Cookies on this website

We use cookies to ensure that we give you the best experience on our website. If you click 'Accept all cookies' we'll assume that you are happy to receive all cookies and you won't see this message again. If you click 'Reject all non-essential cookies' only necessary cookies providing core functionality such as security, network management, and accessibility will be enabled. Click 'Find out more' for information on how to change your cookie settings.

Dermo-epidermal separation in dystrophic epidermolysis bullosa (DEB) is thought to results from an abnormality of anchoring fibrils which, in normal skin, are stained selectively by the periodic acid-thiosemicarbazide-silver proteinate technique. This method was applied to non-lesional skin from four patients with generalized recessive DEB, five with localized recessive DEB and one with dominant DEB. No anchoring fibrils were present in the subjects with generalized recessive DEB, but they were morphologically normal in the case of dominant DEB and in three patients with mild localized recessive DEB. Two patients with a more severe form of localized recessive DEB possessed attenuated anchoring fibrils which lacked the normal banding of the mid-portion. The lamina densa, normally unlabelled, was consistently stained in patients with generalized recessive DEB, suggesting a biochemical abnormality of this structure.

Original publication

DOI

10.1111/j.1365-2133.1985.tb02353.x

Type

Journal article

Journal

Br j dermatol

Publication Date

10/1985

Volume

113

Pages

397 - 404

Keywords

Adolescent, Adult, Child, Epidermis, Epidermolysis Bullosa, Female, Genes, Dominant, Genes, Recessive, Humans, Male, Middle Aged, Periodic Acid, Silver Proteins, Skin, Staining and Labeling, Thiosemicarbazones