Lupus pernio (LP) is a chronic non-life threatining type of cutaneous sarcoidosis that can be related to chronic fibrotic sarcoidosis, hyperglobulinemia and hypercalcemia. The aim of this case report is to evaluate the clinical and demographic features of cutaneous sarcoidosis mainly presenting with a rare manifestation of LP. In this paper we report a case of systemic sarcoidosis presenting with LP and a review of the available literature. LP is a rare presentation with infiltrated erythematoviolaceous plaques affecting the nose. We address the main management approach, and possible association with an underlying systemic sarcoidosis. LP is a rare but chronic manifestation of systemic sarcoidosis that needs to be treated in order to prevent cosmetic defects and psychological effects. It is important to recognize such a condition early in order to avoid a delay in treatment and worsening of the condition, both physically and psychologically. Further research regarding the diagnostic approach and management is required to understand this condition thoroughly.
PurposeThe aim of this study was to evaluate the progression of a case of a patient with epidermolysis bullosa (EB) since early age who survived to adulthood, presenting with recurrent skin blisters and disfiguring scars and disabling musculoskeletal deformities.BackgroundEB is a rare group of inherited diseases that affect the skin fragility causing it to blister in response to even minor trauma. Established novel treatments are limited in the literature due to its rarity, and more research is needed to set a global management approach. Clinical manifestations range widely from localized to generalized blistering.MethodsA rare case of EB surviving to adulthood despite the complications, which has been evaluated, treated during a relapse, and followed up.ConclusionThe described case is of considerable clinical interest due to its rarity and severity. Optimal management requires a multidisciplinary approach and revolves around the protection of the skin against slightest injury, use of careful wound care dressings, aggressive nutritional support, and early medical or surgical interventions if needed to manage any complications. Prognosis varies considerably depending on each case.
Successful reconstruction of the aortic valve requires the preliminary creation of the anatomical design of its image, which includes a set of parameters of the valves of a particular valve. Errors in operation at the stage of design of valve components are fraught with subsequent dysfunction. In the present, the optimal sizes of the aortic root and the valves of the aortic valve were evaluated and the presence and values of correlations between them were determined. Statistically significant anatomical and functional correlations of morphometric characteristics of aortic root structures were revealed to achieve the optimal level of their correspondence in autologous grafts during surgical correction of the aortic valve. The main morphometric parameters of the aortic root were analyzed on the example of 54 samples obtained post mortem from patients whose death occurred from causes not associated with heart disease. The cases with aortic valve disease were excluded from the study. The following parameters were evaluated: circumference of the ventriculo-arterial junction; circumference of the sinuses of Valsalva; circumference of the sinutubular junction. Separately, for each of the three cusps of the native aortic valve, were evaluated: the length of the free edge of the cusp (L1); the length of the junction of the leaflet and the fibrous ring of the aortic valve (L2); leaflet height (A); intercommissural distance (IC). A search was made for the correlation between the size of the valves and the parameters of the circumference of the aortic root at the level of the ventriculo-arterial junction, of the sinuses of Valsalva and of sinutubular junction. A significant presence of a correlations was found between all parameters of the aortic valve cusps and the length of the aortic circumference at the level of the ventriculo-arterial junction, of the sinuses of Valsalva and sinutubular junction (r>0.6 for IC, L1, L2 and r>0.5 for A at p<0,01). The data obtained by correlation analysis make it possible to standardize the design of the autograft of the aortic valve, thereby ensuring the best coaptation of the valves and, as a result, the function of the aortic valve.
The results of a study of the clinical effectiveness of the correction of involutional changes in the skin by the method of photodynamic therapy (PDT) with the use of a photosensitizer gel (PS), the active substance of which is the trismeglumine salt of chlorin e6, are presented. The data of fluorescence spectroscopy for monitoring the level of PS fluorescence in order to determine the optimal time of its exposure are demonstrated. The study of the optimal exposure time involved 80 patients with different skin phototypes. The study of the effectiveness of PDT involved 42 patients aged 44–68 years with signs of chrono- and photoaging. The PDT procedure was performed by irradiating the entire surface treated with the PS with a power density of 100 mW/cm2, a light dose of 120–140 J/cm2, a laser radiation wavelength of 660 nm, and a light beam area of 400–800 cm2. It was found that exposure to PS for 10–20 min gives the highest fluorescence and does not depend on the skin phototype. The clinical effect of PDT was achieved in 85.7% of patients; there were no negative subjective sensations. Moisture metrics increased on the skin of the face by 53%, reaching the control values in young healthy volunteers, on the skin of the hands - by 64%. Elastometry indicators on the skin of the face and hands increased by 19% and 16%, respectively. Thus, the PDT procedure with PS based on chlorin e6 is an effective method for correcting involutional changes in the skin, leads to a pronounced clinical effect, improves the parameters of skin moisture measurement and elastometry, and passes without undesirable local reactions. Optical coherence tomography showed an increase in collagen ordering.
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