Localized Cutaneous Nodular AmyloidosisE85.8

Gottron, 1950; Degos, 1961

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.

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 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).

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

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.

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.

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

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