Amyloidosis cutaneous nodular E85.8

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

Amyloidosis cutis nodularis atrophicans; Amyloidosis nodular cutaneous; nodular amyloidosis; nodular cutaneous amyloidosis; Nodular cutaneous amyloidosis; Nodular skin amyloidosis; Skin amyloidosis nodular

History
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Gottron, 1950; Degos, 1961

Definition
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Rare localized cutaneous amyloidosis, which occurs slowly without itching (in contrast to lichen amyloidosus) and in which system involvement cannot (or initially cannot) be detected. The amyloid is derived from immunoglobulin light chains (AL amyloid). Transition to systemic amyloidosis is possible (transitions are seen in 10-50% of cases), so that patients require constant medical monitoring.

Manifestation
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Preferred in middle-aged men (24-87 years, median: 57 years); m:w=1.2:1.0 (this statement is not confirmed in all clinical studies).

Localization
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Face, capillitium, abdominal wall, extremities, soles of feet, glans penis, vulva.

Clinical features
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Single or multiple, soft to coarse, from a few millimetres to several centimetres in diameter, brownish to yellow, possibly also white, asymptomatic (especially non-itching) lumps and plaques. Larger "callous" plaques can be found on the soles of the feet. In the genital area (e.g. glans penis) large, bulging plaques or nodules can occur and are to be distinguished from condylomata acuminata.

After central regression formation of anetodermic foci with yellowish shimmering fat tissue possible (see initial description by H.Gottron with the additional designation "atrophicans").

About 7% of the cases later develop into systemic amyloidosis (minus variant of systemic AL amyloidosis associated with plasmocytoma or gammopathy).

Laboratory
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In about 50% of patients with nodular cutaneous amyloidosis paraproteinemia can be detected at the time of diagnosis.

Histology
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Amyloid masses infiltrate homogeneously under a normal to discretely flattened epidermis the entire corium up to the subcutis, leaving out the stratum papillare. Antisera against immunoglobulin light chains react positively. Antisera against keratin filaments are negative.

Direct Immunofluorescence
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Cytokeratin antibodies negative.

Differential diagnosis
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Therapy
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The therapy of choice is surgical debridement or ablative laser therapy (CO2 or Erbium-YAG laser).

Literature
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  1. Blixt EK et al (2014) Chronic lymphocytic leukemia: cutaneous involvement associated with nodular amyloidosis. Int J Dermatol doi: 10.1111/ijd.12202
  2. Borrowman TA (2003) Cutaneous nodular amyloidosis masquerading as a foot callus. J Am Acad Dermatol 49: 307-310
  3. Degos A et al (1965) Isolated amyloidosis at the glans penis. Bull Soc Fr. Dermatol Syphiligr 68: 159-160
  4. Fujimoto N (2002) Advanced glycation end product-modified beta2-microglobulin is a component of amyloid fibrils of primary localized cutaneous nodular amyloidosis. J Invest Dermatol 118: 479-484
  5. Gartner S et al (2015) Therapy of nodular amyloidosis by curettage: A case report. Act Dermatol 41: 385
  6. Goerttler E et al (1976) Amyloidosis cutis nodularis. Clinical, histopathological and ultrastructural findings. Dermatologist 27: 16-25
  7. Jievaltaite V et al (2018) Primary localized cutaneous nodular amyloidosis of the cheeks. Nude Dermatol 44: 156-159
  8. Kaltoft B et al (2013) Primary localised cutaneous amyloidosis--a systematic review. Dan Med J 60:A4727
  9. Katz KA (2003) Nodular localized primary cutaneous amyloidosis. Br J Dermatol 147: 400
  10. Konopinski JC et al (2013) A case of nodular cutaneous amyloidosis and review of the literature. Dermatol Online J 19:10
  11. Nakai N et al (2014) Nodular primary localized cutaneous amyloidosis in a patient with pulmonary sarcoidosis. Indian J Dermatol 59:307-308
  12. Reidel U et al (2015) solitary lesion on the glans penis. JDDG 13: 703-705
  13. Ritchie SA et al (2014) Primary localized cutaneous nodular amyloidosis of the feet: a case report and review of the
  14. literature. Cutis 93:89-94
  15. Ung CY et al (2014) Primary cutaneous nodular amyloidosis associated with psoriasis. Clin Exp Dermatol 39:608-611

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Last updated on: 29.10.2020