Acquired Pseudoxanthoma elasticum-like syndrome in β-thalassaemia patients

Pseudoxanthoma elasticum (Grönblad-Strandberg syndrome).

Pseudoxanthoma elasticum (PXE) is a rare disorder of elastin which primarily involves the skin, cardiovascular system, and eyes, but also manifests in the central nervous system, skeleton, and gastrointestinal tract. Rigall first described the cutaneous lesions of PXE in 1881 and thought the disorder was akin to the xanthomatoses. In 1896, Darier2 showed the typical histological changes in elas...

Acquired pseudoxanthoma elasticum presenting after liver transplantation.

BACKGROUND Pseudoxanthoma elasticum (PXE) is thought to be a metabolic disorder resulting from mutations in the gene encoding the cellular transporter, ABCC6, which is primarily expressed in liver and kidney. We encountered 3 patients who developed clinical and histopathological evidence of PXE after liver transplantation, suggesting that PXE could have been acquired from the transplanted organ...

Pseudoxanthoma elasticum

Pseudoxanthoma elasticum (PXE) is a genetic metabolic disease with autosomal recessive inheritance caused by mutations in the ABCC6 gene. The lack of functional ABCC6 protein leads to ectopic mineralization that is most apparent in the elastic tissues of the skin, eyes and blood vessels. The clinical prevalence of PXE has been estimated at between 1 per 100,000 and 1 per 25,000, with slight fem...

Pseudoxanthoma elasticum

Pseudoxanthoma elasticum-like papillary dermal elastolysis*

An Bras Dermatol. 2017;92(6):891-900. According to the literature, hospitalization is required in some cases, which did not happen in our case because of the early intervention (Table 2).5 Considering a reduced risk of agranulocytosis development and in accordance with Carneiro et al. (2011),5 our aim was not to question DDS therapy for leprosy, but to stimulate clinical awareness of its risks ...