Image taken from Clinical Guide to Oral Diseases.
A 52-year-old woman presented with painful ulcerated gingivae and loose teeth.
The problem with her mouth started three years ago with pain from her lower anterior teeth and swollen gingivae. Despite root canal treatment of suspected teeth and scaling, no improvement was recorded.
Diabetes insipidus (mild) together with various myoskeletal problems were recorded over the last two years and partially responded to fluid uptake and NSAIDs. A few episodes of otitis were recorded and responded well to antibiotics.
Oral examination revealed soft, inflamed and partially ulcerated gingivae that were very sensitive and bled on probing, while the adjacent teeth were loose and painful, despite her root canal treatment. A few nodular-plaque lesions of both buccal mucosae were also found (Figure 18.4b) and associated with cervical lymphadenopathy. Radiological examination revealed multiple radiolucencies particularly in the mandible, skull and the right tibia. Blood tests revealed reduced antidiuretic hormone (ADH), an increased white blood count but with normal pattern,lack of paraproteins, and calcium and phosphorus within the normal values. Gingival biopsy revealed a plethora of aggregation of Langerhans cells and eosinophils within the submucosa.
Which is the possible cause: Histiocytosis X
Histiocytosis X (Langerhans cell histiocytosis, LCH) is the cause. This is a group of rare diseases characterized by local or generalized proliferation of histiocyte cells arising from the monocyte macrophage lineage. It affects one (unifocal) or numerous organs (multifocal) of young children to adult patients and is characterized by numerous bony osteolyses alone or together with one or more extra-bony involvements such as in lungs, spleen or liver, lymph nodes and oral mucosa and skin, with variable prognosis. Diabetes insipidus together with bone defects and mucocutaneous lesions are characteristic of the Hand-Schuller-Christian histiocytosis (chronic multifocal type) as seen in this patient.
Birbeck granules or bodies are rod-shaped or tennis racket-shaped cytoplasmic organelles seen in electron microscopy exclusively in Langerhans histiocytosis.
Coffee-bean like histiocytes are atypical cells that are distinguished by their eosinophilic cytoplasm and the longitudinal nuclear grooves in hematoxylin and eosin (HXE) stains, and their positive reaction in CD1a and Langerin (CD207) receptors in immunohistochemical stains.
Chemotherapy with vinblastine in combination with steroids is the first line of treatment for multisystemic LCH.
Antimetabolites like 2-chlorodeoxyadenosine and methotrexate interfere with DNA production and are widely used to stop division of abnormal Langerhans histiocytes.
Surgical excision or a low dose of radiotherapy, and sometimes intralesional injections of steroids like triamcinolone acetonide are effective only in the treatment of monostotic lesions.