INNOVATIVE MULTIMODAL IMAGING APPROACHES FOR THE ASSESSMENT OF THE SKIN LESIONS IN PSEUDOXANTHOMA ELASTICUM
Farkas Klára Zsuzsanna
KÁROLY RÁCZ CONSERVATIVE MEDICINE PROGRAM
Dr. Reusz György
SE Bőr-, Nemikórtani és Bőronkológiai Klinika Tanterme
2024-09-11 14:00:00
Dermatology and Venereology
Dr. Sárdy Miklós
Dr. Medvecz Márta
Dr. Szlávicz Eszter
Dr. Varsányi Balázs
Dr. Becker Dávid
Dr. Szegedi Márta
Dr. Jókai Hajnalka
Pseudoxanthoma elasticum (PXE) is a rare connective tissue disorder that can cause severe ocular and cardiovascular complications.
Novel imaging modalities have been used to investigate and quantify the severity of skin lesions and to reveal the relationship between skin calcification and systemic involvement in PXE patients.
In addition to dermoscopy, we introduced a novel multispectral imaging (MSI) device and used autofluorescence (AF) and diffuse reflectance (DR) imaging techniques, to assess the affected skin sites of five PXE patients. Our results showed significantly higher AF intensity values in areas of PXE-affected skin when compared to uninvolved skin. This suggests that AF imaging can be used to observe skin lesions and objectively monitor the efficacy of novel therapeutic approaches in PXE patients. Furthermore, it is a safe, fast, and cost-effective method that can be applied conveniently.
Ex vivo nonlinear microscopy (NLM) imaging was utilized to assess the extent of skin calcification in 25 PXE patients. Two-photon absorption fluorescence (TPEF) method was used to acquire images for quantitative analyses of ectopic calcification and elastic fibers. After statistical analyses, a significant correlation was found between the calcified area (CA) and the number of affected typical skin sites, disease duration, and ophthalmological and cardiovascular complications. We also found a significant association between higher CA and the presence of macula atrophy and acneiform skin lesions. The findings suggest that NLM imaging may be useful for clinicians to identify PXE patients who are at risk of developing severe systemic complications.
The first sign of PXE often appears on the skin. Consequently, the diagnosis based on skin manifestations may prevent the development of systemic manifestations and slow down the progression of the disease with prompt, early management. The quantitative assessment of the skin lesions with MSI and NLM techniques may provide novel possibilities to monitor the severity of skin lesions, predict the development of systemic involvements and may be implemented into diagnostic approaches used in PXE in the future.
