This entity also is termed amyloidosis cutis nodularis atrophicans. By definition, NLCA describes a primary disease of the skin, although lesions occasionally appear similar to the skin manifestations of systemic amyloidosis. In NLCA, the amyloid is believed to derive from local plasma cells, in contrast to lichenoid or macular amyloidosis, which have keratinocyte-derived amyloid. Progression to systemic disease is unlikely. Several occurrences have been associated with Sj gren syndrome.
Epidemiologic data can be difficult to establish when so few patients are reported.
Although numbers are small, reports indicate that NLCA is likely to occur during adulthood. Patients can present with single or multiple lesions. In some reports, lesions were present for several years before patients sought medical attention. Troublesome aspects of NLCA primarily result from patient concerns about appearance, although plaques eventually fissured in one patient in whom the plantar aspects of the feet were affected. Nodules vary from a few millimeters to a few centimeters.