Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0042109 (urticaria)
 6,569 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 61-year-old woman presented with circumscribed eczematous eruptions with maceration, erosions and patchy infiltration in the perineum and inframammary regions. A diagnosis of eosinophilic granuloma (cutaneous histiocytosis X) was established. T lymphocytes from a skin biopsy were grown in vitro for three weeks after which chromosomal studies revealed a break or gap at chromosome 16q22 in 15% of the lymphocytes. The addition of alpha-interferon increased the percentage of affected cells to 28%. T lymphocytes from the patient's blood did not show the defect. The biological significance of the chromosomal defect is uncertain. It has been described before in healthy persons, malignant lymphoma, cold urticaria and IgA deficiency, and mental retardation. It has not been seen in patients with eosinophilic granuloma.
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PMID:Eosinophilic granuloma associated with a 16q22 chromosomal defect of cutaneous T lymphocytes. 608 32

The antibody NA1/34 is a murine monoclonal antibody directed against the CD1a surface antigen expressed on normal Langerhans cells, cortical thymocytes, and on lesional cells in Langerhans cell histiocytosis (LCH). Our hypothesis was that NA1/34 would localize sites of disease activity in patients with multisystem LCH. To test this hypothesis, indium 111-labeled NA1/34 was administered to five patients with multisystem LCH and serial gamma scans were obtained for up to 120 hours. Serial serum samples were obtained from one patient for analysis of anti-mouse Ig antibody and NA1/34 levels. Direct and indirect immunofluorescence staining for CD1a and NA1/34 were performed on a tissue biopsy specimen from one patient after administration of the antibody. The 1- and 4-hour scans showed distribution of antibody in the blood pool, but in later scans localization of the antibody was noted in areas of known disease activity in all five patients. Bony lesions, previously seen on skeletal radiographs, were especially well identified. Serum kinetics studies showed clearance of the antibody from the blood pool within 12 hours of administration. Direct binding of NA1/34 to lesional cells was demonstrated by direct immunofluorescence. The only adverse effect was urticaria in one patient. We conclude that NA1/34 localizes disease activity in vivo in bones of patients with LCH with minimal toxic effects. An evaluation of its role in determining disease extent ("staging") and in treatment is now needed.
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PMID:Successful in vivo immunolocalization of Langerhans cell histiocytosis with use of a monoclonal antibody, NA1/34. 752 14

We present the case of a 27-year-old Caucasian woman who suffered from Langerhans cell histiocytosis with axillary and scalp involvement. She also developed diabetes insipidus after 2 years of skin lesions. Topical nitrogen mustard therapy was performed for the skin lesions, but we had to stop this therapy because of severe local irritation and systemic urticaria. Afterwards, we administered etoposide systemically, but no improvement appeared in 6 weeks. Finally we used methotrexate for 3 months and the result was very good at the end of the first month.
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PMID:Langerhans cell histiocytosis in an adult. 807 73

Red-brown papules developing during the neonatal period may present a diagnostic dilemma. The Darier sign has been a reliable feature in the diagnosis of mastocytosis. However, the cutaneous infiltrate of Hashimoto-Pritzker Langerhans cell histiocytosis (LCH) may contain a large number of mast cells, leading to confusion both clinically and histologically. We report an infant who developed red-brown papules of Hashimoto-Pritzker LCH during the neonatal period and presented with a positive Darier sign and acute urticaria.
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PMID:Urticating Hashimoto-Pritzker Langerhans cell histiocytosis. 1120 70

Juvenile xanthogranuloma (JXG) is one of the most common forms of non-Langerhans cell histiocytosis in children. Although it usually presents as a self-limited skin lesion with typical histopathology, JXG can be challenging to diagnose due to an atypical initial presentation with corresponding variable histopathology for different stages of development. We present challenging cases of JXG from Sydney Children's Hospital, collected over 10 years - two with multisystem involvement and concomitant urticaria, one associated with neurofibromatosis, and one case of giant JXG with an initial histopathological challenge. Although JXG has been reported with urticaria pigmentosa, in two of our cases persistent urticaria, in association with JXG is discussed.
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PMID:Juvenile xanthogranuloma: challenges in complicated cases. 2207 Jul 4

Scabies, a mite infestation caused by 'Sarcoptes scabiei', most commonly presents as pruritic linear burrows where the mite has invaded the skin. Scabies variant such as bullous, crusted, hidden, incognito, nodular and scalp-mimic the other conditions. In addition, atypical presentations of scabies can masquerade as dermatitis herpetiformis, ecchymosis, Langerhans cell histiocytosis, systemic lupus erythematosus, urticaria, and urticaria pigmentosa. A 59-year-old male presented with non-pruritic papulosquamous plaques on his chest, abdomen, and back resembling lesions of pityriasis rosea in morphology and distribution. The complete cutaneous examination also demonstrated burrows on his finger webs. A mineral oil preparation of skin scrapings showed scabies mites, ova, and scybala. His infestation resolved after the treatment with topical permethrin 5% cream and oral ivermectin 15 mg on days one and eight. In conclusion, scabies surrepticius is a term that has recently been established to unify not only the numerous variants but also the atypical presentations of scabies. The inaccurate diagnosis of scabies infestation can lead to increased medical costs and the side effects of unnecessary tests and the treatment. Pityriasis rosea-like scabies can be added to the list of atypical presentations that are included under the unifying designation scabies surrepticius.
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PMID:Scabies Surrepticius: Scabies Masquerading as Pityriasis Rosea. 2949 50