Journal
JOURNAL OF THE AMERICAN ACADEMY OF DERMATOLOGY
Volume 78, Issue 6, Pages 1047-1056Publisher
MOSBY-ELSEVIER
DOI: 10.1016/j.jaad.2017.05.060
Keywords
BRAF; cladribine; clofarabine; cytarabine; diabetes insipidus; Langerhans cell histiocytosis; steroids; vinblastine
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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.
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