Clinical Medicine VII. (Poster discussion will take place in the Aula during the Coffee Break)
Introduction: Connective tissue disorders (CTDs) are characterised by demaged collagen and/or elastin fibers, which can lead to various symptoms. At the preliminary stages of CTDs, cutaneous manifestations may indicate the disease and draw attention to the diagnosis. Loss of dermal elasticity and loose, fragile skin can develop and can decrease quality of life. However, in most cases, skin symptoms are unremarkable. Later in life, several organs can be affected, and severe systemic involvement may develop. We can facilitate the diagnosis of CTDs with the use of various novel skin imaging modalities. Thus, the early management of the disorders could delay or stop the progression of systemic complications.
Aims: The aims of this study were to introduce the novel imaging techniques in the assessment of the skin lesions of patients with CTDs and describe the clinical applications of these modalities.
Methods: Nonlinear optical (NLO) microscopy is a novel method which can be utilized for the high-resolution imaging of the skin. Among these techniques, two-photon absorption fluorescence (TPF) microscopy can visualize elastin fibers and calcium deposits, while second-harmonic generation (SHG) microscopy can be used for the imaging of the morphology of collagen fibers. In addition to dermoscopy, multispectral imaging and high-frequency ultrasonography (HFUS) were utilized as a portable, non-invasive tools for the observation of the skin lesions in CTDs.
Results: NLO microscopy revealed mid-dermal calcium salts in pseudoxanthoma elasticum (PXE). Significantly higher number and shorter elastin fibers were found while collagen fibers showed shorter and thinner structure. In patients with Ehlers-Danlos syndrome loosely, disorganized collagen fibers were identified. Typical dermoscopic pattern of PXE appeared in higher contrast in multispectral images and significantly higher intensity were found between affected and healthy skin. On HFUS, our results showed oval hypoechogenic area in CTD patients with calcinosis cutis.
Conclusion: Novel imaging techniques may prove useful in the early diagnosis of CTDs. They may be also used for the objective follow up of the progression of CTDs, and the assessment of the efficacy of novel therapeutic approaches in the future.
Funding: NKFIH [FK_131916, 2019,M.M.]; ÚNKP-21-4-II-SE-10 (N.K.); EFOP-3.6.3-VEKOP-16-2017-00009 (K.F.)