Skip to main content
SpringerLink
Log in

The Amyloidoses

  • Chapter
Dermatology

Abstract

The amyloidoses are a group of uncommon disorders in which a fibrillar protein, amyloid, is deposited in tissue. There are localized and generalized forms of amyloidosis; both types may affect the skin. Virchow first described amyloid as a starchlike substance, not realizing how many forms it could take. As Table 41.1 makes clear, amyloid is not one substance but many. The main types of amyloid in the skin are primary or light-chain amyloid (AL), secondary or wear-and-tear amyloid (AA) and keratin-related or skin amyloid (AK). In tissue amyloid appears as a homogeneous, amorphous almost translucent material with a variety of special staining characteristics, as shown in Table 41.2. Amyloid is typically deposited just below the basement membrane zone, about vessels or along collagen fibers. One cannot always distinguish amyloid histologically from other similar deposits, such as those in porphyria cutanea tarda, lipoid proteinosis and colloid milium.

Table 41.1 Types of amyloid
Full size table
Table 41.2 Staining characteristics of amyloid
Full size table

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Log in via an institution
Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 74.99
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

Preview

Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

Bibliography

  • Akpolat I, Akpolat T, Danaci M et al. (1997) Behçet’s disease and amyloidosis. Review of the literature. Scand J Rheu matol 26: 477–479

    Article  CAS  Google Scholar 

  • Al-Hoqail I, Naddaf H, Al-Rikabi A et al. (1997) Systemic lupus erythematosus and amyloidosis. Clin Rheumatol 16: 422–424

    Article  PubMed  CAS  Google Scholar 

  • Alster TS, Manaloto RM (1999) Nodular amyloidosis treated with a pulsed dye laser. Dermatol Surg 25: 133–135

    Article  PubMed  CAS  Google Scholar 

  • Bieber T, Ruzicka T, Linke RP et al. (1988) Hemorrhagic bullous amyloidosis. A histologic, immunocytochemical, and ultrastructural study of two patients. Arch Dermatol 124: 1683–1686

    Article  PubMed  CAS  Google Scholar 

  • Bonar L, Cohen AS, Skinner MM (1969) Characterization of the amyloid fibril as a cross-beta protein. Proc Soc Exp Biol Med 131: 1373–1375

    PubMed  CAS  Google Scholar 

  • Breathnach SM (1988) Amyloid and amyloidosis. J Am Acad Dermatol 18: 1–16

    Article  PubMed  CAS  Google Scholar 

  • Carrell RW, Lomas DA (1997) Conformational disease. Lancet 350: 134–138

    Article  PubMed  CAS  Google Scholar 

  • Chang YT, Liu HN, Wong CK et al. (1997) Detection of Epstein-Barr virus in primary cutaneous amyloidosis. Br J Dermatol 136: 823–826

    Article  PubMed  CAS  Google Scholar 

  • Chang YT, Wong CK, Chow KC et al. (1999) Apoptosis in primary cutaneous amyloidosis. Br J Dermatol 140: 210–215

    Article  PubMed  CAS  Google Scholar 

  • Cohen AS, Calkins E (1959) Electron microscopic observations on a fibrous component in amyloid of diverse origins. Nature 183: 1202–1203

    Article  PubMed  CAS  Google Scholar 

  • Falk RH, Comenzo RL, Skinner M (1997) The systemic amy-loidoses. New Engl J Med 337: 898–909

    Article  PubMed  CAS  Google Scholar 

  • Gillmore JD, Hawkins PN, Pepys MB (1997) Amyloidosis: a review of recent diagnostic and therapeutic developments. Br J Haematol 99: 245–256

    Article  PubMed  CAS  Google Scholar 

  • Glenner GG, Terry W, Harada M et al. (1971) Amyloid fibril proteins: proof of homology with immunoglobulin light chains by sequence analyses. Science 172: 1150–1151

    Article  PubMed  CAS  Google Scholar 

  • Goller MM, Cohen PR, Duvic M (1997) Lichen amyloidosis presenting as a papular pruritus syndrome in a human-immunodeficiency-virus-infected man. Dermatology 194: 62–64

    Article  PubMed  CAS  Google Scholar 

  • Gottron HA (1950) Amyloidosis cutis nodularis atrophicans diabetica. Dtsch Med Wochenschr 75: 19–24

    Article  Google Scholar 

  • Goulden V, Highet AS, Shamy HK (1994) Notalgia paraesthetica — report of an association with macular amyloidosis. Clin Exp Dermatol 19: 346–349

    Article  PubMed  CAS  Google Scholar 

  • Hagari Y, Mihara M, Hagari S (1996) Nodular localized cutaneous amyloidosis: detection of monoclonality of infiltrating plasma cells by polymerase chain reaction. Br J Dermatol 135: 630–633

    Article  PubMed  CAS  Google Scholar 

  • Harahap M, Marwali MR (1998) The treatment of lichen amyloidosis. A review and a new technique. Dermatol Surg 24: 251–254

    Article  PubMed  CAS  Google Scholar 

  • Hashimoto K, Ito K, Kumakiri M et al. (1987) Nylon brush macular amyloidosis. Arch Dermatol 123: 633–637

    Article  PubMed  CAS  Google Scholar 

  • Helander I, Hopsu-Havu VK (1986) Treatment of lichen amyloidosus by etretinate. Clin Exp Dermatol 11: 574–577

    Article  PubMed  CAS  Google Scholar 

  • Holzman TF, Krafft GA (1996) The nanometer-scale structure of amyloid-beta visualized by atomic force microscopy. J Protein Chem 15: 193–203

    Article  PubMed  Google Scholar 

  • Horiguchi Y, Fine JD, Leigh IM et al. (1992) Lamina densa malformation involved in histogenesis of primary localized cutaneous amyloidosis. J Invest Dermatol 99: 12–18

    Article  PubMed  CAS  Google Scholar 

  • Humphreys F, Spencer J, Mclaren K et al. (1996) An histological and ultrastructural study of the “dirty neck” appearance in atopic eczema. Clin Exp Dermatol 21: 17–19

    Article  PubMed  CAS  Google Scholar 

  • Inazumi T, Hakuno M, Yamada H et al. (1994) Characterization of the amyloid fibril from primary localized cutaneous nodular amyloidosis associated with Sjögren’s syndrome. Dermatology 189: 125–128

    Article  PubMed  CAS  Google Scholar 

  • Iwasaki K, Mihara M, Nishura S et al. (1991) Biphasic amyloidosis arising from friction melanosis. J Dermatol 18: 86–91

    PubMed  CAS  Google Scholar 

  • Konig A, Wennemuth G, Soyer HP et al. (1999) Vulvar amyloidosis mimicking giant condylomata acuminata in a patient with multiple myeloma. Eur J Dermatol 9: 29–31

    PubMed  CAS  Google Scholar 

  • Kousseff BG, Espinoza C, Zamore GA (1991) Sipple syndrome with lichen amyloidosis as a paracrinopathy: pleiotropy, heterogeneity, or a contiguous gene? J Am Acad Dermatol 25: 651–657

    Article  PubMed  CAS  Google Scholar 

  • Kyle RA, Gertz MA, Greipp PR et al. (1997) A trial of three regimens for primary amyloidosis: colchicine alone, melphalan and prednisone, and melphalan, prednisone, and colchicine. N Engl J Med 336: 1202–1207

    Article  PubMed  CAS  Google Scholar 

  • Lee DD, Huang CY, Wang CK (1998) Dermatopathologic findings in 20 cases of systemic amyloidosis. Am J Der-matopathol 20: 438–442

    CAS  Google Scholar 

  • Lien MH, Railan D, Nelson BR (1997) The efficacy of dermabrasion in the treatment of nodular amyloidosis. J Am Acad Dermatol 36: 315–316

    Article  PubMed  CAS  Google Scholar 

  • Lomakin A, Teplow DB, Kirschner DA et al. (1997) Kinetic theory of fibrillogenesis of amyloid-beta protein. Proc Natl Acad Sci U S A 94: 7942–7947

    Article  PubMed  CAS  Google Scholar 

  • Lubarsch O (1929) Zur Kenntnis ungewöhnlicher Amyloid-ablagerungen. Virchows Arch Pathol 271: 867–889

    Article  Google Scholar 

  • Macsween RM, Saihan EM (1997) Nylon cloth macular amyloidosis. Clin Exp Dermatol 22: 28–29

    Article  PubMed  CAS  Google Scholar 

  • Masouyé I (1997) Diagnostic screening of systemic amyloidosis by abdominal fat aspiration: an analysis of 100 cases. Am J Dermatopathol 19: 41–45

    Article  PubMed  Google Scholar 

  • Muckle TJ (1979) The “Muckle Wells” syndrome. Br J Dermatol 100: 87–92

    Article  PubMed  CAS  Google Scholar 

  • Ogiyama Y, Hayashi Y, Kou C et al. (1996) Cutaneous amyloidosis in patients with progressive systemic sclerosis. Cutis 57: 28–32

    PubMed  CAS  Google Scholar 

  • Ozkaya-Bayazit E, Kavak A, Gungor H et al. (1998) Intermittent use of topical dimethylsulfoxide in macular and papular amyloidosis. Int J Dermatol 37: 949–954

    Article  PubMed  CAS  Google Scholar 

  • Palitz LL, Peck S (1952) Amyloidosis cutis: a macular variant. Arch Dermatol 65: 451–457

    Article  CAS  Google Scholar 

  • Reider N, Sepp N, Fritsch P (1997) Remission of lichen amyloidosus after treatment with acitretin. Dermatology 194: 309-X311

    Article  PubMed  CAS  Google Scholar 

  • Robert C, Aractingi S, Prost C et al. (1993) Bullous amyloidosis. Report of 3 cases and review of the literature. Medicine (Baltimore) 72: 38–44

    CAS  Google Scholar 

  • Rocken C, Schwotzer EB, Linke RP et al. (1996) The classification of amyloid deposits in clinicopathological practice. Histopathology 29: 325–335

    Article  PubMed  CAS  Google Scholar 

  • Romero LS, Kantor GR, Lewin MW et al. (1997) Localized cutaneous amyloidosis associated with mycosis fungoides. J Am Acad Dermatol 37: 124–127

    Article  PubMed  CAS  Google Scholar 

  • Rubenow A, Cohen AS (1978) Skin involvement in generalized amyloidosis: a study of clinically involved and un-involved skin in 50 patients with primary and secondary amyloidosis. Ann Intern Med 88: 781–785

    Google Scholar 

  • Ruzicka T, Schmoeckel C, Ring J et al. (1985) Bullous amyloidosis. Br J Dermatol 113: 85–95

    Article  PubMed  CAS  Google Scholar 

  • Sumitra S, Yesudian P (1993) Friction amyloidosis: a variant of an etiologic factor in amyloidosis cutis? Int J Dermatol 321: 422–423

    Article  Google Scholar 

  • Tay CH, Dacosta JL (1970) Lichen amyloidosis: clinical study of 40 cases. Br J Dermatol 82: 129–136

    Article  PubMed  CAS  Google Scholar 

  • Tiitinen S, Kaarela K, Helin H et al. (1993) Amyloidosis-incidence and early risk factors in patients with rheumatoid arthritis. Scand J Rheumatol 22: 158–161

    Article  PubMed  CAS  Google Scholar 

  • Valentin R, Gurtler KF, Schaker A (1997) Renal amyloidosis and renal failure-a novel complication of the SAPHO syndrome. Nephrol Dial Transplant 12: 2420–2423

    Article  PubMed  CAS  Google Scholar 

  • Vestey JP, Tidman MJ, Mclaren KM (1994) Primary nodular cutaneous amyloidosis-long-term follow-up and treatment. Clin Exp Dermatol 19: 159–162

    Article  PubMed  CAS  Google Scholar 

  • Wang CK (1987) Cutaneous amyloidosis. Int J Dermatol 26: 273–277

    Article  Google Scholar 

  • Wang CK (1990) Mucocutaneous manifestations in systemic amyloidosis. Clin Dermatol 8: 7–12

    Article  Google Scholar 

  • Wang CK, Lin CS (1988) Friction amyloidosis. Int J Dermatol 27: 302–307

    Article  Google Scholar 

  • Wang CK, Lee JY (1996) Macular amyloidosis with widespread diffuse pigmentation. Br J Dermatol 135: 135–138

    Article  PubMed  CAS  Google Scholar 

  • Wang WJ, Lin CS, Wong CK (1986) Response of systemic amyloidosis to dimethylsulfoxide. J Am Acad Dermatol 15: 402–405

    Article  PubMed  CAS  Google Scholar 

  • Westermark P, Noren P (1986) Two different pathogenetic pathways in lichen amyloidosis and macular amyloidosis. Arch Dermatol Res 278: 206–213

    Article  PubMed  CAS  Google Scholar 

  • Westermark P, Ridderstrom E, Valquist A (1996) Macular posterior pigmentary incontinence: its relation to macular amyloidosis and notalgia paresthetica. Acta Derm Venereol (Stockh) 76: 302–304

    CAS  Google Scholar 

  • Weyers W, Weyers I, Bonczkowitz M et al. (1997) Lichen amyloidosus: a consequence of scratching. J Am Acad Dermatol 37: 923–928

    Article  PubMed  CAS  Google Scholar 

  • WHO-IUIS Nomenclature Sub-Committee (1993) Nomenclature of amyloid and amyloidosis. Bull World Health Organ 71:.105–112

    Google Scholar 

  • Winkelman RK, Peters MS, Venencie PY (1985) Amyloid elastosis. A new cutaneous and systemic pattern of amyloidosis. Arch Dermatol 121: 498–502

    Article  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Dermatology and Allergology, Ludwig Maximilians University, Frauenlobstraße 9-11, D-80337, Munich, Germany

    Prof. Dr. med. Dr. h.c.mult. Otto Braun-Falco (Emeritus Professor and Chairman), Prof. Dr. med. Gerd Plewig (Professor and Chairman) & Walter H. C. Burgdorf M.D. (Clinical Lecturer)

  2. Department of Dermatology and Venerology, University of Lübeck, Ratzeburger Allee 160, D-23562, Lübeck, Germany

    Prof. Dr. med. Helmut H. Wolff (Professor and Chairman)

Authors
  1. Prof. Dr. med. Dr. h.c.mult. Otto Braun-Falco
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Prof. Dr. med. Gerd Plewig
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Prof. Dr. med. Helmut H. Wolff
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Walter H. C. Burgdorf M.D.
    View author publications

    You can also search for this author in PubMed Google Scholar

Rights and permissions

Reprints and permissions

Copyright information

© 2000 Springer-Verlag Berlin Heidelberg

About this chapter

Cite this chapter

Braun-Falco, O., Plewig, G., Wolff, H.H., Burgdorf, W.H.C. (2000). The Amyloidoses. In: Dermatology. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-97931-6_41

Download citation

  • DOI: https://doi.org/10.1007/978-3-642-97931-6_41

  • Publisher Name: Springer, Berlin, Heidelberg

  • Print ISBN: 978-3-642-97933-0

  • Online ISBN: 978-3-642-97931-6

  • eBook Packages: Springer Book Archive

Publish with us

Policies and ethics

Search

Navigation