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Systemic light-chain amyloidosis revealed by progressive nail involvement, diffuse alopecia and sicca syndrome: report of an unusual case with a review of the literature.
Journal article

Systemic light-chain amyloidosis revealed by progressive nail involvement, diffuse alopecia and sicca syndrome: report of an unusual case with a review of the literature.

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  • 2014-01-18
Published in:
  • Dermatology (Basel, Switzerland). - 2014
Aged
Alopecia Areata
Amyloidosis
Diagnosis, Differential
Disease Progression
Early Diagnosis
Female
Humans
Immunoglobulin Light Chains
Immunoglobulin Light-chain Amyloidosis
Immunologic Factors
Nail Diseases
Sjogren's Syndrome
Skin
English Immunoglobulin light-chain (AL) amyloidosis is a form of systemic amyloidosis in which the fibrils are derived from monoclonal light chains. We report a case of a 66-year-old woman presenting with nail changes, parchment-like hand changes, progressive alopecia and sicca syndrome. Histopathological studies of biopsy specimens of the scalp, the nail, minor labial salivary glands and abdominal skin revealed deposits of AL κ-type amyloid. Urine protein electrophoresis exhibited a weak band of κ-type light chains. Based on this striking case, we here review the characteristic nail and hair manifestations associated with systemic amyloidosis. Knowledge of these signs is important for an early diagnosis of systemic amyloidosis, identification of the underlying disease and patient management.
Language
  • English
Open access status
closed
Identifiers
Persistent URL
https://sonar.ch/global/documents/162706
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