scholarly journals Vascular Effects of Pseudoxanthoma Elasticum

2021 ◽  
Vol 5 (5) ◽  
pp. 453-461
Author(s):  
Erika Hubbard ◽  
Mark Lebwohl
Keyword(s):  
Vascular Disease ◽  
Peripheral Vascular Disease ◽  
Case Reports ◽  
Pseudoxanthoma Elasticum ◽  
The Body ◽  
Ectopic Calcification ◽  
Peripheral Vascular ◽  
Rete Mirabile ◽  
Pubmed Database ◽  
Carotid Rete Mirabile

Background: Pseudoxanthoma elasticum (PXE) is a rare hereditary disease caused by mutations in the ABCC6 gene, characterized by ectopic calcification of connective tissue throughout the body. Vascular conditions associated with PXE have been well-documented in the literature, but to our knowledge, analysis of the myriad of PXE case reports with associated vascular diseases in addition to larger cohort studies, has not been undertaken. Objective: To review existing literature reporting peripheral vascular disease (PVD), cardiovascular disease (CVD), cerebrovascular disease (CeVD), hypertension, and carotid rete mirabile (CRM) in PXE patients as of June 2021. Methods: A search of the PubMed database using the key words “pseudoxanthoma elasticum” and “vascular” was performed. Results: A total of 345 cases of PVD, 97 cases of CVD, and 123 case of CeVD were reported.  Additionally, 88 cases of hypertension and 5 cases of CRM were reported. Conclusions: PXE patients are at risk of developing serious vascular conditions, particularly peripheral vascular disease.  This condition also appears to have some connection to carotid rete mirabile, which is extremely rare in humans.  Further research should be conducted to analyze the connection between PXE and CRM in order to better understand and treat both conditions.

Clinical Update on Pentoxifylline Therapy for Diabetes-Induced Peripheral Vascular Disease

1993 ◽  
Vol 27 (9) ◽  
pp. 1099-1105 ◽  
Author(s):  
R. Keith Campbell
Keyword(s):  
Clinical Trials ◽  
Vascular Surgery ◽  
Vascular Disease ◽  
Peripheral Vascular Disease ◽  
Diabetic Foot ◽  
Case Reports ◽  
Walking Distance ◽  
Diabetic Patients ◽  
Peripheral Vascular ◽  
Economic Implications

OBJECTIVE: To introduce readers to the use of pentoxifylline for diabetes-induced peripheral vascular disease. The article provides background on the pathophysiology of diabetic foot ulcers as well as a review of the literature on the therapeutic use of pentoxifylline for treating this disorder. DATA SOURCES: A MEDLINE search was used to identify pertinent literature, including review articles and case reports. Key index terms included pentoxifylline, diabetic foot ulcer, neuropathy, peripheral vascular disease, and intermittent claudication DATA EXTRACTION: Basic pharmacologic data regarding absorption, distribution, metabolism, and excretion were reported in normal subjects as well as in patients with renal impairment. Open and controlled clinical trials also were analyzed; subjective symptoms were reported. The economic implications also were reported. The pharmacist's role in patient education is discussed. DATA SYNTHESIS: Pentoxifylline 800 mg/d was found to be effective in improving the symptoms in patients with noninsulin-dependent diabetes mellitus, including improvement in walking distance, paresthesia, skin temperature, and subjective overall response. In nondiabetic patients, statistically significant differences in leg-ulcer healing were found between the treatment and placebo groups. Case reports illustrated healing times, which ranged from two weeks to six months. Pentoxifylline use in both insulin-dependent and noninsulin-dependent patients was assessed in clinical trials, with improvement of symptoms in both patient types. CONCLUSIONS: Studies show that pentoxifylline is an alternative to vascular surgery in the management of peripheral vascular disease in diabetic patients, particularly in those with chronic ulceration of the lower extremities that does not heal despite other optimal treatment regimens, including cessation of smoking, maintenance of normoglycemia, elimination of vasoconstrictive drugs, correctly fitted shoes, and appropriate wound care. Pentoxifylline therapy may provide an appropriate, economical treatment modality by reducing the need for hospitalization and vascular surgery.

scholarly journals A Systematic Review of Neurovascular Complications in Patients with Pseudoxanthoma Elasticum

2020 ◽  
Vol 4 (6) ◽  
pp. 491-496
Author(s):  
Colin Domzalski ◽  
Nancy Wei ◽  
Mark Lebwohl
Keyword(s):  
English Language ◽  
Case Reports ◽  
Anticoagulation Therapy ◽  
Pseudoxanthoma Elasticum ◽  
Cardiovascular Complications ◽  
Connective Tissue Disorder ◽  
Ectopic Calcification ◽  
Cerebral Disease ◽  
Organ Systems ◽  
Pubmed Database

Background: Pseudoxanthoma elasticum (PXE) is a rare, hereditary connective tissue disorder characterized by ectopic calcification of multiple organ systems. Cardiovascular complications secondary to accelerated atherosclerosis are well-documented in published literature; however, there is a paucity of studies that reviews serious neurovascular complications including CVA(cerebral vascular accident), TIA(Transient ischemic attack), and intracranial aneurysm development in PXE patients.   Objective: We aim to review current literature reporting cases of serious ischemic and hemorrhagic neurovascular complications in PXE patients as of September 2020 and reexamine current guidelines on anticoagulation and antiplatelet therapy in PXE patients Methods: A search of the PubMed database limited to English language case reports using the key words “pseudoxanthoma elasticum”, “neuro*”, “cerebr*”, “CVA”, “TIA”, and “aneurysm” was performed. Results: A total of 67 cases of cerebral disease were reported; ranging from ages 2-71 years from 12 countries. The most common neurovascular complication seen was ischemic strokes (68.7%) followed by TIAs (20.8%), intracranial aneurysms (6.0%) and lastly hemorrhagic strokes (4.5%). Conclusions: PXE patients have a greater incidence of ischemic strokes compared to the general population. Clinicians should thus carefully weigh the benefits of anticoagulation therapy in patients with known cardiovascular disease against the risk of bleeding.

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