Oral phosphate binders in the treatment of pseudoxanthoma elasticum

Daniel W. Sherer, Giselle Singer, Jaime Uribarri, Robert G. Phelps, Allen N. Sapadin, K. Bailey Freund, Lawrence Yanuzzi, Wayne Fuchs, Mark Lebwohl

Research output: Contribution to journalArticlepeer-review

45 Scopus citations

Abstract

Background: Pseudoxanthoma elasticum (PXE) is a systemic connective tissue disorder involving elastic fiber calcification and fragmentation with major clinical manifestations occurring in the cutaneous, ocular, and cardiovascular systems. Normalization of the serum calcium-phosphate product through hemodialysis in a previous patient with perforating periumbilical PXE and elevated serum phosphate resulted in regression of skin lesions. Objective: We sought to study the effect of pharmacologically limiting the intestinal absorption of phosphate in patients with PXE. Methods: Patients received baseline skin examinations, target skin lesion evaluation, and photography; renal function tests and serum calcium and phosphate levels; urine calcium, phosphate, and creatinine levels; skin biopsy; and eye examinations and indocyanine-green angiography. Patients were treated with aluminium hydroxide tablets or liquid and returned every 2 to 4 months for skin photography and lesion evaluation. Repeated skin biopsies were performed on clinically improved target sites. Ophthalmologic evaluation was obtained at yearly intervals. Results: Of 6 patients, 3 showed significant clinical improvement of skin lesions and all 3 of these patients showed histopathologic regression of disease in their target lesions. No deterioration of eye disease was seen in any of the 6 patients at 1-year follow-up. Conclusion: Our results demonstrate that the calcification seen in PXE may be reversible in some patients. This could hold true for eye and vascular lesions and for skin. Further studies supporting these results could reveal the first real treatment option for PXE.

Original languageEnglish
Pages (from-to)610-615
Number of pages6
JournalJournal of the American Academy of Dermatology
Volume53
Issue number4
DOIs
StatePublished - Oct 2005

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