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February 1994

Fibrillary Protein Deposits With Tubular Substructure in a Systemic Disease Beginning as Cutis Laxa-Reply

Author Affiliations

Department of Dermatology Helsinki University Central Hospital Meilahdentie 2 SF-00250 Helsinki, Finland Ingrun Anton-Lamprecht, PhD Heidelberg, Germany

Helsinki, Finland

Munich, Germany

Arch Dermatol. 1994;130(2):256-257. doi:10.1001/archderm.1994.01690020126027

Drs Pakasa and Cohen suggested similarities between immunotactoid glomerulopathy1 and the findings in a patient we described,2 both demonstrating nonamyloid fibrillar protein deposits.

In contrast with the mentioned glomerulopathy, our patient's kidney function was normal throughout his illness, with creatinine and urea being within normal limits and qualitative urinanalyses showing no proteinuria. In 1991, 8 months before his death, a quantitative 24-hour urine sample (1800 mL) was analyzed for the presence of proteins. In 10 mg/L of total protein, 1.3 mg/L of albumin and 8.7 mg/L of globulins were detected. During that period, his serum creatinine level was 73 mmol/L (normal, <115 mmol/L). In conclusion, neither clinical symptoms nor laboratory signs for renal (glomerular) dysfunction were found, and our patient's disease was therefore seen to be different from both fibrillary and ''immunotactoid'' glomerulopathy with regard to renal function.

The ultrastructural features of pathogenic nonamyloid fibrillar proteins as described

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