1. Clinical Spectrum, Quality of Life, BRAF Mutation Status and Treatment of Skin Involvement in Adult Langerhans Cell Histiocytosis.
- Author
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Crickx E, Bouaziz JD, Lorillon G, de Menthon M, Cordoliani F, Bugnet E, Bagot M, Rybojad M, Mourah S, and Tazi A
- Subjects
- Adult, Aged, Aged, 80 and over, DNA Mutational Analysis, Female, France, Genetic Markers, Genetic Predisposition to Disease, Histiocytosis, Langerhans-Cell enzymology, Histiocytosis, Langerhans-Cell pathology, Humans, Male, Middle Aged, Phenotype, Registries, Retrospective Studies, Severity of Illness Index, Time Factors, Treatment Outcome, Young Adult, Histiocytosis, Langerhans-Cell genetics, Histiocytosis, Langerhans-Cell therapy, Mutation, Proto-Oncogene Proteins B-raf genetics, Quality of Life, Skin pathology
- Abstract
Langerhans cell histiocytosis is a rare histiocytic disorder for which skin involvement and management are poorly described in adults. The aim of this retrospective monocentric study in a national reference centre is to describe the clinical characteristics, quality of life, BRAF mutation status and outcomes of skin involvement in adult patients with Langerhans cell histiocytosis. Twenty-five patients (14 females, mean age 47 years) were included, with a median follow-up of 33 months (range 4-420 months). Patients experienced poor dermatological quality of life despite low body surface involvement. BRAFV600 mutations were detected in 8 of the 18 patients analysed (45%). Eight patients had an associated malignancy. Several treatment options were used and consisted of surgery, topical steroids and carmustine, thalidomide, methotrexate, vinblastine and steroids and cladribine. This study highlights the need to evaluate quality of life and to screen for associated malignancy in adult patients with Langerhans cell histiocytosis.
- Published
- 2017
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