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

Polymorphous skin rashes are one of the major presentations in children with Kawasaki disease. This report describes an unusual presentation of a skin rash (non-Langerhans cell histiocytosis) in a 4-month-old baby with resistance to intravenous immunoglobulin (IVIG) treatment and coronary artery dilatation. Though refractory to repeat dosages of IVIG treatment, the patient had a favourable response to methylprednisolone pulse therapy.
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PMID:Non-Langerhans cell histiocytosis in a child with Kawasaki disease. 2168 3

Langerhans cell histiocytosis (LCH) is a rare proliferative disease accompanied by the accumulation of pathological Langerhans cells, which often spreads into multi-site and multi-organ systems. We here describe a girl with a history of Kawasaki disease and cervical lymphadenopathy who presented with occipital LCH. Adrenal tumor was detected on staging evaluation of LCH and was diagnosed as neuroblastoma on resection using laparoscopic surgery. Neither tumor relapsed following chemotherapy for LCH and resection of neuroblastoma. Although LCH often spreads into multi-organ lesions, invasive biopsy may be needed for tumors with atypical localization for LCH in consideration of the synchronous occurrence of malignancies.
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PMID:Coexistence of neuroblastoma detected on staging of Langerhans cell histiocytosis. 2525 48

On examination, the oral cavity may exhibit manifestations of underlying systemic disease and serve as an indicator of overall health. Systemic diseases with oral findings include autoimmune, hematologic, endocrine, and neoplastic processes. Autoimmune disease may manifest as oral ulcerations, changes in the salivary and parotid glands, and changes in the tongue. Patients with hematologic illnesses may present with gingival bleeding or tongue changes such as glossitis, depending on the etiology. Oral changes associated with endocrine illness are variable and depend on the underlying condition. Neoplastic changes include metastatic lesions to the bony and soft tissues of the oral cavity. Patients with chronic diseases such as gastroesophageal reflux and eating disorders may present with dental erosions that cause oral pain or halitosis. In the pediatric population, oral changes can be related to rare cancers, such as Langerhans cell histiocytosis, or infectious etiologies, such as Kawasaki disease. In both adults and pediatric patients, poor oral health has been linked to poorer health outcomes overall. Thorough history taking and physical examination by dentists may aid in determining the underlying etiology of oral changes and allow for earlier intervention by medical colleagues.
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PMID:Oral manifestations of systemic disease. 2909 62