title: 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.
abstract: 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.
authors.affiliation: Department of Dermatology, University Hospital Bern, Bern, Switzerland.
authors.forename: T
authors.initials: T
authors.lastname: Renker
authors.forename: E
authors.initials: E
authors.lastname: Haneke
authors.forename: C
authors.initials: C
authors.lastname: Röcken
authors.forename: L
authors.initials: L
authors.lastname: Borradori
mesh_terms: D000368:Aged; D000506:Alopecia Areata / Q000209:etiology / Q000473:pathology*; D000686:Amyloidosis / Q000150:complications / Q000175:diagnosis* / Q000473:pathology; D003937:Diagnosis, Differential; D018450:Disease Progression; D042241:Early Diagnosis; D005260:Female; D006801:Humans; D007147:Immunoglobulin Light Chains; D000075363:Immunoglobulin Light-chain Amyloidosis; D007155:Immunologic Factors; D009260:Nail Diseases / Q000209:etiology / Q000473:pathology*; D012859:Sjogren's Syndrome / Q000209:etiology / Q000473:pathology*; D012867:Skin / Q000473:pathology*
chemical_list: D007147:Immunoglobulin Light Chains; D007155:Immunologic Factors