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)

Langerhans cell histiocytosis is a rare idiopathic disorder with protean clinical presentations. Primary unifocal single-system disease of the vulva is even less common. We report a 62-year-old female patient presenting with an 18-month history of pruritus and burning of the vulva. Clinical examination of the vulva showed a tender nodule of the right labium minus. Histology confirmed Langerhans cell histiocytosis. Systemic involvement was excluded. Within 1 month the use of clobetasol propionate ointment led to resolution of both the patient's symptoms and the clinical appearance of the affected right labium minus. This resolution was maintained 12 months later.
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PMID:Rapid resolution of primary vulval adult Langerhans cell histiocytosis with very potent topical corticosteroids. 2133 84

Langerhans cell histiocytosis (LCH), previously known as histio-cytosis X, is a rare idiopathic disorder of reticulo-endothelial system with abnormal proliferation of bone marrow derived Langerhans cells along with a variable number of leukocytes, such as eosinophils, neutrophils, lymphocytes and plasma cells. Three years old male child presented with multifocal osteolytic lesions and papulosquamous skin lesions. Clinical and radio-graphic features, such as severe alveolar bone loss, mobility of teeth, precocious eruption of teeth, foating appearance of teeth in orthopantomogram (OPG), osteolytic lesion in skull and cutaneous lesions were highly suggestive of LCH disease. Skin biopsy confirmed a diagnosis of LCH. Induction chemotherapy with oral prednisolone and intravenous vinblastine was started. Child responded well to chemotherapy. The clinical significance of the presented case is to diagnose the case of LCH on the basis of the manifestation of severe periodontal disease as this can be first or only manifestation of LCH. A dentist plays a major role in the multidisciplinary treatment of LCH through routine examination and periodic follow-up. How to cite this article: Bansal M, Srivastava VK, Bansal R, Gupta V, Bansal M, Patne S. Severe Periodontal Disease Manifested in Chronic Disseminated Type of Langerhans Cell Histiocytosis in a 3-Year Old Child. Int J Clin Pediatr Dent 2014;7(3):217-219.
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PMID:Severe periodontal disease manifested in chronic disseminated type of langerhans cell histiocytosis in a 3-year old child. 2570 6