study. The age at disease onset was 8.6 years. The mean duration of the disease was 3.6 years. The most common type was plaque type morphea. In two cases, there was movement restriction in the legs, and lichen sclerosus was concurrently present in another case. 12 patients had antinuclear antibody positivity, while 3 cases had positive Borrelia antibodies.

Conclusion: Morphea has lifelong complications for children. Early diagnosis and monitoring of morphea in the childhood period is important in order to avoid both physical and psychological sequelae that may occur in the future.

Anahtar Kelimeler

• Epidemiology
• Localized scleroderma
• Morphea
• Pediatric patients

Lokalize Skleroderma Tanılı Çocuk Hastaların Klinik ve Demografik Özellikleri

Öz

Amaç: Morfea olarak da bilinen lokalize skleroderma (LS), etyolojisi tam olarak aydınlatılamamış nadir bir deri hastalığıdır. LS tipik olarak dermis ve subkutan dokuda skleroz ile karakterizedir. Türkiye’de juvenil LS hastalarının epidemiyolojik, klinik ve laboratuvar özelliklerinin araştırıldığı retrospektif çalışma sayısı oldukça azdır. Bu çalışmanın amacı LS tanısı ile takip edilen 18 yaş altı pediatrik olgularda klinik ve demografik özelliklerin araştırılması ve bulguların mevcut literatür ile karşılaştırmalı gözden geçirilmesidir.

Gereç ve Yöntemler: 2012-2018 yılları arasında kliniğimizde klinik ve histopatolojik açıdan LS tanısı konulan 39 hastanın medikal kayıtları retrospektif olarak incelendi. Demografik, klinik ve laboratuvar bulguları, ayrıca tedavi modaliteleri kaydedildi.

Bulgular: Çalışmaya 39 LS tanısı olan pediatrik hasta (8 erkek, 31 kız, ortalama yaş 12.1 yıl) dahil edildi. Hastalığın başlangıç yaşı 8.6 yıl olarak bulundu. Ortalama hastalık süresi 3.6 yıldı. En sık görülen tip plak morfeaydı. İki vakada bacaklarda hareket kısıtlılığı varken, bir vakada eş zamanlı liken skleroz mevcuttu. 12 hastada antinükleer antikor pozitifliği varken, üç vakada Borrelia antikorları tespit edildi.

Sonuç: Morfea çocuk olgularda hayat boyu devam eden komplikasyonlara neden olabilir. Ilerleyen yıllarda ortaya çıkabilecek hem fiziksel, hem de psikolojik sekellerin önlenmesi için çocuk yaş grubunda morfeanın erken teşhis ve takibi oldukça önemlidir.

Anahtar Kelimeler

• Epidemiyoloji
• Lokalize skleroderma
• Morfea
• Pediatrik hasta

Kaynakça

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