Limited scleroderma: sonographic pattern in the stage of erythema/edema

Objective. To describe the sonopattern of limited scleroderma (LS) in the early stages after the onset of erythema.Materials and methods. The work describes a clinical case of limited plaque scleroderma. The sonographic examination was carried out on an ultrasound scanner using a transducer with operating frequencies of 10–16–18 MHz. Material sampling for the histologic examination of the skin was performed from the area with the most pronounced inflammatory changes under sonographic control.Results. It has been found that increased echogenicity of the dermis, “blurring” of the dermis/hypodermis boundary, increased echogenicity and the “stalactite-like” pattern of subcutaneous fat occur in the frst week of the disease; normalization or a signifcant improvement of the sonopattern is noted by the end of the second week or by the beginning of the third week after the onset of erythema.Conclusion. There is a certain parallelism between the histologic and sonographic patterns, which makes it possible to adequately assess both the activity and the stage of the LS process.

Lichen sclerosus as a variant of limited scleroderma with damage to the anogenital region

Lichen sclerosus is one of the clinical variants of limited scleroderma, a feature of which is the defeat of the anogenital zone. The issues of the etiology and pathogenesis of lichen sclerosus remain not fully understood, despite numerous studies in which the polygenic nature of inheritance and multifactoriality in the implementation of dermatosis are noted. With the progression of the scleroatrophic process and the absence of timely pathogenetic therapy, there is a high probability of the development of comorbid pathology with a whole range of symptoms, such as urinary (dysuria, recurrent urinary tract infections - cystitis, urethritis, cystourethritis), gastrointestinal (pain during defecation, chronic constipation), vaginal severe dryness and increased sensitivity of the vaginal mucosa, burning, itching). Treatment of patients with lichen sclerosus with lesions of the anogenital zone causes great difficulties. In cases of isolated localization of scleroatrophic lesions of anogenital localization, difficulties may arise in the differential diagnosis with diseases of similar localization, such as vitiligo, atrophic lichen planus, basal cell carcinoma, contact dermatitis, nonspecific balanoposthitis, Keir's erythroplasia. Treatment of limited scleroderma, the clinical variant of which is lichen sclerosus, should be multi-course and complex, with the obligatory use of penicillin antibiotics, hyaluronidase-based drugs, drugs that improve microcirculation, vitamins, immunoregulators and physiotherapeutic methods. Thus, early diagnosis and timely pathogenetic therapy contribute to the prevention of the risks of developing genitourinary syndrome, progression of tissue sclerosis, and psychoemotional disorders. This article presents a clinical case of diagnosis and treatment of a patient with lichen sclerosus.

Evaluation of the optic nerve head vessel density in patients with limited scleroderma

Objectives: To investigate the optical coherence tomography angiography (OCTA) parameters of the optic nerve head and peripapillary retina and to assess macular and peripapillary retinal nerve fiber layer (RNFL) thickness by using spectral-domain optical coherence tomography (SD-OCT) in patients with limited scleroderma and to compare these results with those of healthy control subjects. Materials and Methods: 42 patients with a confirmed diagnosis of limited scleroderma and 32 age- and sex-matched healthy control subjects were included in the study. OCTA was performed for the radial peripapillary capillary plexus (RPCP) whole image, inside disc, and peripapillary vessel densities in all participants with XR Avanti AngioVue OCTA (Optovue, Fremont, California, USA). OCT images were obtained with Spectralis OCT with eye-tracking dual-beam technology (Heidelberg Engineering GmbH, Heidelberg, Germany), and peripapillary RNFL thickness was evaluated with circle program. The data from the right eyes of all participants were used for statistical analysis. Results: No significant difference was found between the radial RPCP whole image, inside disc, and peripapillary vessel density values or the RNFL parameters of the scleroderma patients when compared with the controls ( p > 0.05 for all). Conclusion: Decreased peripapillary vessel density on OCTA, which can be an early sign of glaucoma, could not be observed in scleroderma patients in this study. However, further long-term studies are still needed to identify glaucoma tendency in patients with scleroderma before clinically detectable glaucoma.

Gender, clinical and topographic features of the localized scleroderma lesions in anogenital area

Background: Currently, there is an increase in the number of skin lesions of anogenital localization, which is a silent epidemic, both due to the steady increase in the incidence of this pathology, and the interdisciplinary aspect of this problem. Materials and methods: In the article, the authors first analyzed and presented the data of clinical and morphological analysis of 104 patients with various clinical variants of limited scleroderma, on the basis of which the main phenotypic and gender-specific clinical and topographical features of anogenital zone lesions in this group of patients are presented. Results: Scleroatrophic lichen is one of the clinical variants of limited scleroderma, which is characterized by damage to the mucous membranes of the external genitals in both women and men. Lesions of such localization are late and often mistakenly diagnosed by specialists of related disciplines (obstetricians, gynecologists, urologists, family doctors, allergists, cosmetologists), which leads to high risks of developing genitourenal syndrome. Conclusions: The development of scleroatrophic lesions in the anogenital zone is accompanied by pronounced clinical symptoms, including: itching, pain of varying intensity, dysuria, dyspareunia and significant sexual dysfunction.

LICHEN SCLEROSUS OF THE VULVA IN PATIENTS WITH LIMITED SCLERODERMA

Modern literature information on the etiopathogenetic prevalence of lichen sclerosus has been presented by the authors; clinical manifestations and classification of the disease have been outlined; diagnosis and treatment issues have been discussed in the article. 3 clinical cases of genital lesions in the form of lichen sclerosus in patients with limited scleroderma have been described. In our opinion, lichen sclerosus is one of the forms of limited scleroderma. Purpose. To investigate the conditions for the diagnosis and treatment of genital lesions in the form of lichen sclerosus in patients with limited scleroderma. Methods. The first line of therapy for lichen sclerosus is the use of super-powerful topical glucocorticoids. Clobetasol propionate 0.05% is to be used for 3 months (1 month daily, then every other day for a month, then 2 times a week for a month). After 3 months of treatment, if a positive effect is declared, maintenance therapy is recommended once a week. Topical calcineurin inhibitors are recommended as a second-line therapy. Effective and safe excipients are emollients that moisturize the skin and mucous membranes, prevent transdermal fluid loss, and reduce subclinical inflammation. The possibility of using triamcinolone (1-2 mg) onto pathologically altered tissue in women with lichen sclerosus where there are hyperkeratotic sites resistant to GCS can be considered after exclusion of intraepithelial neoplasia or malignant transformation. The effectiveness of physiotherapeutic methods, such as low-intensity helium-neon laser radiation in the red or infrared range, treatment with sunlight and radiation generated by medical equipment, photodynamic therapy has been described. Results. One of the important measures is the elimination of irritating factors, as well as careful care of the vulva and the treatment of secondary infection. It consists in adherence to a diet (exclusion of spicy, salty, sweet foods, caffeine-containing products, alcohol) and intimate hygiene rules (limitation / exclusion of soap-containing products, deodorants, synthetic underwear, pads, tampons). The underwear should not be very tight. In addition, patients should exclude mechanical irritation of the affected area, including shaving. With extreme manifestation of itching, it is recommended to prescribe desensitizing therapy and sedatives. Conclusions. Clinical cases of practical interest in connection with the clinical manifestations of limited scleroderma on the skin and genitals have been considered. Frequent lesions of the genital organs in the form of a typical clinic of lichen sclerosus in women suffering from limited scleroderma, the similarity of their clinic, histological picture and pathogenesis have been noted. All this gives a reason to consider lichen sclerosus as a manifestation of limited scleroderma.

Clinical and X-ray diagnostic criteria for maxillofacial damage in children with juvenile limited scleroderma

The objective of our study was to improve the diagnosis of maxillofacial lesions in children with juvenile scleroderma. We performed a dental examination of 41 children from 4 to 17 years old with juvenile scleroderma. Based on the clinical X-ray examination we identified the main diagnostic signs of the maxillofacial damage in children with juvenile scleroderma, including partial hemiatrophy, plaque or linear facial lesions, reduced salivation, atrophic glossitis, plaque spots of mucous tongue atrophy, ischemia or shortening of the sublingual bridle, local recession of the gums of the lower jaw, dystopia and tooth supraposition, disocclusion, delay teething, spontaneous resorption of the permanent teeth roots, one-sided delay in the development of jaw bones. Using this complex of symptoms a dentist at the first visit can pre-diagnose scleroderma, which is especially important for the selection of adequate methods of treatment and prevention.