Lipocalcinogranulomatosis

The Lipokalzinogranulomatose is a very rare disease with the main features of subcutaneous calcification near large joints .

Synonyms are: Lipocalcinogranulomatose Teutschlaender; Lip (o) idcalcinosis progrediens; tumorous calcinosis; Teutschlander Syndrome; Latin Calcinosis universalis; Lipocalcinogranulomatosis; Lipoid calcinosis progrediens; calcinosis dystrophica disseminated; calcinosis lipogranulomatosa progrediens, lipocalcinosis progrediens ; english tumoral calcinosis; lipoid calcium gout; Teutschlander's syndrome

The first description comes from the year 1935 by Otto Teutschländer.

distribution

The frequency is unknown; more than 200 people have been reported to be affected. An - at least partially - autosomal dominant inheritance is assumed. Mostly Africans are affected, there should be regional clusters. The female gender is more often affected.

root cause

A benign ( hereditary ) disorder of the calcium and phosphate balance is assumed.

Clinical manifestations

Clinical criteria are:

Manifestation especially in the 1st and 2nd decades of life
Pronounced calcium deposits in the connective tissue dermal and subcutaneous as well as in the muscles without previous damage
If the accompanying inflammatory reaction progresses, perforation can occur with the emergence of milky, calcium-containing to purulent substances (lipocalcinogranulomatosis)
in several places mostly near the joints or starting from bursa
possibly preceding exanthema
fast-growing calcifying connective tissue tumors develop
after puberty tendency to improve

Furthermore, a can gingivitis , hoarseness or lime stripes in the retina , possibly in the great vessels occur.

diagnosis

The excretion of calcium is reduced, in the blood serum there are increased cholecalciferol values with normal potassium values . A hyperphosphatemia may, but need not be present. The calcifications can be detected by imaging , most clearly on the X-ray .

Differential diagnosis

The following are to be distinguished:

therapy

A targeted treatment is not known. Pronounced inflammatory reactions can be contained with glucocorticoids , painful lesions can be surgically removed.

course

The course is usually chronic, and secondary infections can be fatal.

literature

P. Binnani, V. Aggarwal, MM Bahadur, N. Fulara: Tumoral calcinosis (Teutschlander disease) in a dialysis patient. In: Indian journal of nephrology. Volume 18, number 3, July 2008, pp. 122-124, doi: 10.4103 / 0971-4065.43692 , PMID 20142918 , PMC 2813132 (free full text).
MH Baums, HM Klinger, S. Otte: Morbus Teutschländer - a massive soft-tissue calcification of the foot in a patient on long-term hemodialysis. In: Archives of orthopedic and trauma surgery. Volume 123, Number 1, February 2003, pp. 51-53, doi: 10.1007 / s00402-002-0447-y , PMID 12582798 .
F. Kuchenbauer, P. Biberthaler, H. Stöger, W. Mutschler: [Teutschländer disease. A rare benign differential diagnosis in proliferating space-occupying lesions of soft tissues]. In: The trauma surgeon. Volume 105, Number 6, June 2002, pp. 572-575, doi: 10.1007 / s00113-001-0397-9 , PMID 12132199 . !!

Individual evidence

↑ Encyclopedia Dermatology
↑ ^a ^b ^c ^d ^e R. Witkowski, O. Prokop, E. Ullrich: In: Lexicon of Syndromes and Malformations: Causes, Genetics and Risks , p. 676, 1995, ISBN 978-3-642-97629-2 , online ISBN 978-3-642-97628-5 doi: 10.1007 / 978-3-642-97628-5_24
^ O. Teutschländer: About progressive lipogranulomatosis of the muscles. At the same time contributes to the pathogenesis of myopathia osteoplastica progressiva. In: Klinische Wochenschrift Vol. 14, p. 451, 1935
↑ ^a ^b ^c T. Mumme, H. Griefingholt, B. Schmidt-Rohlfing, R. Müller-Rath, A. Kochs: Morbus Teutschländer. A rare benign differential diagnosis of proliferative masses in para-articular soft tissue. In: The orthopedist. Volume 33, Number 7, July 2004, pp. 829-835, doi: 10.1007 / s00132-004-0641-4 , PMID 14999446 .
↑ ^a ^b ^c ^d ^e ^f O. Braun-Falco et al .: Calcinosis. In: O. Braun-Falco, G. Plewig, HH Wolff, RKS Winkelmann (eds.): Dermatology. 1991, pp. 913-917, doi: 10.1007 / 978-3-662-00181-3_45 , ISBN 978-3-662-00183-7 online ISBN 978-3-662-00181-3
↑ ^a ^b W. Schuster, D. Färber (editor): Children's radiology. Imaging diagnostics. Vol. I, 2nd edition, Springer 1996, p. 665, ISBN 3-540-60224-0. Schuster Vol. I 665

[Altmeyer-1] Encyclopedia Dermatology

[Wit-2] R. Witkowski, O. Prokop, E. Ullrich: In: Lexicon of Syndromes and Malformations: Causes, Genetics and Risks , p. 676, 1995, ISBN 978-3-642-97629-2 , online ISBN 978-3-642-97628-5 doi: 10.1007 / 978-3-642-97628-5_24

[3] O. Teutschländer: About progressive lipogranulomatosis of the muscles. At the same time contributes to the pathogenesis of myopathia osteoplastica progressiva. In: Klinische Wochenschrift Vol. 14, p. 451, 1935

[Mumme-4] T. Mumme, H. Griefingholt, B. Schmidt-Rohlfing, R. Müller-Rath, A. Kochs: Morbus Teutschländer. A rare benign differential diagnosis of proliferative masses in para-articular soft tissue. In: The orthopedist. Volume 33, Number 7, July 2004, pp. 829-835, doi: 10.1007 / s00132-004-0641-4 , PMID 14999446 .

[Braun-5] ↑ ^a ^b ^c ^d ^e ^f O. Braun-Falco et al .: Calcinosis. In: O. Braun-Falco, G. Plewig, HH Wolff, RKS Winkelmann (eds.): Dermatology. 1991, pp. 913-917, doi: 10.1007 / 978-3-662-00181-3_45 , ISBN 978-3-662-00183-7 online ISBN 978-3-662-00181-3

[Schuster-6] W. Schuster, D. Färber (editor): Children's radiology. Imaging diagnostics. Vol. I, 2nd edition, Springer 1996, p. 665, ISBN 3-540-60224-0. Schuster Vol. I 665