Autofluorescence Imaging of the Skin Is an Objective Non-Invasive Technique for Diagnosing Pseudoxanthoma Elasticum

Abstract

Pseudoxanthoma elasticum (PXE) is a rare multisystemic autosomal recessive connective tissue disease. In most cases, skin manifestations of PXE are the first to develop, followed later by severe ocular and cardiovascular complications. In our present study, in addition to dermoscopy, we introduced novel techniques, autofluorescence (AF) and diffuse reflectance (DR) imaging for the assessment of affected skin sites of five PXE patients. PXE-affected skin areas in most skin sites showed a previously observed pattern upon dermoscopic examination. With the novel imaging, PXE-affected skin lesions displayed high AF intensity. During our measurements, significantly higher mean, minimum and maximum AF intensity values were found in areas of PXE-affected skin when compared to uninvolved skin. Conversely, images acquired with the use of 660 and 940 nm illumination showed no mentionable difference. Our results demonstrate that AF imaging may be used in the in vivo diagnostics and quantification of the severity of the skin lesions of PXE patients. In addition, it is a safe, fast and cost-effective diagnostic method. AF imaging may be also used to objectively monitor the efficacy of the possible novel therapeutic approaches of PXE in the future.

Quantitative Multispectral Imaging Differentiates Melanoma from Seborrheic Keratosis

“…An LED-based MSI device was recently successfully introduced for the detection of recurring skin cancers [43,44] and proved to be applicable in the differentiation of different tumor types from benign lesions based on their mean intensity of autofluorescence (AF) [45]. In addition, MSI has been also utilized for the detection of rare skin disorders [46,47].…”

Serum Calcification Propensity T50 Associates with Disease Severity in Patients with Pseudoxanthoma Elasticum

“…As for the Phenodex skin scores, the absence of significant associations with T50 in the current study might be attributed to the fact that the severity of cutaneous alterations in PXE is only partially reflected by the Phenodex S score as, for example, redundant skin folds due to loss of elasticity might not always reflect higher disease activity compared to extensive papules or plaques. Future studies should, therefore, focus on novel evaluation and grading methods of skin lesion severity, for example, by implementing in vivo autofluorescence and dermoscopy techniques [42,43]. Potential associations between Phenodex cardiac and gastrointestinal scores and T50 could not be adequately evaluated in our current PXE patient population due to the low prevalence of Phenodex-scored events such as myocardial infarction or stomach bleeding.…”

Pseudoxanthoma elasticumban szenvedő betegek multidiszciplináris ellátása

“…A dermatoszkópia széles körben elérhető in vivo bőrgyógyászati diagnosztikai eszköz, a PXE dermatoszkópos jellegzetességeinak leírásáról már több tanulmány áll rendelkezésünkre [40,41]. A dermatoszkópos képen a halvány livid-vörös háttérből finom retikularis érstruktúrák tűnnek elő, közöttük különböző elrendeződésű mintázatot mutató ovális sárgás-fehér globulusok helyezkednek el (5. ábra) [40].…”

“…A dermatoszkópos képen a halvány livid-vörös háttérből finom retikularis érstruktúrák tűnnek elő, közöttük különböző elrendeződésű mintázatot mutató ovális sárgás-fehér globulusok helyezkednek el (5. ábra) [40]. Munkacsoportunk LED alapú autofluoreszcens nem invazív képalkotó eszköz alkalmazásával kontrasztosabb, részletgazdagabb módon jelenítette meg a dermatoszkópos specifikus struktúrákat a betegek bőrén [41].…”