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Juvenile xanthogranuloma with hematologic changes in dizygotic twins: report of two newborn infants
K Kobayashi1, T Imai, S Adachi
1Department of Pediatrics, Kyoto University, Japan.
Insights
This study reports the first cases of juvenile xanthogranuloma (JXG) in non-identical twins, presenting with skin, liver, and blood abnormalities. Symptoms resolved within five months, highlighting the need for careful diagnosis and monitoring.
Area of Science:
- Dermatology
- Pediatrics
- Hematology
Background:
- Juvenile xanthogranuloma (JXG) is a rare histiocytic disorder typically presenting with cutaneous lesions.
- Extracutaneous involvement in JXG can mimic more severe conditions, necessitating accurate diagnosis.
Observation:
- The study details the first documented cases of JXG in dizygotic (non-identical) twins.
- Both twins exhibited characteristic skin lesions, subcutaneous nodules, hepatomegaly, anemia, and thrombocytopenia.
Findings:
- Extracutaneous manifestations, including organomegaly and hematologic abnormalities, resolved spontaneously within five months.
- Resolution of systemic symptoms correlated with the regression of cutaneous JXG lesions.
Implications:
- Accurate diagnosis of JXG requires specific immunohistochemical and ultrastructural analysis to rule out malignancy.
- Comprehensive evaluation for extracutaneous organ involvement and hematologic abnormalities is crucial in managing JXG.
Abstract:
We describe the first reported instances of juvenile xanthogranuloma (JXG) in dizygotic twins. They had characteristic skin lesions and subcutaneous nodules as well as hepatomegaly, anemia, and thrombocytopenia. These extracutaneous symptoms improved in 5 months, coincident with the resolution of the skin lesions. Although most patients with JXG have only cutaneous symptoms, some show such dramatic extracutaneous manifestations that the possibility of malignant disease is occasionally the principle concern. It is therefore necessary to make a precise diagnosis based on specific immunohistochemical and ultrastructural findings, and to evaluate for other organ involvement, including hematologic abnormalities.