[Calciphylaxis of the skin as a sequela of terminal kidney failure. Report and discussion of 3 cases].
Calciphylaxis is a rare syndrome mostly affecting patients with secondary hyperparathyroidism and in some cases with functional protein C or protein S deficiency. Skin lesions begin as superficial painful patches that progress to deep necrotic lesions. The findings are often misdiagnosed as livedo vasculitis and the prognosis is poor. Histopathologically, calcification in the media of small arteries and arterioles with intimal hyperplasia is seen. It is unclear if this morphologic hallmark is pathogenetic. Therapeutically, the calcium-phosphate product should be lowered pharmacologically by an intensified and modified dialysis treatment and parathyroidectomy.
Klinik für Dermatologie und Venerologie der Medizinischen Universität zu Lübeck.
U
U
Hiss
M
M
Tronnier
P M
PM
Rob
H U
HU
Gutsche
H H
HH
Wolff
D000368:Aged; D002115:Calciphylaxis / Q000209:etiology* / Q000473:pathology / Q000628:therapy; D003922:Diabetes Mellitus, Type 1 / Q000150:complications / Q000473:pathology / Q000628:therapy; D003928:Diabetic Nephropathies / Q000150:complications* / Q000473:pathology / Q000628:therapy; D005260:Female; D006801:Humans; D007676:Kidney Failure, Chronic / Q000150:complications* / Q000473:pathology / Q000628:therapy; D007871:Leg Ulcer / Q000209:etiology* / Q000473:pathology / Q000628:therapy; D008297:Male; D008875:Middle Aged; D009131:Muscle, Smooth, Vascular / Q000473:pathology; D009336:Necrosis; D012867:Skin / Q000098:blood supply / Q000473:pathology; D012871:Skin Diseases / Q000209:etiology* / Q000473:pathology / Q000628:therapy
Calciphylaxis der Haut in Folge einer terminalen Niereninsuffizienz. Bericht und Diskussion von 3 Fällen.