[Pseudoxanthoma elasticum. Clinically typical but frequently overlooked].
A 52-year-old man had small yellowish, striated or reticular papules since the age of 5 years. These appeared first on the large flexures, and later on the neck, abdomen, penis and the mucosa of the lips. He developed angina at the age of 47 years. A bypass operation, however, did not lead to complete regression of cardiac symptoms. Four years later, the vision in his right eye deteriorated because of arterial occlusion, and the patient was treated with laser coagulation; he was started on long-term treatment with acetylsalicylic acid (250 mg/d) and calcium dobesilate (1,500 mg/d). Despite this, the vision in his left eye also deteriorated progressively. Fundoscopy revealed bilateral pigmentary changes in the macular region, variations in the retinal arterial diameters and "angioid streaks". Pulses were absent in the right radial artery, the left posterior tibial artery and both dorsalis pedis arteries, but there were no trophic changes or symptoms. The serum total cholesterol (246 mg/dl), triglycerides (177 mg/dl) and LDL-cholesterol (193 mg/dl) were only slightly elevated. The diagnosis of pseudoxanthoma elasticum with typical changes in the elastic fibres was confirmed by elastin staining in a biopsy from one of the lesions.
Schäfer
U
U
Medizinische Poliklinik, Universität Münster.
Thiem
P
P
Kolde
G
G
Barenbrock
M
M
Zidek
W
W
D001706:Biopsy; D003937:Diagnosis, Differential; D004550:Elbow; D006801:Humans; D008297:Male; D008875:Middle Aged; D011561:Pseudoxanthoma Elasticum / Q000175:diagnosis* / Q000473:pathology; D012867:Skin / Q000473:pathology