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June 1993

Fibrillar Protein Deposits With Tubular Substructure in a Systemic Disease Beginning as Cutis Laxa

Author Affiliations

From the Department of Dermatology (Dr Niemi) and the Third Department of Medicine (Dr Somer), Helsinki (Finland) University Central Hospital; the Institut für Ultrastrukturforschung der Haut, University of Heidelberg (Germany) (Dr Anton-Lamprecht); the Department of Anatomy, Helsinki University (Dr Virtanen); the Department of Pathology, Aurora Hospital, Helsinki (Dr Suomalainen); and the Max-Planck-Institut für Biochemie, Martinsried bei München (Germany) (Dr Linke).

Arch Dermatol. 1993;129(6):757-762. doi:10.1001/archderm.1993.01680270095013

• Background.—  The homogeneous material found in the skin is commonly identified as amyloid. We describe a previously unknown disease that is caused 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 ultrastructurally composed of fibrillar material with a tubular substructure. Immunohistologically, the material was tested using a large panel of antibodies, 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 syndromes by ultrastructural examination. Further biochemical analysis is necessary to identify the origin of the material.(Arch Dermatol. 1993;129:757-762)

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