Ultrastructural aspects of the skin in lipoid proteinosis (Urbach-Wiethe disease)
An. bras. dermatol
;
96(6): 730-734, Nov.-Dec. 2021. graf
Article
in English
| LILACS
| ID: biblio-1355621
ABSTRACT
Abstract Lipoid proteinosis is a rare autosomal recessive disease, characterized by hyaline deposits of PAS-positive material in tissues due to mutations in the ECM1 gene. This study evaluated the ultrastructure of the skin of a 6-year-old child affected by this condition. The light microscopy identified PAS-positive hyaline deposits, which were more intense in the papillary dermis. Scanning electron microscopy of the dermis showed a compact papillary dermis and fibrillar deposits in the middle dermis. Transmission electron microscopy clearly showed the deposition of fibrillar material in the dermis, forming clusters adherent to elastic fibers, between the collagen bundles and the collagen fibers, and also filling up the cytoplasm of dermal fibroblasts.
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Index:
LILACS (Americas)
Main subject:
Lipoid Proteinosis of Urbach and Wiethe
Type of study:
Prognostic study
Limits:
Child
/
Humans
Language:
English
Journal:
An. bras. dermatol
Journal subject:
Dermatology
Year:
2021
Type:
Article
Affiliation country:
Brazil
Institution/Affiliation country:
Universidade Católica de Pelotas/BR
/
Universidade Federal da São Paulo/BR
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