Thibierge-Weissenbach syndrome
ICD code:M34.870:
(cutaneous systemic sclerosis)
Thibierge and Weissenbach (1911) described 'subcutaneous calcareous concretions and scleroderma' in a single patient and found 8 other similar cases in the literature.
A physicochemical study of subcutaneous calcium deposits was performed in a patient with typical Thibierge-Weissenbach syndrome which had begun, ten years previously, with Raynaud's phenomenon and sclerodactylia. Calcium deposits had progressively developed, forming large plates on the arms, flanks and thighs. In the course of the disease they had become ulcerated, exuding a white, chalky material. X-ray films and xerograms demonstrated the extent of these deposits. The calcium-phosphorus balance was normal, and the other clinical and laboratory examinations were concordant with a diagnosis of Thibierge-Weissenbach syndrome (calcinosis). Only systemic corticosteroid therapy slowed down the pathological process; calcium chelating agents (diphosphonate) proved ineffective. The physicochemical study showed that the deposits consisted of carbonated apatite type B with the following formula: Ca8.0(PO4)4.1(CO3)1.2(HPO4)0.8 The mineral substance seemed to be less reactive and more stable than normal bone. This would account for the total lack of effectiveness of all treatments, notably calcium chelating agents, on the course of the deposits. Treatments usually produce bone demineralization before they influence the calcium deposits. This study provides much more detailed information than was hitherto available on calcium deposits and on carbonate ion contents in the apatite phase. The presence of carbonates increases the solubility and reactivity of calcium apatite.
posted by Wild Thing @ 6:10 AM
1 comments
