1/3. Langerhans' cell histiocytosis diagnosed through periodontal lesions: a case report.BACKGROUND: Langerhans' cell histiocytosis (LCH) is a rare disorder in which a neoplastic proliferation of cells similar to the Langerhans' cell of the epidermis is observed. The disease may affect different organs as solitary or multiple lesions with a complete dissemination in different organs such as the bone (jaws), lung, hypothalamus, skin and mucous membranes, lymph nodes, liver, and other tissues. Although LCH is considered a childhood or juvenile disease, the diagnosis is often made in adults, and in many cases, a juvenile form progresses into adult life. hand-Schuller-Christian disease (HSCD) is an LCH form in which the typical lesions Involve the cranial bones, the eyes, and the pituitary gland. methods: This article describes a case of a patient diagnosed, through periodontal lesions and diabetes insipidus, as having Langerhans' cell histiocytosis. CONCLUSION: As far as LCH lesions appearing in periodontal tissues, the periodontist should be involved in the detection of oral lesions.- - - - - - - - - - ranking = 1keywords = histiocytosis (Clic here for more details about this article) |
2/3. Multisystem Langerhans' cell histiocytosis (hand-Schuller-Christian disease) in an adult: a case report and review of the literature.Langerhans' cell histiocytosis (LCH) is a rare and enigmatic clonal disorder that affects mainly children. It is characterized by single or multiple granulomatous mass lesions composed of cells with the Langerhans' cell phenotype. Clinical presentation and behavior are heterogeneous and can range from a solitary lytic bone lesion (i.e., eosinophilic granuloma) with a favorable course to a fatal disseminated leukaemia-like form, with a wide spectrum of intermediate clinical presentations between these two extremes. Although LCH typically involves the bone, lesions can be found in almost all organs. We are reporting the case of a multisystem LCH in a 47-year-old patient who presented with a panhypopituitarism and diabetes insipidus, and who, 5 years later, developed mandibular, mastoid and femoral lesions. The final diagnosis of LCH was made on mandibular biopsy.- - - - - - - - - - ranking = 0.83333333333333keywords = histiocytosis (Clic here for more details about this article) |
3/3. Central diabetes insipidus: Is it Langerhans cell histiocytosis of the pituitary stalk? A diagnostic pitfall.Central diabetes insipidus (CDI) is a rare disorder that may be caused by a variety of diseases. In pediatric and adolescent patients the most common causes for CDI are Langerhans cell histiocytosis (LCH) and germinoma. To avoid a potentially hazardous biopsy of the hypothalamic pituitary region it is recommended to evaluate patients with CDI carefully to identify potential extracranial lesions. Since LCH is the most common systemic disease that may cause CDI, special focus is paid to the identification of LCH lesions. We report on a 9(1/2) year old girl who presented with central diabetes insipidus and a thickening of the pituitary stalk on magnetic resonance imaging. Diagnostic workup revealed a history of recurrent ear infections and a compressed 6th thoracic vertebral body on radiographs. Based on these findings LCH was anticipated. Upon growth of the pituitary stalk lesion the patient was treated with LCH standard chemotherapy. After an initial shrinkage of the lesion, a further growth of the pituitary stalk lesion was observed and the tumor was resected. Histopathology revealed germinoma. This case underscores the importance of a istopathologically proven diagnosis in patients with HPR tumors before the initiation of a specific therapy, even if the clinical findings are highly suggestive.- - - - - - - - - - ranking = 0.83333333333333keywords = histiocytosis (Clic here for more details about this article) |