Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
1
pubmed:dateCreated
2008-1-7
pubmed:abstractText
Multiple self-healing squamous epithelioma of Ferguson-Smith (MSSE) is a rare autosomal dominantly inherited disease, almost exclusively reported in patients of Scottish origin, with recurrent, histologically malignant tumours that undergo spontaneous regression. We report clinical observations in a Danish family with 11 affected patients, which appears to be the first Scandinavian family published with the disease. Anamnestic data and data concerning age of onset, localization, number of tumours and treatment modalities were collected from the family and from medical records, and all living patients were examined. Detailed clinical findings in 6 patients are described, together with a summary of clinical data from all 11 affected family members. The mean age of onset in the family was 52.6 years (range 30-81 years). A total of 44 tumours, chiefly located in sun-exposed areas, were recorded and the average number of tumours per patient was 4 (range 1-23). 61.4% of the tumours were excised, 31.8% regressed spontaneously without treatment. MSSE may thus also affect patients of non-Scottish origin. The disease severity can vary greatly among patients and the onset can be at an advanced age. MSSE constitutes an important model for research into tumour biology.
pubmed:language
eng
pubmed:journal
pubmed:citationSubset
IM
pubmed:status
MEDLINE
pubmed:issn
0001-5555
pubmed:author
pubmed:issnType
Print
pubmed:volume
88
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
52-6
pubmed:meshHeading
pubmed:year
2008
pubmed:articleTitle
Multiple self-healing squamous epithelioma of Ferguson-Smith: observations in a Danish family covering four generations.
pubmed:affiliation
Department of Dermatology, Odense University Hospital, Odense C, Denmark. s.broesby-olsen@dadlnet.dk
pubmed:publicationType
Journal Article, Case Reports