FIBRILLAR PROTEIN DEPOSITS WITH TUBULAR SUBSTRUCTURE IN A SYSTEMIC-DISEASE BEGINNING AS CUTIS LAXA

Background.-The homogeneous material found in the skin is commonly ide ntified as amyloid. We describe a previously unknown disease that is c aused by proteinaceous deposits and that does not fulfill the criteria of the earlier recognized amyloid diseases. Observations.-The unusual deposits, which were initially found in the dermis, were ultrastructu rally composed of fibrillar material with a tubular substructure. Immu nohistologically, the material was tested using a large panel of antib odies, and the results revealed that it was unlike any commonly known proteinaceous material. The deposits later spread to other organs and disturbed the vital functions of the body. Conclusions.-We describe a unique syndrome characterized by fibrillar extracellular deposits that was recognized and differentiated from other similar clinical syndrom es by ultrastructural examination. Further biochemical analysis is nec essary to identify the origin of the material.