Calcinosis dystrophica disseminated E83.5

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

calcinosis interstitialis; Calcinosis lipogranulomatosa progressive; calcinosis metabolica unversalis; disseminated dystrophic calcinosis; German Nationality Syndrome; Lipocalcinogranulomatosis; Lipoid lime gout; Progressive lipocalcinosis

History
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Germany, 1935; Inclan et al., 1943

Definition
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Rare disease with deposition of calcium salts in the cutaneous and subcutaneous tissue as well as the musculature without tangible calcium or phosphate metabolic disorder.

Etiopathogenesis
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Localization
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Extremities here around the large joints, also on the trunk.

Clinical features
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Mostly symmetrical, multiple calcifications in the skin. Inflammatory redness, perforation, discharge of a calcareous, creamy or purulent appearing material. Poorly healing ulcerations, formation of retracted scars. Possibly painful restriction of movement in the case of calcified deposits near joints.

Histology
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Calcareous salt deposits in the subcutaneous and cutaneous tissue.

Differential diagnosis
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Myositis ossificans; gout; other forms of calcinosis cutis.

Therapy
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Treatment of the underlying disease. As far as possible surgical removal of circumscribed or painful calcific knots. In case of ulceration, wound cleansing and granulation promoting measures, see wound treatment below. In case of severe inflammatory changes glucocorticoids can be applied externally such as 0.25% prednicarbate (e.g. Dermatop cream).

Progression/forecast
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Chronic progressive course with remissions.

Literature
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  1. Inclan A, Leon P, Gomez CM (1943) Tumoral calcinosis. JAMA 121: 490-495
  2. German O (1935) About progressive lipogranulomatosis of the musculature. At the same time a contribution to the pathogenesis of osteopathia osteoplastica progressiva. Clin weekday 14: 451

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Last updated on: 29.10.2020