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Langerhans cell histiocytosis: A great imitator
Kayla St Claire1, Ryan Bunney1, Kurt A Ashack1
1Department of Dermatology, University of Illinois at Chicago College of Medicine, Chicago, Illinois, USA.
Clinics in Dermatology
|June 10, 2020
Summary
Langerhans cell histiocytosis (LCH) is a rare inflammatory neoplasm affecting multiple organs. Diagnosis relies on characteristic histology and immunohistochemistry, distinguishing it from other skin conditions.
Area of Science:
- Dermatology
- Oncology
- Pathology
Background:
- Langerhans cell histiocytosis (LCH) is a rare neoplastic proliferation of Langerhans cells.
- It can affect various organs, including the skin, and may mimic common dermatoses.
Purpose of the Study:
- To highlight LCH as a critical differential diagnosis in treatment-resistant skin conditions.
- To outline diagnostic criteria and differentiate LCH from its clinical mimickers.
Main Methods:
- Review of clinical presentation and diagnostic workup for LCH.
- Histopathological examination including characteristic cellular morphology.
- Immunohistochemical staining for CD1a, S100, and CD207 (langerin).
Main Results:
- LCH presents as a histiocytic infiltrate with specific nuclear and cytoplasmic features.
- Immunohistochemistry confirms LCH diagnosis, identifying CD1a, S100, and CD207 positive cells.
- Electron microscopy showing Birbeck granules is largely replaced by immunohistochemistry.
Conclusions:
- LCH requires consideration in refractory dermatological cases.
- Accurate diagnosis is achieved through a combination of clinical suspicion, biopsy, and specialized stains.
- Timely diagnosis is crucial for appropriate management, especially in cases with systemic involvement.
