OxalosisE74.8

Author:Prof. Dr. med. Peter Altmeyer

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Last updated on: 06.04.2021

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Synonym(s)

Hyperoxaluria

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DefinitionThis section has been translated automatically.

Disease characterized by hyperoxaluria and deposition of oxalate salts in the skin and other organs.

ClassificationThis section has been translated automatically.

  • One distinguishes:
    • Primary oxalosis type I (OMIM:259900)
    • Primary Oxalosis Type II (OMIM: 260000)
    • Secondary enteral oxalosis
    • Secondary idiopathic oxalosis.

EtiopathogenesisThis section has been translated automatically.

Primary oxalosis type I and type II: Autosomal recessive inherited dysfunctions in the oxalic acid metabolism are discussed. In type I, the liver-specific peroxysomal enzyme "glyoxalate transferase" is diminished or absent, in type II the enzyme "D-glycerol dehydrogenase".

In both disorders, there is increased glyoxalate, which is not further degraded toCO2 but oxidized to oxalate. With normal kidney function and ample water intake, the oxalic acid can be excreted in the urine (oxaluria). In most cases, however, it binds to calcium and forms kidney and bladder stones from calcium oxalate, and calcium oxalate is deposited in bones or the skin, among other places.

Secondary enteric oxalosis: Usually calcium deficiency as a result of chronic diarrhea or intestinal surgery. Excess oxalic acid cannot be bound to calcium and is therefore eliminated renally. Triggering factors include enteritis regionalis, pancreatic insufficiency, liver cirrhosis, and surgical procedures.

Secondary idiopathic oxalosis: Passenger or permanent hyperoxaluria or formation of calcium oxalate deposits due to diet with excess oxalic acid foods (e.g. spinach).

Clinical featuresThis section has been translated automatically.

Integument: Isolated or multiple, partly miliary, coarse, whitish papules in the acral region of the upper limb. Occasional livedo patterns of the skin (due to deposits of crystals in the vessel walls) or circumscribed skin necroses.

HistologyThis section has been translated automatically.

Detection of birefringent oxalate crystals in corium (more rarely in the subcutis) in a star-shaped or rhomboid arrangement. In HE-preparation the deposits turn yellow to brown-yellow. Kossa staining clearly positive.

Differential diagnosisThis section has been translated automatically.

Primary and secondary calcinosis of the skin.

TherapyThis section has been translated automatically.

A treatment of hereditary oxalosis is not possible. Oxalate formation can be reduced by increased administration of pyridoxol (vitamin B6) or pyridoxal phosphate. In severe cases (e.g. in cases of terminal renal failure) haemodialysis.

LiteratureThis section has been translated automatically.

  1. Galimberti RL et al (1999) Fatal cutaneous necrosis in a hemodialyzed patient with oxalosis. Int J Dermatol 38: 918-920
  2. Johnson SA et al (2002) Primary hyperoxaluria type 2 in children. Pediatric nephrol 17: 597-601
  3. Halil O et al (2003) Oxalosis: an unusual cause of leucoerythroblastic anaemia. Br J Haematol 122: 2
  4. Matsumura Y et al (2001) Calcinosis cutis in a patient with primary hyperoxaluria due to hepatic enzyme deficiency. J Dermatol 28: 578-579
  5. Shih HA et al (2000) Livedo reticularis, ulcers, and peripheral gangrene: cutaneous manifestations of primary hyperoxaluria. Arch Dermatol 136: 1272-1274
  6. Winship et al (1991) Primary oxalosis - an unusual cause of livedo reticularis. Clin Exp Dermatol 16: 367-370

Authors

Last updated on: 06.04.2021

Author: Prof. Dr. med. Peter Altmeyer