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 27-month-old girl had a rapidly expanding orbital frontal bone mass due to Langerhans' cell histiocytosis. The CT of the orbit showed a lateral orbital mass containing an irregular density that extended into the intracranial cavity through a lytic lesion of the bone. This is the first report of the use in the orbit of an intralesional steroid injection. Five months later the lesion had not recurred. This treatment spared the patient radiation therapy or a craniotomy with curettage.
J Pediatr Ophthalmol Strabismus
PMID:Intralesional methylprednisolone for Langerhans' cell histiocytosis of the orbit and cranium. 366 68

An 11-month-old boy presented with uniocular hypopyon, elevated intraocular pressure, and iris nodules. A differential diagnosis of physical abuse, infection, retinoblastoma, juvenile xanthogranuloma, and histiocytosis X were considered but initial physical and laboratory investigations all had normal results. Three weeks following initial presentation, the child developed fulminant acute myelogenous leukemia. The clinical course, investigations, and outcome are reviewed.
J Pediatr Ophthalmol Strabismus
PMID:Pseudohypopyon in acute myelogeneous leukemia. 762 68

Juvenile xanthogranuloma is a benign non-Langerhans cell histiocytosis characterized by skin lesions that tend to be self-limited. Ocular lesions can occur in juvenile xanthogranuloma, most commonly presenting as an iris granuloma. Skin lesions of juvenile xanthogranuloma may appear similar to lesions of mastocytosis. Mastocytosis includes a heterogeneous group of diseases characterized by the proliferation and abnormal infiltration of mast cells. Rubbing of cutaneous lesions leads to the release of histamine, causing the lesions to urticate. Juvenile xanthogranuloma and mastocytosis skin lesions occurring concurrently is extremely rare, with only four cases reported. Ocular juvenile xanthogranuloma and cutaneous lesions of mastocytosis have never been described in the same patient in the literature. The authors describe a patient with an ocular juvenile xanthogranuloma presenting at birth with cutaneous mastocytosis developing several years later.
J Pediatr Ophthalmol Strabismus 2014 Dec 12
PMID:Presumed ocular juvenile xanthogranuloma and biopsy-proven cutaneous mastocytosis occurring sequentially in a young boy. 2549 Feb 41