Langerhans cell histiocytosis

Key points

Most common histiocytic disorder

Characterised by granulomatous lesions formed by langerin-positive cells

Typically affects children aged 6 to 10 years, occasionally seen in young adults

Affects multiple organs: skin, liver, gastrointestinal tract, CNS (including skull and pituitary gland)

Lesions may be asymptomatic or present as palpable, tender masses

Seen as 3 clinical syndromes:

• Eosinophilic Granuloma: Limited to bone or lung, most common form, often involves the skull (punched out osteolytic lesions)

• Hand-Schüller-Christian Disease: Characterised by calvarial lesions, exophthalmos, and diabetes insipidus.

• Letterer-Siwe Disease: Most aggressive form, involving multiple organs

Biopsy is indicated

Management varies between children and adults

Often steroid and chemotherapy based

May occaisonally require resection of lesions

References

Tillotson CV, Anjum F, Patel BC. Langerhans Cell Histiocytosis. [Updated 2024 Apr 18]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2024 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK430885/

Gomez CK, Schiffman SR, Bhatt AA. Radiological review of skull lesions. Insights Imaging. 2018 Oct;9(5):857-882. doi: 10.1007/s13244-018-0643-0. Epub 2018 Sep 19. PMID: 30232767; PMCID: PMC6206383.