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A Metadata Extraction Approach for Clinical Case Reports to Enable Advanced Understanding of Biomedical Concepts
Published on: September 20, 2018
Juvenile xanthogranuloma: retrospective analysis of 44 pediatric cases (single tertiary care center experience)
Selcen Kundak1, Yasemin Çakır2
1Department of Dermatology, Dr. Behcet Uz Children's Research and Training Hospital, Izmir, Turkey.
Background/Objective:
Juvenile xanthogranuloma (JXG) is a rarely encountered skin disorder, which is characterized by the proliferation of non-Langerhans cell histiocytes. As JXG primarily affects infants and young children, this study aims to describe the epidemiologic, clinical, and histopathologic characteristics of 44 children diagnosed with JXG at a tertiary health care center.
Methods:
Fourty-four children with a histopathologic diagnosis of JXG between January 2003 and January 2017 were retrospectively reviewed. Data related to epidemiologic, clinical, and histopathologic features were extracted from hospital records.
Results:
The mean age of the affected patients was 4.6 years old (range: 0-17 years old) at the time of diagnosis. Twenty-five patients (56.8%) were male, and 19 patients were female (43.2%). Thirty-six children (81.8%) had solitary JXG, one of which was a giant congenital JXG; eight children (18.2%) had eruptive JXG. The heterozygote mutation associated with neurofibromatosis 1 gene was detected in one patient who had both eruptive JXG and numerous café-au-lait spots. Another patient with eruptive JXG was identified to have hypercholesterolemia. None of the children with eruptive JXG developed symptoms or signs of extracutaneous involvement during their clinical follow-up.
Conclusion:
Since JXG is rarely encountered, there may be a tendency toward over-treatment, given concerns for extracutaneous involvement. However, our review revealed no instances of extracutaneous involvement.