Multisystem Langerhans cell histiocytosis in an adult non‐smoker treated with steroid therapy

Abstract

We describe the case of a 29‐year‐old female non‐smoker who was treated with steroid therapy for a subacute exacerbation of multisystem Langerhans cell histiocytosis (MS‐LCH) with worsening lung, skin, and oral mucosal lesions. The patient developed pneumonia, and computed tomography (CT) showed multiple thin‐walled cavities. Transbronchial lung cryobiopsy (TBLC) specimens revealed Langerhans cells, which were positive for CD1a and S‐100 expression. Similar histological findings were detected in the submandibular gland, skin, and tooth. On the basis of these findings, the patient was diagnosed with MS‐LCH and subsequently treated with steroid therapy. From the literature review, case reports of non‐smokers with pulmonary lesions that worsened and required treatment are rare. Almost all cases recurred and needed additional treatment. This case study contributes to our understanding of the potential role of steroid therapy in MS‐LCH treatment. Additionally, TBLC is a novel, potentially safer, diagnostic tool that has not been previously described for LCH.

Pulmonary Langerhans cell histiocytosis diagnosed using transbronchial lung cryobiopsy: A case report

“…To the best of our knowledge, no study has evaluated the diagnostic yield of TBLC for PLCH. However, few cases in which PLCH has been diagnosed using TBLC have been reported [ 8 , 16 , 17 ]. Furthermore, in a study of 699 cases, in which TBLC was performed to diagnose diffuse lung diseases, seven out of eight cases of PLCH (88%) were diagnosed using TBLC and one case was diagnosed using SLB after TBLC failed to provide a diagnosis [ 18 ].…”