Vol 97, No 6 (2021)

Inherited epidermolysis bullosa is a group of genetic skin disorders characterized by skin erosions, ulceration, skin and mucosal blistering requiring topical treatment. This review demonstrates major clinical manifestations of epidermolysis bullosa and its mechanisms of development. According to these features the main principles of topical treatment and drug therapy were developed, including physical protection from trauma, moisturizing, improvement of wound healing, prevention and management of infection, itch and pain management. Drug therapy is outlined with dosage forms, drug routes of administration, age restrictions indicated in the instruction for medical use for the medications that could be used in epidermolysis bullosa patients. The authors provide indications for clinical use of antiseptics, disinfectants, antibiotics, antimicrobial agents, emollient cream and drugs reducing itch and pain.

The notion of skin microbiome encompasses a heterogeneous group of microorganisms that belong to various taxonomic units, such as bacteria, archaea, viruses, and fungi. The impact of these microbial community constituents upon the epidermal barrier condition, and upon the immune system functioning, is being intensely scrutinized. There is a particular interest in studying the role that the microorganisms of genus Staphylococcus spp. play in the course of physiological and pathological processes occurring in the skin. This review examines in detail the interaction of the microorganisms of genus Staphylococcus spp. with the microbial community constituents, as well as with the skin immune system in normal condition and in the condition associated with inflammatory dermatoses. There are also the data given on S. aureus pathogenicity factors, the data on the impact of this microorganism upon the course of atopic dermatitis, and upon the course of psoriasis. The review examines the role that coagulase-negative staphylococci, S. epidermidis in particular, play in maintaining the microbiome homeostasis. The review as well examines the impact of the skin microbiome upon the development and activity of the skin immune system, and upon maintaining the integrity of the epidermal barrier.

The review is devoted to the appearance of resistance of a slowly developing disease — leprosy — to antimicrobial therapy (AMP), primarily recommended by the World Health Organization. The main danger of drug resistant leprosy is in the difficulty of identifying, since the causative agent of the disease is not cultivated on artificial media, and the methods for diagnosing drug resistance that are currently used take a long time. The drug resistance of the Mycobacterium leprae strain even to individual components of combination drug therapy result to the development of symptoms of the disease despite undergo anti-leprosy therapy, which in turn can cause the patient to become disabled. Currently, in the Russian Federation, there is no approved test for detecting Mycobacterium leprae DNA, and the determination of genetic determinants of resistance is carried out by sequencing genome regions determined by WHO recommendations: small gyrA, folP and rpoB genes loci. At the same time, modern studies in endemic regions reveal an increased level of Mycobacterium leprae strains resistant to individual components of combined drug therapy. The use of next generation sequencing (NGS) has made it possible to identify additional genetic determinants of leprosy resistance to the components of combination drug therapy. The current situation is relevant to antimicrobal drug resistance surveillance by using of quick identification systems for most frequent genetic resistance determinants of Mycobacterium leprae.

The literature search was carried out using keywords in the Scopus, PubMed and RSCI databases.

Background. There is a need to assess the diagnostic significance of the symptoms of nail damage, as well as available effective methods for diagnosing psoriatic arthritis in the early stages.

Aims. To study of the occurrence of symptoms of psoriatic onychodystrophy in patients with psoriatic arthritis and assessment of the diagnostic significance of the ultrasound method for diagnosing enthesitis as an early symptom of psoriatic arthritis among patients with onychodystrophy and without psoriatic arthritis in case history.

Methods. It was a prospective clinical study based on the Clinic of Skin and Venereal Diseases of the Military Medical Academy. We made the clinical assessment of symptoms of onychodystrophy among patients with and without psoriatic arthritis and the evaluation of the diagnostic significance of the ultrasound method for diagnosing psoriatic arthritis under the control of magnetic resonance imaging of the joints.

Results. 45 patients with psoriatic arthritis and 134 patients with psoriasis without arthritis were examined. Psoriatic onychodystrophy was significantly more common in the group of patients with psoriatic arthritis (p = 0.028). The most characteristic symptoms of onychodystrophy in patients with psoriatic arthritis are oil spots and symptom combinations: oil spots and subungual hyperkeratosis, onycholysis and subungual hyperkeratosis, pits and longitudinal ridges, oil spots and longitudinal ridges. The ultrasound method for the diagnosis of psoriatic arthritis has shown its high diagnostic significance (Se = 81.8%, Sp = 88.0%). The most characteristic symptoms of arthritis on ultrasound examination are hypoechoic structures of the Achilles tendons and the presence of fluid in the peri- and retro-Achilles bursa.

Conclusions. The symptom of oil spots and combinations of oil spots with subungual hyperkeratosis, onycholysis with subungual hyperkeratosis, pits with longitudinal ridges and oil spots with longitudinal ridges can be considered diagnostically significant in the examination of early psoriatic arthritis, and the ultrasound method is highly effective in diagnosing early manifestations of arthritis.

Pagetoid reticulosis (PR) is a rare type of mycosis fungoides. Clinical symptoms of PR can mimic other skin diseases of papulosquamous, neoplastic, and infectious origin that hampers PR diagnostics. The main histopathologic feature of PR is dense intraepidermal infiltration by medium to large-size lymphocytes through the epidermis leading to pagetoid plaque formation. There are three common immunophenotypes of PR: CD4-positive T-helper phenotype (CD3⁺, CD4⁺, CD8^–); T-cytotoxic/suppressor (CD3⁺, CD4^–, CD8⁺); and double negative phenotype (CD3⁺, CD4^–, CD8^–). The clinical case of PR with rare immunophenotype (CD2⁺, CD3⁺, CD8⁺ lymphoid infiltrate) is presented. The careful analysis of the symptoms, pathomorphological and immunohistochemical data is necessary for accurate PR diagnostics.

The development of both malignant neoplasms — mycosis fungoides and cutaneous malignant melanoma — is a rare condition; nevertheless, the literature describes both cases of the development of mycosis fungoides in patients with a primary diagnosis of melanoma and the detection of cutaneous malignant melanoma in patients with T-cell lymphomas of the skin. The question of the effect of previous therapy for mycosis fungoides on the risk of cutaneous malignant melanoma remains controversial. Currently, the world community is considering a possible pathogenetic relationship between these two oncopathologies. The clinical observations of the development of cutaneous malignant melanoma in 2 patients with mycosis fungoides presented in the article emphasize the importance of a thorough clinical and dermatoscopic examination of all pigmented formations for the timely detection of malignant melanocytic neoplasms, the presence of which radically affects the further choice of treatment tactics for patients.