Primary system amyloidosis / 대한피부과학회지

ABSTRACT

Amyloidosis is a disease complex associated with deposition of inscluble fibrillar protein in vnrious tissues of the body. Since the term, amyloidosis was first introduced by Virchow in 1853, there have been many reports in English literature, but only a few cases of iriinary systemic amyloidosis have been reported in Korea. A 56-year-old male was seven for facial purpuras, macroglossia, myilgia and arthralgia for 5 years. Histologically, the skin biopsy specimen showed amorphous, faintly eosinophilic and fissured masses of amyloid in the upper dermis that demonstrated characteristic green brefringence on Congo red staining when viewed under polarized light. Electron microscopic exanintion showed that nonbranching and nonanastomosing straigh. fibrils are irregularly arranged arouned tlie collagen fibers. Therefore, he was diagncsed with primary systemic amyloidsis by the characteristic clinical, histopathologic and ultrastructural findings.