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Lipocalcinogranulomatosis

Lipocalcinogranulomatosis (synonym: interstitial calcification, lipocalcinosis, calcareous gout) is a rare chronic disease characterized by the deposition of calcium salts in the skin, subcutaneous fatty tissue, muscles, articular bags and tendon sheaths in the absence of hypercalcemia. More often, the lesion is limited to any one part of the body, but it can also be universal.

Clinically, the disease is sometimes manifested by painful indurations, which on radiographs have the form of clearly defined intense shadows. Two clinical forms of lipocalcinogranulomatosis are observed: muscular-hygromatous (with predominant damage to the articular bags) and subcutaneous (with predominant damage to the subcutaneous fat). The muscular-hygromatous form can lead to the development of contractures, since the process is accompanied by severe scarring. In subcutaneous forms, the skin over the nodes sometimes ulcerates, fistulas appear, from which crummy or mushy masses containing lime are emitted.

Deposition of lime in lesions is preceded by dystrophic changes in connective, adipose or muscle tissue, which, according to O. Teutschlander, is associated with local disorders of cholesterol metabolism that develop as a result of diencephalic endocrine regulation disorders. The hereditary constitutional nature of lipocalcinogranulomatosis is emphasized by many authors. The disease sometimes develops after a skull injury or is combined with various trophic (muscular dystrophy, osteoporosis) or hormonal (impotence) disorders.

In the case of the muscular-hygromatous form, the process begins with an uneven swelling and sudanophilia of muscle fibers, the myoplasm of which becomes homogeneous. Then lime is deposited in the muscle fibers, and lipogranulomas develop, which are further exposed to sclerosis and hyalinosis. In the subcutaneous form, first, the fatty tissue disintegrates with the formation of lipogranulomas. Then lime is deposited, which is also accompanied by sclerosis and hyalinosis. As the process progresses due to the necrosis of significant areas of the affected tissue, cavities appear that contain tissue debris and calcareous masses. On the periphery of the cavities, granulation tissue grows with foci of xanthomatosis, which is further fibrosed and hyalinized. Sometimes as a result of the breakthrough of cavities outward, fistulas are formed.

There is no effective treatment. With limited subcutaneous forms, the affected areas are excised. Relapse is possible.

See also Calcinosis.