Linked Life Data

18286355

Source:http://linkedlifedata.com/resource/pubmed/id/18286355

Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
2
pubmed:dateCreated
2008-4-3
pubmed:abstractText
Fasciitis panniculitis syndrome (FPS) has been proposed as a new category of 'fasciitis' and includes the well-established eosinophilic fasciitis (EF). Unlike EF, FPS exhibits inconsistent eosinophilia and/or eosinophilic infiltration of the lesions. Principal histological FPS findings include dermal thickening, inflammation and thickening of the subcutaneous fat tissue, fibrous thickening of the fascia and inflammation of the adjacent muscle. FPS is commonly resistant to corticosteroids, and cimetidine is effective in approximately 80% of FPS patients. A new therapy for FPS is required for cases refractory to treatment or intolerant to cimetidine because of adverse drug reaction. In this report, two FPS patients were resistant to corticosteroids. Both received intravenous cyclophosphamide (IVCY) concomitant with moderate- to high-dose prednisolone (PSL), and this effectively treated the induration of the FPS lesions. Patient 1 was a 50-year-old woman who had been diagnosed with fasciitis following en bloc muscle biopsy of the thigh. She had been treated with high-dose PSL for 6 years, but the fasciitis was refractory. Induration of the neck, thorax and thighs resulted in impaired neck rotation, restrictive respiratory failure and impaired walking. A diagnosis of FPS was made by re-assessing the en bloc muscle biopsy. Although PSL (40 mg/day) for 18 days was ineffective, the addition of IVCY (400 mg) dramatically improved the disease manifestations. Patient 2 was a 68-year-old man who was diagnosed with fasciitis based on en bloc muscle biopsy of the left foot. He had been treated with PSL for 16 years, but the fasciitis was refractory. He exhibited lower limb induration and a refractory skin ulcer of the left foot. A diagnosis of FPS was made by re-assessing the en bloc muscle biopsy. Although PSL (40 mg/day) for 2 weeks was ineffective, the addition of IVCY (450 mg) improved both the lower limb induration and the skin ulcer. FPS may cause both entrapment vasculopathy of subcutis and perivasculitis of the subcutaneous fat tissue such that the skin ulcer might be closely related with the ischemic mechanism triggered by FPS. According to the clinical courses of our cases, IVCY combined with moderate- to high-dose PSL may be a new therapeutic choice for corticosteroid-resistant FPS patients.
pubmed:language
eng
pubmed:journal
pubmed:citationSubset
IM
pubmed:chemical
pubmed:status
MEDLINE
pubmed:issn
1439-7595
pubmed:author
pubmed:issnType
Print
pubmed:volume
18
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
193-9
pubmed:meshHeading
pubmed-meshheading:18286355-Adipose Tissue, pubmed-meshheading:18286355-Aged, pubmed-meshheading:18286355-Cyclophosphamide, pubmed-meshheading:18286355-Dose-Response Relationship, Drug, pubmed-meshheading:18286355-Drug Resistance, pubmed-meshheading:18286355-Drug Therapy, Combination, pubmed-meshheading:18286355-Fasciitis, pubmed-meshheading:18286355-Female, pubmed-meshheading:18286355-Glucocorticoids, pubmed-meshheading:18286355-Humans, pubmed-meshheading:18286355-Immunosuppressive Agents, pubmed-meshheading:18286355-Injections, Intravenous, pubmed-meshheading:18286355-Male, pubmed-meshheading:18286355-Middle Aged, pubmed-meshheading:18286355-Panniculitis, pubmed-meshheading:18286355-Prednisolone, pubmed-meshheading:18286355-Respiratory Insufficiency, pubmed-meshheading:18286355-Syndrome, pubmed-meshheading:18286355-Treatment Outcome
pubmed:year
2008
pubmed:articleTitle
Therapeutic efficacy of intravenous cyclophosphamide concomitant with moderate- to high-dose prednisolone in two patients with fasciitis panniculitis syndrome.
pubmed:affiliation
Institute of Rheumatology, Aoyama Hospital, Tokyo Women's Medical University, Tokyo, Japan. kintarou@s9.dion.ne.jp
pubmed:publicationType
Journal Article, Case Reports