Langerhans cell histiocytosis revisited: Case report with review

Abstract

Langerhans cell histiocytosis (LCH) is a group of idiopathic disorders characterized by proliferation of bone marrow derived Langerhans cells and mature eosinophils. Their clinical features simulate common oral findings such as gingival enlargement, oral ulcers, and mobility of teeth, along with nonspecific radiographic features; hence, diagnosing such lesions becomes difficult for the oral physicians. These lesions are commonly seen in childhood; however, we are reporting a case of LCH in 29-year-old adult male. A provisional diagnosis of giant cell granuloma was considered based on history and examination, although the lesion was histologically proven to be LCH and was confirmed with immunohistochemical staining of S100 protein and CD1a antigen. The purpose of this paper is to enhance the understanding of diverse, nonpathognomical oral presentation of LCH that is easily misdiagnosed and overlooked by dentist.

An Aggressive Form of Langerhan Cell Histiocytosis in an Adult: Therapeutic Challenges

“…Oral manifestations could be the first sign of disease [ 1 ]; however, it initially presented as an incidental pelvic lesion in this case. Treatment is reserved for symptomatic patients, while asymptomatic patients are kept under observation because some lesions resolve spontaneously [ 2 ]. It is imperative that follow-up studies be done as the disease can reoccur [ 2 ].…”

“…Treatment is reserved for symptomatic patients, while asymptomatic patients are kept under observation because some lesions resolve spontaneously [ 2 ]. It is imperative that follow-up studies be done as the disease can reoccur [ 2 ]. Recurrences after initial treatment occur 12–18 months after [ 2 ].…”

“…It is imperative that follow-up studies be done as the disease can reoccur [ 2 ]. Recurrences after initial treatment occur 12–18 months after [ 2 ]. In our patient despite treatment with one of the front-line agents for refractory disease, in this case cytarabine [ 3 ], he still had spread of the disease.…”

“…Langerhans cell histiocytosis is rare and more commonly reported in children [ 1 ]. Due to its rare nature, therapeutic management is controversial, especially in adults [ 2 ]. Reoccurrence or spread of the disease is possible; therefore, regular follow-up is important in these patients [ 2 ].…”

“…Due to its rare nature, therapeutic management is controversial, especially in adults [ 2 ]. Reoccurrence or spread of the disease is possible; therefore, regular follow-up is important in these patients [ 2 ]. This case highlights an aggressive form of LCH that responded to vinblastine after failing cytarabine and nivolumab.…”