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Pretibial epidermolysis bullosa: is this case a new subtype with loss of types IV and VII collagen?
Author(s) -
Lee Hongsun,
Park Kun,
Son Sookja,
Song Kyeyong,
Kim Seong Eon
Publication year - 2009
Publication title -
international journal of dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.677
H-Index - 93
eISSN - 1365-4632
pISSN - 0011-9059
DOI - 10.1111/j.1365-4632.2008.03983.x
Subject(s) - medicine , epidermolysis bullosa , anchoring fibrils , scars , basement membrane , dermatology , pathology , junctional epidermolysis bullosa (veterinary medicine) , extracellular matrix , laminin , biology , microbiology and biotechnology
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.