Langerhans cell histiocytosis—An update and rare case presentation

Abstract

Langerhans cell histiocytosis (LCH) is a rare condition caused by the abnormal proliferation of a type of dendritic cell of the immune system (Langerhans cells). Accumulation of the cells can take place in one or multiple organs of the body and results in localised tissue destruction. The most commonly affected site is the bones and 50% of these cases present in facial bones, meaning an awareness of the condition by dental specialists is important. Although debated, LCH is currently categorised as a cancerous process that is treated in some cases with chemotherapy. Its first mention in medical literature was in the mid‐1800s but it was not recognised as LCH until 1973. Here we present a case of LCH presenting in the mandibular condyle of a 27‐year‐old female with symptoms commonly associated with temporomandibular joint dysfunction. Failing to respond to simple conservative measures by her general dental practitioner, the patient was referred to her local oral and maxillofacial surgery unit for further management. Complicated by a pathological fracture of the mandible, the diagnosis of LCH required a multi‐disciplinary approach to management and subsequent surgical resection of the lesion and reconstruction with a costochondral rib graft.

Langerhans cell histiocytosis in the mandibular condyle and ramus. Endoscopic diagnosis. Case report

“…The present case presents the novel treatment of LCH in an uncommon location, in the mandibular ramus and condyle. In addition, the lesion presented an osteolytic radiological pattern with poorly-defined borders and cortical loss of the lesion, added to an increase in volume of the affected area, making it necessary to perform a differential diagnosis with entities such as osteomyelitis, lymphoma, Ewing's sarcoma and malignant metastases from a distant organs 4 . For the diagnosis, considering the age of the patient, the choice of the oral endoscopic approach for biopsy collection considerably reduced the morbidity compared to an extraoral approach, such as transient or permanent facial nerve damage, salivary fistula, in addition to the advantage of not leaving visible scars and promoting faster functional recovery.…”

“…In the latter, it displays an incidence of 10-20 % of all cases 1 , highest in the mandibular body, reaching 40 % in the pediatric population 3 . Localization to the mandibular condyle is infrequent; only 16 cases have been reported so far in the English literature, 5 of them in children 4 .…”

Rare diseases with temporomandibular joint manifestations: a systematic review

“…Kawasaki Disease [49] Pain, swelling/arthritis Vasculitis/Inflammation Unknown Klippel-Feil syndrome [50] Limited RDs had disc disorders and four RDs had diagnoses of degenerative joint disease (DJD). While three RDs reported TMD developmental disorders as hypoplasia of condyle, one RD had hyperplasia, one RD had calcified deposits, and one RD had TMJ constriction.…”