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Cutaneous histiocytoses in children.
Sylvie Fraitag1, Jean-Francois Emile2,3
1Paediatric Dermatopathology Unit, Department of Pathology, Hôpital Necker-Enfants Malades, AP-HP, Paris, France.
Histopathology
|December 27, 2021
Summary
Cutaneous histiocytoses are skin diseases involving abnormal cell buildup. Recent molecular findings are driving a new classification beyond just cell type, impacting diagnosis and treatment.
Area of Science:
- Dermatology
- Pathology
- Immunology
Background:
- Cutaneous histiocytoses are diverse skin diseases characterized by macrophage or dendritic cell accumulation.
- These conditions range from mild, self-resolving skin issues to severe, multi-organ diseases.
- Traditional classification relied on immunophenotypes, distinguishing Langerhans cell histiocytosis (LCH) from non-LCH.
Purpose of the Study:
- To review the evolving classification of cutaneous histiocytoses.
- To highlight the impact of recent molecular pathology findings.
- To emphasize the integration of molecular, clinical, and imaging data.
Main Methods:
- Review of recent pathophysiological and molecular findings in cutaneous histiocytoses.
- Analysis of updated classification criteria.
- Comparison of traditional and novel classification approaches.
Main Results:
- A shift in classification from immunophenotype to molecular alterations, clinical, and imaging characteristics.
- Identification of key molecular drivers in various histiocytoses.
- Recognition of juvenile xanthogranuloma and LCH as frequent pediatric entities.
Conclusions:
- The classification of cutaneous histiocytoses is increasingly based on molecular pathology.
- This new approach refines understanding and potentially improves patient management.
- Accurate classification is crucial for differentiating self-resolving from severe forms.
Keywords:
ALK positive-histiocytosesErdheim-Chester diseaseLangerhans cell histiocytosisRosai-Dorfman-Destombes diseasebenign cephalic histiocytosiscongenital self-healing reticulohistiocytosis (Hashimoto-Pritzker disease)generalised eruptive histiocytosisindeterminate cell histiocytosisjuvenile xanthogranulomamitogen-activated protein kinase pathway activationnon-Langerhans cell histiocytosisprogressive mucinous histiocytosisprogressive nodular histiocytosisxanthoma disseminatumRelated Concept Videos
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