Amyloidosis is a disease complex sassociated with deposition of insoluble fibrillar protein in various 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 primary systemic amyloidosis have been reported in Korea.
A 56-year-old male was seen for facial purpuras, macroglossia, myalgia 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 birefringence on Congo red staining when viewed under polarized light. Electron microscopic examination showed that nonbranching and nonanastomosing straight fibrils are irregularly arranged around the collagen fibers.
Therefore, he was diagnosed with primary systemic amyoidosis by the characteristic clinical, histopathologic and ultrastructural findings. (Kor J Dermatol 1993;31(6) : 963-967)
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