Primary systemic amyloidosis of the hair and nails

Bedlow,; Sampson,; Holden,

doi:10.1046/j.1365-2230.1998.00385.x

Cited by 12 publications

(5 citation statements)

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“…They tend to slowly worsen over the course of several years [8,9]. To our knowledge, 20 cases of systemic amyloidosis associated with nail changes, including the present case, have been reported in the literature so far [7,8,9,10,11,12,13,14,15,16,17,18,19,20,21,22,23,24,25]. A summary of these cases is given in table 1.…”

Section: Discussionmentioning

confidence: 95%

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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

et al. 2014

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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.

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Section: Discussionmentioning

confidence: 95%

“…Alopecia is a rare complication of systemic amyloidosis. A literature search revealed only 14 cases in which development of an alopecia was highlighted [10,12,13,18,19,20,25,26,27,28]. In 3 of the cases the alopecia has been reported as initial leading sign of occult amyloidosis [20,27,28].…”

Section: Discussionmentioning

confidence: 99%

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

et al. 2014

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“…On review of the literature, we discovered 16 cases of amyloidosis with associated hair loss on examination. 13 - 19 There is one reported case of alopecia universalis as the initial presentation of an occult amyloidosis. Wheeler et al 14 described a case of a woman with anonychia, generalized hair loss, and weight loss for 18 months who was hospitalized with symptoms of heart failure and found to have amyloidosis associated with MM.…”

Section: Discussionmentioning

confidence: 99%

“…Wheeler et al 14 described a case of a woman with anonychia, generalized hair loss, and weight loss for 18 months who was hospitalized with symptoms of heart failure and found to have amyloidosis associated with MM. Among the case reports of alopecia secondary to amyloidosis, only 2 cases reported by Hunt et al 16 and Bedlow et al 17 described renal failure developing after diagnosis of amyloidosis. In this article, we report a similar case that is remarkable in presentation with alopecia universalis as the earliest indicator of amyloidosis and subsequent development of renal failure.…”

Section: Discussionmentioning

confidence: 99%

Oligosecretory Myeloma With Amyloidosis and Alopecia

Bilal

Mesropian

Lam

et al. 2018

Journal of Investigative Medicine High Impact Case Reports

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Amyloidosis is a systemic illness characterized by the extracellular deposition of abnormal proteins in body tissues and organs. In addition to renal involvement, amyloidosis can also present with a variety of skin manifestations, though rarely with alopecia. Sixteen cases of alopecia secondary to systemic amyloidosis are reported. There is one reported case that presented with alopecia universalis. We report a case of a 68-year-old woman presenting with alopecia universalis, rapid decline in kidney function, and nephrotic syndrome who was found to have multiple myeloma-associated AL amyloidosis (immunoglobulin light chain). Her serological workup including serum electrophoresis was negative and she underwent renal biopsy. Pathology revealed eosinophilic material within the mesangium that was Congo-red positive, had apple-green birefringence under polarized light, and ultramicroscopically appeared as fibrillary material. Subsequent bone marrow examination showed a diffuse increase in plasma cells with atypia indicating plasma cell neoplasm. This case underlines several interesting aspects of multiple myeloma and the way it may present with amyloidosis. The lack of monoclonal spike on electrophoresis yet positive light chain analysis deserves special attention by clinicians to avoid a missed diagnosis. The extensive skin involvement also raises several questions regarding the pathologic mechanisms of alopecia in a patient with amyloidosis.

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“…Alopecia is a less recognized feature of PSA, with only a few patients reported to date (Table ). Our case is uncommon because the scalp alopecia preceded the clinical findings of overt PSA by approximately 2 years.…”

Section: Discussionmentioning

confidence: 99%