Amyloidosis macular cutaneous E85.4

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 07.08.2023

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friction amyloidosis; interscapular skin amyloidosis; macular amyloidosis; Macular cutaneous amyloidosis; Macular skin amyloidosis; Primary cutaneous macular amyloidosis; Primary localized cutaneous amyloidosis; Skin amyloidosis interscapular; Skin amyloidosis macular

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Palitz & Peck 1952

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Cutaneous amyloidosis with mostly extensive, little or slightly itchy, brownish or greyish-brownish patches or plaques. On closer inspection, a ribbed surface structure can be seen. The stains/plaques are usually only perceived as "cosmetically" disturbing discolorations of the skin. The risk of developing systemic amyloidosis is about 1%.

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m:w=1:1 (some studies indicate a slight preference for the female sex); first manifestation in early and middle adulthood,

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Mainly interscapular, but also on arms and over large areas of the trunk.

Clinical features
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Blurred, 2.0-10 cm large, hyperpigmented, grey-brown or medium-brown, slightly itchy, but also not very symptomatic spots or plaques, which are also large and map-like due to confluence. Scaling is missing. On close inspection a blunt ribbed surface structure of the areas becomes visible. It is not uncommon for the localisation of the lesions to be associated with permanent trauma due to constant, firm brushing of the skin (friction amyloidosis). The relationship to the notalgia paraesthetica is not certain.

In the flexures areal infiltrates of dirty grey-brown colour with signs of lichenification.

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Acanthosis, papillomatosis, perivascular lymphohistiocytic infiltrates, severe pigment incontinence.electron microscopy: clumpy amyloid deposits, extensive basement membrane destruction.

Direct Immunofluorescence
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Amyloid, additional positive reaction with cytokeratin antibodies.

Differential diagnosis
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Hyperpigmentation, postinflammatory: Acute event, residual hyperpigmentation; these do not cause itching.

Lichen simplex chronicus: The leading symptom is itching, which is perceived as severe and distressing. There are 10-15 cm roundish or oval, rarely elongated or striate plaques, typically with a three-zone structure: planar central lichenification, marginal lichenoid nodules, peripheral hyperpigmentation. The plaques are composed of 0.1-0.2 cm, solid, planar, gray to brown-reddish or skin-colored, not infrequently scratched, lichenoid papules.

Atopic dermatitis: signs of atopy (rhinitis, bronchial asthma); chronic pruritic dermatitis, no wesnetic hyperpigmentation. IgE elevated.

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Symptomatic treatment of itching.

External therapy
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Trial of glucocorticoids occlusive with ointments/greasy ointments, e.g. 0.1% mometasone (e.g. Ecural ointment/greasy ointment), 0.25% prednicarbate (e.g. Dermatop ointment/greasy ointment). Often only moderately successful. Alternative: Injection intrafocally with triamcinolone acetonide crystal suspension(e.g. Volon A diluted 1:2-1:3 with local anesthetics such as Scandicaine). Long-lasting therapy is necessary.

If necessary, try therapy with antipruriginosa such as polidocanol shaking mixture R200, again the results are moderate.

Trial local DMSO therapy: After thorough cleansing of the affected skin areas, apply DMSO 50% R079 1 time/day. Leads to a suppression of the itching, no effect on the amyloid deposits. See also Lichen amyloidosus.

Internal therapy
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In individual cases, systemic retinoids, e.g. acitretin, may be used.

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Highly chronic course, no tendency to spontaneous remission.

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  3. Cornejo KM et al (2015) Nodular Amyloidosis Derived From Keratinocytes: An Unusual Type of Primary Localized Cutaneous Nodular Amyloidosis. Am J Dermatopathol 37:e129-133
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  5. Kaltoft B et al (2013) Primary localised cutaneous amyloidosis--a systematic review. Dan Med J 60:A4727
  6. Krishna A et al (2012) Study on epidemiology of cutaneous amyloidosis in northern India and effectiveness of dimethylsulphoxide in cutaneous amyloidosis. Indian Dermatol Online J 3:182:186
  7. Mullins RF (2000) Drusen associated with aging and age-related macular degeneration contain proteins common to extracellular deposits associated with atherosclerosis, elastosis, amyloidosis, and dense deposit disease. FASEB J 14: 835-846
  8. Özkaya-Bayazit et al (1997) Local DMSO treatment of macular and papular amyloidosis. Dermatologist: 48: 31-37
  9. Palitz LL, Peck S (1952) Amyloidosis cutis: a macular variant. Arch Dermatol Syph 65: 451-457.
  10. Ritter M et al (2003) Localized amyloidosis of the glans penis: a case report and literature review. J Cutan Pathol 30: 37-40


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Last updated on: 07.08.2023