Oral mucosal involvement in Langerhans’ cell histiocytosis: long‐term follow‐up of a rare case

Abstract

Langerhans' cell histiocytosis (LCH) is a rare disease where different organs and systems may be affected. Oral involvement generally consists of mucosal ulceration associated with lesions of the underlying bone. Many reports exist about the misdiagnosis of this disease. Various symptoms may lead the clinician to an incorrect diagnosis, especially with multiple organ involvement. Oral manifestations are common, and dentists should be aware of this disease and evaluate intraoral findings accordingly. This study presents an LCH case characterized by oral mucosal ulcerations with no involvement of the underlying bone. A definitive diagnosis was made by open biopsy from the oral mucosa.

Langerhans cell histiocytosis: Differences and similarities in long-term outcome of paediatric and adult patients at a single institutional centre

“…LCH affecting mandible and/or soft tissues of the oral region may lead dentists to make the wrong diagnosis. 26 Therefore, attention should also be paid to periodontal damage, which can have a similar clinical presentation as aggressive periodontitis. 27,28 Orbital lesions, accompanied or not by proptosis or exophthalmos, were more common affected bone lesions in our paediatric patients, which is a striking contrast to the adults (P = 0.001).…”

Langerhans Cell Histiocytosis: A Diagnostic Challenge in the Oral Cavity

“…LCH most frequently involves the bone and skin, followed by the hematopoietic system, lymph node, liver, spleen, soft tissue, lung, thymus, and pituitary gland [ 15 ]. Among the most commonly reported systemic symptoms are development of a soft tissue mass, bone pain, skin rash, fever, and lymphadenopathy [ 5 ], while oral manifestations of LCH include hyperplasia of the gingiva or ulcers of the cheek, palate, or tongue mucosa and underlying bone lesions [ 16 ].…”

Solitary Langerhans cell histiocytosis of the hard palate: a diagnostic pitfall

“…The organs most commonly involved in adults are the lungs, skeleton, and skin. Isolated oral lesions occur in about 5% of cases and are usually due to underlying bone involvement [3], [4], [6]. In oral soft tissue, the sites commonly involved are the gingiva followed by the maxillary and the hard palate [6].…”

“…Various treatment modalities for LCH have been proposed, including close observation, surgical curettage, local injection of corticosteroids, low-dose radiotherapy, high-dose systemic corticosteroids, chemotherapy and bone marrow transplantation and antibody therapy for resistant cases [4]. Recent studies documenting mutations in BRAF and MAP2K1 genes could revolutionize the prevalent therapeutic regimens and pave way for targeted therapy in LCH, resulting in a better prognosis [2].…”

“…Isolated oral lesions occur in about 5% of cases and are usually due to underlying bone involvement. Exclusive mucosal involvement in adults has rarely been reported with only a handful cases in literature [4], [5], [6], [7]. …”