151. Target-like skin lesions in primary amyloidosis
- Author
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Marius Rademaker, P. Hasleton, S. G. Brear, and L. Doyle
- Subjects
Male ,Pathology ,medicine.medical_specialty ,Proteinuria ,medicine.diagnostic_test ,business.industry ,Amyloidosis ,Connective tissue ,Dermatology ,medicine.disease ,Lesion ,Purpura ,medicine.anatomical_structure ,Biopsy ,medicine ,Blood Vessels ,Humans ,Renal biopsy ,medicine.symptom ,business ,Nephrotic syndrome ,Aged ,Skin - Abstract
SUMMARY A case of primary amyloidosis is described, during the course of which transient, purpuric haloes appeared around long-standing Campbell de Morgan spots, forming target-like lesions of the skin. CASE REPORT A 78-year-old man presented with features of congestive cardiac failure. The only skin lesions at presentation were a number of long-standing Campbell de Morgan spots. Further investigation showed him to have nephrotic syndrome and a renal biopsy revealed extensive amyloid deposition in the glomeruli and small renal arteries. In addition there were persistent clinical, ultrasonic and biochemical signs suggesting hepatic involvement as well as electrocardio graphic, echocardiographic and clinical features suggesting cardiac involvement with amyloid. Serum eleetrophoresis showed no paraproteinaemia, but screening for Bence-Jones proteinuria was positive and the urine contained both kappa and lambda chains. The patient responded well to diuretics. During the course of his follow-up he was noted to develop purpura of the uvula at a time when his clotting studies were normal apart from a slight reduction in his Factor X estimation (62",,, compared with the normal range of 70",, to 130",,). As this lesion faded over the next 7 days, purpura appeared as a halo around a number of his Campbell de Morgan spots (Fig. i). Over the ensuing weeks these initial purpuric areas faded but others appeared, in crops, around different Campbell de Morgan spots. Biopsy of one of the typical lesions showed it to be a haemangioma with amyloid deposition in the walls of the blood vessels (Fig. 2), in the connective tissue between the vessels and in the basement membranes of the sweat glands. The patient died suddenly at home 6 months after initial presentation. These target lesions appeared in crops throughout the course of his illness.
- Published
- 1985