Amyloidosis macular cutaneous E85.4

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%.

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

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

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.

Amyloid, additional positive reaction with cytokeratin antibodies.

• Test with glucocorticoids occlusively with ointments/fatty ointments, e.g. 0.1% mometasone (e.g. Ecural ointment/fatty ointment), 0.25% prednicarbate (e.g. Dermatop ointment/fatty ointment). Often only moderately successful. Alternatively: Injection intrafocally with triamcinolone acetonide crystal suspension(e.g. Volon A diluted 1:2-1:3 with local anaesthetics such as scandicain). Long lasting therapy is necessary.
• If necessary, try therapy with antipruriginosa like Polidocanol shaking mixture R200, the results are also moderate.
• Experimental local DMSO therapy: After thorough cleansing of the affected skin areas apply DMSO 50% R079 once/day. Leads to a cessation of itching, no effect on the amyloid deposits. S.a. Lichen amyloidosus.

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