Amyloidosis cutaneous nodular E85.8

Gottron, 1950; Degos, 1961

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.

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).

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

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).

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

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

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