Scleromyxedema-like Syndrome With Systemic Involvement in a Cat

Scleromyxedema—the generalized form of lichen myxedematosus, a primary mucinosis—is a rare disease in human patients. It is characterized by dermal mucin deposits, increased numbers of fibroblasts, and variable fibrosis in the absence of thyroid disease. It is accompanied in 80% of cases by a monocl...

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Bibliographic Details
Published in:Veterinary pathology 2010-03, Vol.47 (2), p.346-350
Main Authors: Müntener, T, Rüfenacht, S, Di Palma, S, Hartmeier, G, Welle, M
Format: Article
Language:English
Subjects:
Animals
blood protein disorders
Cat Diseases - pathology
Cats
connective tissues
disease course
Fatal Outcome
Female
fibrosis
gamma-globulins
gammaglobulinemia
glycosaminoglycans
histopathology
Immunohistochemistry - veterinary
mucin deposition
mucinosis
noninfectious diseases
protein deposition
Scleromyxedema - pathology
Scleromyxedema - veterinary
Skin Diseases - pathology
Skin Diseases - veterinary
systemic disease
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Summary:Scleromyxedema—the generalized form of lichen myxedematosus, a primary mucinosis—is a rare disease in human patients. It is characterized by dermal mucin deposits, increased numbers of fibroblasts, and variable fibrosis in the absence of thyroid disease. It is accompanied in 80% of cases by a monoclonal gammopathy. To date, scleromyxedema with systemic involvement has not been documented in domestic animals. This is the first report of a scleromyxedema-like syndrome in a cat, which had a substantial deposition of mucin in the dermis of the head and paws with a mild gammaglobulinemia of 2.25 g/dl (reference range, 1.39–2.22 g/dl). At necropsy, multiple nodules of connective tissue intermingled with mucin deposits were conspicuous on the surface of thoracic and abdominal organs. Such severe systemic accumulations of mucin have not been reported in human or veterinary medicine.
ISSN:0300-9858
1544-2217
DOI:10.1177/0300985809358039