Localized Cutaneous Nodular Amyloidosis E85.8

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 07.08.2023

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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 occurring without pruritus (in contrast to the highly pruritic lichen amyloidosus) slowly insidiously, in which systemic involvement cannot (or initially cannot) be detected.

In this case, the amyloid is derived from immunoglobulin light chains (AL amyloid or amyloid L). Transition to systemic amyloidosis is possible (transitions are seen in 10-50% of cases), so patients require constant medical monitoring.

In nearly 25% of reported cases of primary localized cutaneous nodular amyloidosis, there is coexistence with Sjögren's syndrome (Mori S et al. 2022; Llamas-Molina JM et al.2023). This coexistence has been demonstrated mainly in female patients in the seventh decade of life.

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 millimeters to several centimeters in diameter, brownish to yellow, possibly also white, asymptomatic (especially non-pruritic) nodules and plaques. Larger "callous" plaques may be found on the soles of the feet. In the genital area (e.g. glans penis), large, bulging plaques or nodules may occur and must be distinguished from condylomata acuminata.

After central regression, formation of anetoderma-like foci with yellowish shimmering fatty tissue is possible (see first description by H. Gottron with the additional designation "atrophicans").

About 7% of cases later progress to 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 homogeneously infiltrate under a normal to discretely flattened epidermis the entire corium up to the subcutis leaving out the stratum papillare. Chronic inflammatory infiltrates with plasma cells are found in their vicinity. 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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Anetoderma: no nodule formation

Nevus lipomatosus: soft skin-colored nodule formation, typical consistency of fatty tissue

Malignant lymphoma: firm, broad-based, moderately fast-growing nodules. Histology is diagnsotic.

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, histopathologic, and ultrastructural findings. Dermatol 27: 16-25
  7. Jievaltaite V et al (2018) Primary localized cutaneous nodular amyloidosis of the cheeks. Act 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. Llamas-Molina JM et al.(2023) Localized cutaneous nodular amyloidosis: A Specific Cutaneous Manifestation of Sjögren's Syndrome. Int J Mol Sci 24:7378.
  12. Mori S et al (2022) Localized cutaneous nodular amyloidosis associated with Sjögren's syndrome and thymoma. Eur J Dermatol. 32:408-410.
  13. Nakai N et al (2014) Nodular primary localized cutaneous amyloidosis in a patient with pulmonary sarcoidosis. Indian J Dermatol 59:307-308.
  14. Reidel U et al (2015) Solitary lesion on the glans penis. JDDG 13: 703-705
  15. Ritchie SA et al (2014) Primary localized cutaneous nodular amyloidosis of the feet: a case report and review of the
  16. literature. Cutis 93:89-94
  17. 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: 07.08.2023