Langerhans’s cell histiocytosis in old subjects: two rare case reports and review of the literature

Abstract

Langerhans's cell histiocytosis in old subjects: two rare case reports and review of the literature Background: Langerhans cell histiocytosis (LCH) is a proliferative disease of histiocyte-like cells that generally affects children; LCH onset is rare in adults; immunohistochemistry is essential to obtain the correct diagnosis, and treatment protocols are controversial. Objective: To describe two new cases of adult onset oral LCH. Case reports: Case 1: a 71-year-old woman, complaining of diffuse oral pain, presented with erythematous mucosal lesions; the panoramic radiograph and CT scan showed multiple mandible radiolucent areas. Immunohistochemical assay for S-100, CD1a and langerin test was essential in reaching the correct diagnosis. Case 2: a 77-year-old female patient presented with a non-painful, non-bleeding, slightly elevated erythematous palatal lesion of 6 months duration, together with a genital vulvar lesion of uncertain nature. The pathology confirmed the diagnosis of LCH. Many therapies (etoposid, radiotherapy) could induce only a clinical partial remission; Cladribine induced a complete recovery. Conclusion:The first case was difficult to diagnose: the clinical presentation and course of the disease (LCH) in the elderly are multiple and unpredictable. An immunohistochemistry study is often essential to obtain the correct diagnosis. The second case required several therapeutic interventions: even though some cases regress spontaneously, others require systemic chemotherapy.

A rare and unusual case report of Langerhans cell histiocytosis

“…The mucosal lesion may develop as gingival mass if lesion has broken out of the bone. [15] In the present case, similar features were present.…”

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

“…LCH in elderly subjects is rare, and it has been presented mainly as case reports. 18,19 Although other studies have reported that males were more affected than females, [13][14][15] proportion of male and female patients was similar in our study.…”

Multidisciplinary approach in a case of Hand–Schüller–Christian disease with maxillary involvement

“…Systemic involvement of certain organs affects negatively the prognosis. In fact, if high‐risk organs (liver, spleen, bone marrow, and lungs) are involved, mortality ranges reaches 30–50%, in comparison with a mortality risk of 10% when these organs are not affected …”

“…Localized disease is described as eosinophilic granuloma, while multisystem forms of LCH have eponymous descriptions such as Hand–Schüller–Christian disease, Letterer–Siwe disease, and systemic LCH …”