Dendritic cell

Biopsy

Eosinophilic

Histiocytosis

Dermatology

Langerhans cell histiocytosis

Immunology

Immune system

Regimen

Internal medicine

Skin biopsy

Chemotherapy

Medicine

Immunohistochemistry

Vinblastine

Disease

Langerin

Pathology

Journal of the American Academy of Dermatology

A definitive diagnosis of Langerhans cell histiocytosis (LCH) requires a combination of clinical presentation, histology, and immunohistochemistry. The inflammatory infiltrate contains various proportions of LCH cells, the disease hallmark, which are round and have characteristic "coffee-bean" cleaved nuclei and eosinophilic cytoplasm. Positive immunohistochemistry staining for CD1a and CD207 (langerin) are required for a definitive diagnosis. Isolated cutaneous disease should only be treated when symptomatic, because spontaneous resolution is common. Topical steroids are first-line treatment for localized disease of skin and bone. For multifocal single-system or multisystem disease, systemic treatment with steroids and vinblastine for 12 months is the standard first-line regimen. Current research is seeking more effective regimens because recurrence rates, which increase the risk of sequelae, are still high (30-50%) in patients with multisystem disease. An active area of research is the use of targeted therapy directed at the mitogen-activated protein kinase pathway. Adequate follow-up to monitor for disease progression, relapse, and sequelae is recommended in all patients.

Langerhans cell histiocytosis in children