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.

Keywords:
Light-chain amyloidosis, Nail changes, Alopecia, Sicca syndrome
1.
Breathnach SM: Amyloid and amyloidosis. J Am Acad Dermatol 1988;18:1-16.
2.
Borowicz J, Gillespie M, Miller R: Cutaneous amyloidosis. Skin Med 2011;9:96-100.
3.
Sipe JD, Benson MD, Buxbaum JN, Ikeda S, Merlini G, Saraiva MJ, Westermark P: Amyloid fibril protein nomenclature: 2010 recommendations from the nomenclature committee of the International Society of Amyloidosis. Amyloid 2010;17:101-104.
4.
Gertz MA, Lacy MQ, Dispenzieri A: Amyloidosis. Hematol Oncol Clin North Am 1999;13:1211-1233.
5.
Skinner M, Sanchorawala V, Seldin DC, Dember LM, Falk RH, Berk JL, Anderson JJ, O'Hara C, Finn KT, Libbey CA, Wiesman J, Quillen K, Swan N, Wright DG: High-dose melphalan and autologous stem-cell transplantation in patients with AL amyloidosis: an 8-year study. Ann Intern Med 2004;140:85-93.
6.
Kyle RA, Gertz MA, Greipp PR, Witzig TE, Lust JA, Lacy MQ, Thernau TM: Long-term survival (10 years or more) in 30 patients with primary amyloidosis. Blood 1999;93:1062-1066.
7.
Fanti PA, Tosti A, Morelli R, Galbiati G: Nail changes as the first sign of systemic amyloidosis. Dermatologica 1991;183:44-46.
8.
Mancuso G, Fanti PA, Berdondini RM: Nail changes as the only skin abnormality in myeloma-associated systemic amyloidosis. Br J Dermatol 1997;137:471-472.
9.
Prat C, Moreno A, Viñas M, Jucglà A: Nail dystrophy in primary systemic amyloidosis. J Eur Acad Dermatol Venereol 2008;22:107-109.
10.
Goltz RW: Systematized amyloidosis. A review of the skin and mucous membrane lesions and a report of two cases. Medicine 1952;31:381-409.
11.
Breathnach SM, Wilkinson JD, Black MM: Systemic amyloidosis with an underlying lymphoproliferative disorder. Report of a case in which nail involvement was a presenting feature. Clin Exp Dermatol 1979;4:495-499.
12.
Wheeler GE, Barrows GH: Alopecia universalis. A manifestation of occult amyloidosis and multiple myeloma. Arch Dermatol 1981;117:815-816.
13.
Blanc D, Kienzler JL, Faivre R, Hory B, Coulon G, Saint-Hillier Y, Laurent R, Agache P: Amylose systématisée primitive avec alopécie et onychodystrophie généralisées. Ann Dermatol Venereol 1982;109:877-880.
14.
Civatte J, Jeanmougin M, Belaich S, Baran R: Myeloma-associated systemic amyloidosis with nail dystrophy. Clin Exp Dermatol 1982;7:224-225.
15.
Pineda MS, Herrero C, Palou J, Vilalta A, Mascaró JM: Nail alterations in systemic amyloidosis: Report of one case, with histologic study. J Am Acad Dermatol 1988;18:1357-1359.
16.
Derrick E: Primary systemic amyloid with nail dystrophy. J R Soc Med 1995;88:290-291.
17.
Ostlere LS, Stevens H, Mehta A, Rustin MHA: Nail dystrophy. Arch Dermatol 1995;131:953-956.
18.
Richey TK, Bennion SD: Etiologies of the sicca syndrome: primary systemic amyloidosis and others. Int J Dermatol 1996;35:553-557.
19.
Bedlow AJ, Sampson SA, Holden CA: Primary systemic amyloidosis of the hair and nails. Clin Exp Dermatol 1998;23:298-299.
20.
Lutz ME, Pittelkow MR: Progressive generalized alopecia due to systemic amyloidosis. J Am Acad Dermatol 2002;46:434-436.
21.
Cholez C, Cuny JF, Pouaha J, Thomas P, Tromp G, Truchetet F: Atteinte unguéale et infiltration sclérodermiforme du visage au cours d'une amylose systémique. Ann Dermatol Venereol 2005;132:252-254.
22.
Fujita Y, Tsuji-Abe Y, Sato-Matsumura KC, Akiyama M, Shimizu H: Nail dystrophy and blisters as sole manifestations in myeloma-associated amyloidosis. J Am Acad Dermatol 2006;54:712-714.
23.
Hoshino Y, Umezawa K, Machida M, Shimano S, Taya T, Shibasaki T, Hoshizaki H: Nail dystrophy associated with AL amyloidosis. Intern Med 2007;46:525.
24.
Pink AE, Stefanato CM, Breathnach SM: An unusual presentation of systemic AL amyloidosis: bullae, milia and nail dystrophy. Clin Exp Dermatol 2012;22:1-3.
25.
Hart C, Wurm S, Hafner C, Andreesen R, Grube M: Ungewöhnliche Hautveränderungen bei 72-jähriger Patientin mit multiplem Myelom. Internist 2012;53:467-473.
26.
Brownstein MH, Helwig EB: The cutaneous amyloidoses. II. Systemic forms. Arch Dermatol 1970;102:20-28.
27.
Hsieh SD, Yamamoto R, Saito K, Iwamoto Y, Kuzuya T, Ohba S, Kobori S, Saito K: Amyloidosis presented with whitening and loss of hair which improved after dimethylsulfoxide (DMSO) treatment. Jpn J Med 1987;26:393-395.
28.
Hunt SJ, Caserio RJ, Abell E: Primary systemic amyloidosis causing diffuse alopecia by telogen arrest. Arch Dermatol 1991;127:1067-1068.
29.
Reyes CM, Rudinskaya A, Kloss R, Girardi M, Lazova R: Scleroderma-like illness as a presenting feature of multiple myeloma and amyloidosis. J Clin Rheumatol 2008;14:161-165.
30.
Gogel HK, Searles RP, Volpicelli NA, Cornwell GG: Primary amyloidosis presenting as Sjögren's syndrome. Arch Intern Med 1983;143:2325-2326.
31.
Yokota E, Etoh H, Araki Y, Kato E, Nagamine M, Kawachi H, Seita M: A case of sicca syndrome due to primary amyloidosis. Jpn J Med 1984;23:250-253.
32.
Richey TK, Bennion SD: Etiologies of the sicca syndrome: primary systemic amyloidosis and others. Int J Dermatol 1996;35:553-557.
33.
Haneke E: Über die diagnostische Aussage von Biopsien der kleinen Lippenspeicheldrüsen bei Mundtrockenheit. Dtsch Z Mund Kiefer Gesichtschir 1981;5:26-29.
34.
Merlini G, Seldin DC, Gertz MA: Amyloidosis: pathogenesis and new therapeutic options. J Clin Oncol 2011;29:1924-1933.
35.
Desport E, Bridoux F, Sirac C, Delbes S, Bender S, Fernandez B, Quellard N, Lacombe C, Goujon JM, Lavergne D, Abraham J, Touchard G, Fermand JP, Jaccard A: AL amyloidosis. Orphanet J Rare Dis 2012;7:54.
36.
Guidelines on the diagnosis and management of AL amyloidosis. Guidelines Working Group of UK Myeloma Forum, British Committee for Standards in Haematology, British Society for Haematology. Br J Haematol 2004;125:681-700.
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2014
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