מתוך medicontext.co.il
By Emma Hitt, PhD
ATLANTA (Reuters Health) – The US Centers for Disease Control and Prevention (CDC) is reporting a previously undescribed fibrosing skin condition among a small number of renal disease patients.
The condition was first identified when 8 of 265 kidney transplant recipients at a hospital in California were diagnosed with the condition from May 1997 to November 2000, after receiving a transplant.
"On clinical examination, the patients had fibrotic skin lesions histologically resembling scleromyxedema on their distal extremities and trunk, resulting in severe contractions and limited mobility," CDC researchers report in the January 18th issue of its Morbidity and Mortality Weekly Report.
However, these patients lacked the usual IgG lambda paraprotein associated with scleromyxedema, which prompted further investigation. As of January 2002, 49 patients have been identified in the US and Europe.
"Although having renal disease is not a part of the case definition, all patients have had underlying renal disease; approximately half have had renal transplantation," CDC researchers note.
"No consistently effective treatment exists; however, several patients have improved," they add.
Although the cause is unknown, Dr. Michelle Goveia with the CDC's Epidemiology Program Office points out that since all known cases have renal disease "it is possible that some substances either being released or not being filtered by the damaged kidney are increasing in the body, leading to altered physiologic state."
"It is most likely that the etiology of this condition is multi-factorial," Dr. Goveia told Reuters Health, "perhaps involving genetic predisposition to abnormal fibroblast production, which is triggered under conditions of immune suppression and renal disease," she said.
While the condition most closely resembles scleromyxedema, several differences exist, according to Dr. Goveia. "The renal disease comes before this skin condition, where in typical scleromyxedema the reverse is usually true," she said.
"Moreover, in this new condition, the distal extremities and trunk are usually affected and the histopathology appears different, and the affected patients' serum does not display the monoclonal IgG lambda paraprotein that is associated with scleromyxedema."
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