Familial Generalized Dyschromic Amyloidosis Cutis

Abstract

A generalized form of primary cutaneous amyloidosis was found in 2 siblings when microscopic examination was carried out on areas of scattered hypopigmentation. Multiple biopsies from different sites of the skin suggested that the pigmentary disorder was probably a process independent of the amyloidosis. One of the siblings died of interstitial pulmonary fibrosis, congenital pulmonary stenosis and diabetes mellitus. Post mortem examination of the lungs showed the presence of few amyloid deposits in the diffusely fibrotic tissue, suggesting that these were secondary to the chronic pulmonary disease. Because of the absence of amyloid involvement in parenchymatous organs and the absence of cutaneous signs of systemic amyloidosis as macroglosia or petechiae, this case suggests that primary cutaneous amyloidosis is distinctly different from the cutaneous manifestation of primary systemic amyloidosis.