Criteria were established by consensus of clinicians and researchers at the 2010 biennial research meeting of the PXE Research Consortium. and confirmed at the 2014 meeting These consensus criteria state that definitive PXE is characterized by two pathogenic mutations in the ABCC6 or ocular findings – angioid streaks > 1 DD or peau d’orange in an individual <20 years of age together with skin findings:

Characteristic pseudoxanthomatous papules and plaques on the neck or flexural creases. Diagnostic histopathological changes in lesional skin: Calcified elastic fibers in the mid and lower dermis, confirmed by positive calcium stain

Differential diagnosis
Treatment
There is no confirmed treatment that directly interferes with the disease process.Cosmetic surgery to remove excessive skin has been used to improve aesthetic appearance in PXE patients but because of the non-life-threatening nature of these symptoms, should be used with caution.One of the most critical symptom of PXE is choroidal neovascularization which can lead to deterioration of central vision. Photodynamic therapy has been used as a treatment, but this has been replaced with endothelial growth factor (VEGF) inhibitors (such as bevacizumab, ranibizumab, and aflibercept) with efficacy similar to their use in treatment of age-related macular degeneration.To limit cardiovascular symptoms, reduction of cardiovascular risk factors through lifestyle changes is recommended. Generally clinicians recommend avoidance of non-steroidal anti-inflammatory drugs (NSAIDS) that increase bleeding risk, such as aspirin, and ibuprofen to prevent eye and gastrointestinal bleeding.Formerly, dietary restriction of calcium was tried with no benefit, and in fact accelerated mineralization in mice.
In medicine, confusion is the quality or state of being bewildered or unclear. The term "acute mental confusion" is often used interchangeably with delirium in the International Statistical Classification of Diseases and Related Health Problems and the Medical Subject Headings publications to describe the pathology. These refer to the loss of orientation, or the ability to place oneself correctly in the world by time, location and personal identity. Mental confusion is sometimes accompanied by disordered consciousness (the loss of linear thinking) and memory loss (the inability to correctly recall previous events or learn new material). Etymology
The word confusion derives from the Latin word, confundo, which means "confuse, mix, blend, pour together, disorder, embroil." Causes
Confusion may result from drug side effects or from a relatively sudden brain dysfunction. Acute confusion is often called delirium (or "acute confusional state"), although delirium often includes a much broader array of disorders than simple confusion. These disorders include the inability to focus attention; various impairments in awareness, and temporal or spatial dis-orientation. Mental confusion can result from chronic organic brain pathologies, such as dementia, as well. Other
Differential diagnosis
The most common causes of drug induced acute confusion are dopaminergic drugs (used for the treatment of Parkinsons disease), diuretics, tricyclic, tetracyclic antidepressants and benzodiazepines or alcohol. The elderly, and especially those with pre-existing dementia, are most at risk for drug induced acute confusional states. New research is finding a link between vitamin D deficiency and cognitive impairment (which includes "foggy brain").