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.

Renker, Thekla; Haneke, Eckart; Röcken, C; Borradori, Luca (2014). 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. Dermatology, 228(2), pp. 97-102. Karger 10.1159/000354262

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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.

Item Type:

Journal Article (Original Article)

Division/Institute:

04 Faculty of Medicine > Department of Dermatology, Urology, Rheumatology, Nephrology, Osteoporosis (DURN) > Clinic of Dermatology

UniBE Contributor:

Renker, Thekla; Haneke, Eckart and Borradori, Luca

Subjects:

600 Technology > 610 Medicine & health

ISSN:

1018-8665

Publisher:

Karger

Language:

English

Submitter:

Monika Schenk

Date Deposited:

14 Apr 2015 15:29

Last Modified:

19 Oct 2015 11:31

Publisher DOI:

10.1159/000354262

PubMed ID:

24434547

BORIS DOI:

10.7892/boris.59185

URI:

https://boris.unibe.ch/id/eprint/59185

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