Tumoral calcinosis with superinfection and sepsis
Oeggerli, P.; Maurer, R.; Kistler, H.
Schweizerische Medizinische Wochenschrift 121(38): 1387-1392
1991
ISSN/ISBN: 0036-7672 PMID: 1925469 Document Number: 367703
Tumoral calcinosis (TC) is a rare syndrome involving ectopic calcifications in the vicinity of the large joints. In about one third of patients the disorder is familial and is associated with hyperphosphatemia, elevation of 1,25-dihydroxy-vitamin-D levels and peculiar dental lesion. TC is inherited in an autosomal-recessive manner. In a normophosphatemic male patient with alcoholic cirrhosis of the liver, TC occurred first in the thoracic wall. Seven years after excision of the first lesion, a large tumor mass around the right hip developed. Infection of the calcified masses with Staph. aureus led to the extensive abscess formation, septicemia and death at the age of 64. Clinical, dental and biochemical examination of the 7 descendants of the patient revealed no constitutional signs of the disease, thus identifying our patient as a sporadic case. Clinical and pathological findings in the patient are discussed and the literature is reviewed.