Premium
Langerhans’s cell histiocytosis in old subjects: two rare case reports and review of the literature
Author(s) -
Lajolo Carlo,
Campisi Giuseppina,
Deli Giorgio,
Littarru Cristiano,
Guiglia Rosario,
Giuliani Michele
Publication year - 2012
Publication title -
gerodontology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.7
H-Index - 54
eISSN - 1741-2358
pISSN - 0734-0664
DOI - 10.1111/j.1741-2358.2012.00629.x
Subject(s) - medicine , langerhans cell histiocytosis , histiocytosis , rare disease , dermatology , lesion , histiocyte , radiation therapy , langerhans cell , pathology , disease , radiology , antigen , immunology
doi: 10.1111/j.1741‐2358.2012.00629.x 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.