RGD Reference Report - Alterations in fibrillin as well as collagens I and III and elastin occur in vulval lichen sclerosus. - Rat Genome Database

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Alterations in fibrillin as well as collagens I and III and elastin occur in vulval lichen sclerosus.

Authors: Farrell, AM  Dean, D  Millard, PR  Charnock, FM  Wojnarowska, F 
Citation: Farrell AM, etal., J Eur Acad Dermatol Venereol. 2001 May;15(3):212-7.
RGD ID: 9585756
Pubmed: PMID:11683283   (View Abstract at PubMed)

BACKGROUND: The clinical features of lichen sclerosus, which include atrophy, scarring, fragility and tendency to form ecchymoses with only slight trauma, suggest that there is an alteration in the extracellular matrix fibres that are responsible for the tensile strength of the dermis. However, the precise nature of these changes is poorly understood. METHODS: Biopsies from 16 patients with untreated, histologically confirmed, vulval lichen sclerosus were examined immunohistochemically using polyclonal antibodies to collagens I and III and a monoclonal antibody to elastin. Twelve of the lichen sclerosus specimens were also stained with a monoclonal antibody to fibrillin. Normal vulva tissue and patients' uninvolved thigh were used as controls. RESULTS: In the lichen sclerosus specimens, collagens I and III stained with a more homogeneous pattern than in the control tissues. Reduced numbers of elastin fibres were seen in the zone of sclerosus in 15 of the 16 lichen sclerosus specimens. In the control tissue fibrillin fibres were seen as a fine network of fibres in the upper dermis arranged at right angles to and inserting into the basement membrane and forming a fine network throughout the dermis. In the lichen sclerosus specimens, although fibrillin microfibrils were still seen inserting at right angles into the basement membrane, below this the fibrillin staining was reduced in the upper dermis in 11 of the 12 lichen sclerosus specimens. The zone of reduced fibrillin staining was greatest in those specimens where the band of inflammation was deep in the dermis. CONCLUSIONS: The distribution of collagens I and III, elastin and fibrillin are altered in lichen sclerosus and this is likely to contribute to the fragility, scarring and atrophy seen clinically in lichen sclerosus.

RGD Manual Disease Annotations    Click to see Annotation Detail View
TermQualifierEvidenceWithReferenceNotesSourceOriginal Reference(s)
Lichen Sclerosus et Atrophicus  IEP 9585756protein:altered expression:vulva:RGD 
Lichen Sclerosus et Atrophicus  ISOELN (Homo sapiens)9585756; 9585756protein:altered expression:vulva:RGD 

Objects Annotated

Genes (Rattus norvegicus)
Eln  (elastin)

Genes (Mus musculus)
Eln  (elastin)

Genes (Homo sapiens)
ELN  (elastin)


Additional Information