Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019621 (Langerhans cell histiocytosis)
 3,250 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Report of a case of histiocytosis X with adult onset, widespread skin lesions and rare central nervous system involvement resulting in dizziness and ataxia. On autopsy histiocytosis X infiltrates in the central nervous system were demonstrated with granuloma formation in the temporal lobe and nucleus dentatus of the cerebellum.
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PMID:Histiocytosis X in adult with skin and uncommon central nervous system involvement. 30 3

A 39-year-old male was admitted to our clinic with symptoms of headache, dizziness, nausea, otalgia, otorrhea, tinnitus, and hearing loss in both ears for 3 weeks. Physical examination revealed edema in the tympanic membrane and external ear canal, and pain by palpation in the mastoid area bilaterally. There was no nystagmus, and the rest of the physical examination was otherwise normal. Temporal bone high resolution computed tomography (CT) showed a lesion causing erosion in the mastoid cortex, tegmen tympani, ossicles, and in the bone covering the sigmoid sinus bilaterally. There was also erosion in the superior semicircular canal and petrous bone on the left side. Cortical mastoidectomy was performed under general anesthesia. Histopathologic examination of the tissue revealed Langerhans cell histiocytosis (LCH). In this paper a case with LCH, presenting with bilateral mastoid involvement which has been rarely reported in the literature, is discussed with the existing literature.
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PMID:Langerhans cell histiocytosis in bilateral mastoid cavity. 2384 Oct 5

Rosai-Dorfman disease (RDD)-sinus histiocytosis with massive lymphadenopathy-represents a peculiar proliferation of histiocyte-like cells in patients. The condition was described by Rosai and Dorfman in 1969, after examining 4 cases, as an idiopathic histiocytic disorder. In 1972, they studied an additional 30 cases of patients with RDD. A histioproliferative disorder, RDD is characterized by bilateral, painless, cervical lymphadenopathy in 81% of patients. Fever, leukocytosis, elevated sedimentation rate, and polyclonal hypergammaglobulinemia may also be found. In 30% of patients, extranodal involvement is present and may include the skin, eye orbit, upper respiratory tract, or testes. Cases involving the central nervous system are rare and account for < 5% of patients with RDD. We report on a 78-year-old woman presenting with new-onset headache, dizziness, and imbalance, which had been present for a few weeks prior to admission. Magnetic resonance imaging of the brain showed 2 enhancing lesions within the right and left cerebellar hemispheres. Biopsy of the mass demonstrated a lymphohistiocytic infiltrate involving the cerebellum with foci of emperipolesis (phagocytosed lymphocytes). The adjacent cerebellum showed myelinated nerve fibers with reactive gliosis. A thorough work-up and histopathologic exam of the biopsied mass demonstrated lymphohistiocytic infiltrate with foci of emperipolesis (phagocytosed lymphocytes) consistent with RDD. Other differential considerations, such as primary or secondary neoplasms, infections, lymphoproliferative disorders, granulomatoses, Langerhans cell histiocytosis, and lymphocyte-rich meningioma were ruled out by additional histopathologic exam.
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PMID:Case report of intracranial Rosai-Dorfman disease. 2414 94

A 29-year-old female with past medical history of chronic serous otitis media presented with worsening neck stiffness and pain over a period of 2 weeks. The patient described non-specific symptoms that were localized to the right side of her neck. She presented to the hospital only when the pain was so extreme that it limited her range of motion. The differential for acute neck pain without fever, chills or any inciting trauma is vast. They include medical emergencies such as meningitis, acute coronary syndromes and extend to rheumatologic diseases or simply musculoskeletal strain. On review of systems, she denied dizziness, headache, vision changes, dysphagia, or other facial pain. Based on the severity of her pain, she underwent a Computed Tomography scan of the neck, which was concerning for erosive calavarial lesions. Further imaging revealed multiple lytic foci and erosions from the right maxillary sinus to the right mandible to the C1 vertebra. Following requisite surgical intervention, she was found to have Langerhans cell histiocytosis, a rare disease of myeloid cells, usually affecting pediatric populations. Little is known about the adult manifestations of Langerhans Cell Histiocytosis. This review contributes to broadening the literature on this topic which can present with complaints as typical as neck pain.
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PMID:Langerhans Cell Histiocytosis Presenting as Progressively Worsening Neck Pain: A Case Report With a Review of Literature. 3169 99