A Comparison of Clinical, Demographic and Treatment Characteristics of Pediatric-Onset and Adult-Onset Patients Diagnosed With Localized Scleroderma

对儿童期发病和成人期发病的局限性硬皮病患者的临床、人口统计学和治疗特征进行比较

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Abstract

INTRODUCTION: Morphea localized scleroderma (LS) is a rare skin disease with unknown pathogenesis, which causes sclerosis of the dermis and subcutaneous tissue. OBJECTIVES: It was aimed to compare the characteristics of patients with pediatric and adult-onset morphea. METHODS: A retrospective analysis was performed on the records of 183 adult morphea patients. The demographics, clinical and laboratory characteristics, and treatment options of the patients were recorded. Adult patients with morphea over the age of 18 were divided into two groups according to the age of onset and compared. RESULTS: Twenty-two percent (N = 41) of the patients had pediatric-onset morphea (POLS) and 77.6% (n=142) had adult-onset morphea (AOLS). While POLS had a higher head-neck involvement, AOLS had a higher breast involvement (P < 0.001 and P = 0.043). Patients with linear morphea were younger, and more frequently had at least one laboratory anomaly (P = 0.016 versus 0.024). Anti-dsDNA positivity and low hemoglobin (Hb) were observed more frequently in patients with breast involvement. Patients with inguinal involvement, on the other hand, had lower Hb and a higher rate of diabetes, and those patients were older (P = 0.042, 0.040, and 0.012, respectively). CONCLUSIONS: Clinical characteristics and accompanying laboratory anomalies of the patients with morphea depend on the age of onset, involvement areas and the types of morphea, having such data readily available should guide the holistic approach for, and the monitoring process of, the disease.

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