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Dominantly inherited epidermal acantholysis in dogs, simulating human benign familial chronic pemphigus (Hailey-Hailey disease).

Publication ,  Journal Article
Sueki, H; Shanley, K; Goldschmidt, MH; Lazarus, GS; Murphy, GF
Published in: Br J Dermatol
February 1997

We report on dominantly inherited epidermal acantholysis in three dogs, a sire and two female offspring. The skin lesions were characterized by hairless, hypertrophic plaques. Histopathologically, these lesions showed epidermal hyperplasia with individual enlargement of keratinocytes, extensive acantholysis and minimal dyskeratosis. Ultrastructural analysis revealed that attachment plaques of desmosomes were still intact while some tonofilaments were detached from them in early lesions; there were well-developed microvilli at dissociated cell surfaces. The data imply that these animals have undergone a process similar to human benign familial chronic pemphigus (BFCP). Immunohistochemical examination revealed that staining for E-cadherin and actin variably remained in dissociated keratinocytes. Focal intracellular staining for desmosomal glycoproteins and desmosomal proteins were observed within the dissociated keratinocytes. This dominantly inherited acantholytic disease in dogs could be a useful animal model for investigating the pathogenesis of BFCP in humans.

Duke Scholars

Published In

Br J Dermatol

ISSN

0007-0963

Publication Date

February 1997

Volume

136

Issue

2

Start / End Page

190 / 196

Location

England

Related Subject Headings

  • Skin
  • Pemphigus, Benign Familial
  • Pedigree
  • Microscopy, Electron
  • Male
  • Humans
  • Fluorescent Antibody Technique
  • Female
  • Dogs
  • Dog Diseases
 

Citation

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Sueki, H., Shanley, K., Goldschmidt, M. H., Lazarus, G. S., & Murphy, G. F. (1997). Dominantly inherited epidermal acantholysis in dogs, simulating human benign familial chronic pemphigus (Hailey-Hailey disease). Br J Dermatol, 136(2), 190–196.
Sueki, H., K. Shanley, M. H. Goldschmidt, G. S. Lazarus, and G. F. Murphy. “Dominantly inherited epidermal acantholysis in dogs, simulating human benign familial chronic pemphigus (Hailey-Hailey disease).Br J Dermatol 136, no. 2 (February 1997): 190–96.
Sueki H, Shanley K, Goldschmidt MH, Lazarus GS, Murphy GF. Dominantly inherited epidermal acantholysis in dogs, simulating human benign familial chronic pemphigus (Hailey-Hailey disease). Br J Dermatol. 1997 Feb;136(2):190–6.
Sueki H, Shanley K, Goldschmidt MH, Lazarus GS, Murphy GF. Dominantly inherited epidermal acantholysis in dogs, simulating human benign familial chronic pemphigus (Hailey-Hailey disease). Br J Dermatol. 1997 Feb;136(2):190–196.
Journal cover image

Published In

Br J Dermatol

ISSN

0007-0963

Publication Date

February 1997

Volume

136

Issue

2

Start / End Page

190 / 196

Location

England

Related Subject Headings

  • Skin
  • Pemphigus, Benign Familial
  • Pedigree
  • Microscopy, Electron
  • Male
  • Humans
  • Fluorescent Antibody Technique
  • Female
  • Dogs
  • Dog Diseases