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)

A boy with skin eruptions resembling varicella and specific for Langerhans cell histiocytosis (LCH) is reported. At his initial visit when he was four months old, vesiculopustular lesions were present over the entire body; these had first appeared on the third day post partus. Histopathological, immunohistochemical, and electron microscopical examination confirmed the Langerhans cell phenotype and Birbeck granules in the responsible cells. He also had hydronephrosis, recurrent fever, and cutaneous bacterial infections. His parents refused further medical treatment and he died of diarrhea with cachexia about two years later. LCH may present diagnostic difficulties by manifesting as a skin eruption which resembles varicella.
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PMID:Langerhans cell histiocytosis presenting as a varicelliform eruption over the entire skin. 801 75

Concomitant tubercular and fungal cerebellar abscess is rare and we report the first concomitant recurrent multi-lobulated tubercular and fungal cerebellar abscess in an immunocompromised girl with Histiocytosis-X. She presented with cerebellar abscess history diagnosed during the ongoing treatment for tuberculous meningitis. The abscess was drained. Upon the detection of cerebellar abscess recurrence and pulmonary infection, she was referred to our clinic five weeks after the first surgical intervention. Patient was conscious, co-operating but confused. She had severe cachexia, stiff neck and fever. Fundus examination showed bilateral papilledema. Cranial MR images revealed multiple lobulated lesions. Suboccipital craniectomy was performed and abscess was evacuated in toto. Lesion was multi-lobulated. Thick, yellow-gray purulent material was drained. Histopathological examinations yielded Langhans giant cells,budding and branching fungal structures. Fungal infection was identified. We emphasize that posterior decompression and total resection should be considered first in the management of lesions with mass effect in the posterior fossa. Also the presence of concomitant fatal fungal abscess highlights that although the clinic and former diagnoses of the patient may direct the clinician to a certain pathogen, unusual resistant organisms should not be.
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PMID:Concomitant tubercular and fungal cerebellar abscess in an immunocompromised girl. 2334 73