Dystrophic Epidermolysis Bullosa in Goats

Clinical, histopathological and ultrastructural findings of caprine dystrophic epidermolysis bullosa (DEB) with autosomal recessive inheritance are reported. The goats presented with exungulation, erosions, crusts and scars on the skin and ulcers in the oral cavity. Microscopically, the skin showed...

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Published inJournal of comparative pathology Vol. 148; no. 4; pp. 354 - 360
Main Authors Medeiros, G.X., Riet-Correa, F., Barros, S.S., Soares, M.P., Dantas, A.F.M., Galiza, G.J.N., Simões, S.V.D., Borges, A.S.
Format Journal Article
LanguageEnglish
Published England Elsevier Ltd 01.05.2013
Subjects
anchoring fibrils
Animals
basal membrane
basement membrane
Cicatrix - genetics
Cicatrix - pathology
Cicatrix - veterinary
Epidermis - pathology
Epidermis - ultrastructure
epidermolysis bullosa
Epidermolysis Bullosa Dystrophica - genetics
Epidermolysis Bullosa Dystrophica - pathology
Epidermolysis Bullosa Dystrophica - veterinary
Female
goat
Goat Diseases - genetics
Goat Diseases - pathology
Goats
histopathology
humans
Male
mouth
neutrophils
Oral Ulcer - genetics
Oral Ulcer - pathology
Oral Ulcer - veterinary
Pedigree
Skin - pathology
Skin - ultrastructure
goat
anchoring fibrils
epidermolysis bullosa
basal membrane
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Summary:Clinical, histopathological and ultrastructural findings of caprine dystrophic epidermolysis bullosa (DEB) with autosomal recessive inheritance are reported. The goats presented with exungulation, erosions, crusts and scars on the skin and ulcers in the oral cavity. Microscopically, the skin showed subepidermal separation with clefts filled occasionally with clear eosinophilic fluid, cellular debris or neutrophils. Ultrastructurally, the site of blister formation was the sublamina densa in the epidermal basement membrane zone. In skin with blister formation and in clinically uninvolved skin, the basal lamina was preserved, but the anchoring fibrils were sparse and rudimentary. A twin brother of an affected kid was mated over 5 years with his mother; three out of the 10 kids born presented with epidermolysis bullosa, indicating that the disease has an autosomal recessive mode of inheritance. It is suggested that the disease is similar to human severe generalized recessive DEB.
Bibliography:http://dx.doi.org/10.1016/j.jcpa.2012.09.002
ObjectType-Article-1
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content type line 23
ISSN:0021-9975
1532-3129
DOI:10.1016/j.jcpa.2012.09.002