Pretibial epidermolysis bullosa: is this case a new subtype with loss of types IV and VII collagen?

Abstract

Pretibial epidermolysis bullosa (PEB) is an extremely rare subtype of dominant dystrophic epidermolysis bullosa (DDEB), in which recurrent blistering with scarring predominantly involves the pretibial skin. Nail dystrophy, albopapuloid lesions, and hypertrophic scars may also occur. In PEB, immunohistochemical and electron microscopic studies demonstrate the complete or partial loss of the anchoring fibril (AF) in the basement membrane zone, suggesting disturbed synthesis or excessive degradation of collagen VII, the main component of AF. Interestingly, we report a case of PEB with unusual results of joint loss of types IV and VII collagen.

• Authors: Hong-sun Lee, Kun Park, Sook-ja Son, Kye-yong Song, Seong Eon Kim
• Journal: International journal of dermatology (Int J Dermatol) Vol. 48 Issue 8 Pg. 879-81 (Aug 2009) ISSN: 1365-4632 [Electronic] England
• PMID: 19659868 (Publication Type: Case Reports, Journal Article)

Chemical References

• Collagen Type IV
• Collagen Type VII

Topics

• Biopsy
• Collagen Type IV (genetics, metabolism)
• Collagen Type VII (genetics, metabolism)
• Epidermolysis Bullosa (classification, genetics, pathology)
• Fluorescent Antibody Technique, Direct
• Humans
• Male
• Microscopy, Electron, Transmission
• Nails (pathology)
• Skin (pathology, ultrastructure)
• Tibia
• Young Adult