Vol 26, No 5 (2023)

Epidermal growth factor receptor (EGFR) inhibitors are widely used to treat various types of cancer, such as non-small cell lung cancer, head and neck cancer, breast cancer, pancreatic cancer, colorectal cancer. During treatment, class-mediated effects such as skin toxicity, interstitial lung disease, hepatotoxicity, ocular toxicity, hypomagnesemia, stomatitis, and diarrhea may occur. The role of epidermal growth factor and its receptors in the vital activity of keratinocytes is key. Dermatological undesirable effects occur in 60–90% of patients, and often persist throughout the entire period of EGFR inhibitor therapy.

Currently, studies of biomarkers predicting the severity of skin complications of EGFR inhibitor therapy are extremely limited. Thus, it is relevant to study genetic markers reliably associated with the development of severe skin toxicity against the background of EGFR inhibitor therapy, because the EGFR signaling pathway is important for maintaining normal physiological function of the skin. The development of severe skin reactions leads to a reduction in the dose or discontinuation of EGFR inhibitors in the treatment of cancer. Recently, progress has been made in studies of the cutaneous toxicity of EGFR inhibitors.

Here we briefly describe the mechanism of skin toxicity caused by EGFR inhibitors, current data on the relationship of gene polymorphism and severity of manifestations of undesirable dermatological phenomena.

Bloch–Sulzberger syndrome or Incontinentia pigmenti is a genetic disease that causes skin pathology in the first days of a child's life and is suspected of identifying the disease.

The article presents the characteristic signs of this syndrome and the pronounced manifestations of the disease. A presumptive case of a girl born in a family without a burdened anamnesis: the first manifestations of the disease appeared on the fifth day of life, were represented by linearly arranged vesicular-bullous rashes on an erythematous background and were regarded as a skin infection. Due to the ineffectiveness of the therapy, the patient was consulted by a dermatovenerologist, geneticist, neurologist. The child was consulted again at 5.5 years old: the article presents the data of a medical and genetic study and distant clinical signs of this syndrome in the form of hyperpigmented spots in combination with foci of atrophy, alopecia and dental anomalies in the sixth year of life.

Thus, the clinical signs of Bloch–Sulzberger syndrome made it possible to diagnose the disease at the newborn stage, which, in turn, helped to avoid unjustified prescribing of various systemic medications, to develop an individual treatment plan for the child and, as a result, contributed to the prevention of the development of complications of the disease and to improve the patient's quality of life.

BACKGROUND: According to modern data, psoriasis is an immune-mediated disease of a multifactorial nature, affecting not only the skin, but also associated with the development and worsening of the course of diseases of the cardiovascular system. Given the systemic nature of psoriasis, a targeted study of this problem is necessary.

AIM: The aim of this study is to establish the relationship between psoriasis and cardiovascular disease.

MATERIALS AND METHODS: The main group consisted of 160 patients diagnosed with psoriasis, 40 patients were included in the comparison group. Inclusion criteria for the study: age over 18 years, signed informed consent to participate. The exclusion criteria were pregnancy and the patient's refusal to participate further. An open prospective observational clinical study was conducted. The total duration of the study and follow-up was 1 year. All patients had total cholesterol, glucose, creatinine, C-reactive protein measured and calculation of glomerular filtration rate done performed. Body mass index, blood pressure according to Korotkov method, severity of psoriasis (PASI), and dermatological quality of life index (DLQI) were also assessed (by HADS, Hamilton, Zang scales). The psycho-emotional state was assessed, the smoking and the presence of diabetes mellitus were taken into account. Cardiovascular risk was calculated using the SCORE2/2OP scale. In the case of a high cardiovascular risk and/or detection of previously undiagnosed cardiovascular disease, patients were admitted to hospital. All patients received optimal medical and topical therapy.

Patients were followed up during hospitalization and/or telephone calls one year after enrollment in the study.

RESULTS: Among the patients of the main group, from 19 to 85 years old, of which 61% were men and 39% were women, mild psoriasis was diagnosed in 6.2%, moderate in 40%, and severe in 53.8%. Low cardiovascular risk was determined in 39%, moderate in 14%, high in 47%. Among patients in the control group, from 24 to 81, of which 30% are men and 70% are women. Of these, 65% had a low cardiovascular risk, moderate in 5%, and a high in 30%. According to the results of the correlation analysis, there were no statistically significant results on the relationship between psoriasis and cardiovascular disease, however, a multivariate ROC analysis revealed a combined effect of gender, height, weight, body mass index, age, cholesterol level, PASI and DLQI index on the likelihood of development and/or worsening the course cardiovascular disease (AUC 0.9258).

CONCLUSION: Our study provides evidence for a potential causal relationship between psoriasis and cardiovascular disease. Thus, early assessment of CV risk and prevention of the development and worsening of cardiovascular disease requires careful attention from healthcare professionals within multidisciplinary teams.

Kaposi's sarcoma is a multifocal malignant disease of vascular origin with a primary lesion of the skin, as well as internal organs and lymph nodes. According to various authors, it is believed that Kaposi's sarcoma is associated with herpes simplex virus type 8 (HHV-8).

According to the literature, the following types of Kaposi's sarcoma are distinguished: classical (European), immunosuppressive, endemic (African) and epidemic (this form of sarcoma is associated with the state of acquired immunodeficiency). Clinically, with Kaposi's sarcoma, rashes can vary depending on the form of the disease, among which there are spotty, papular and tumor forms. Also in the literature, some authors distinguish the 4th form of Kaposi's sarcoma ― bullous.

Diagnostic search for Kaposi's sarcoma is a difficult problem due to the large number of diseases of vascular origin, as well as their clinical similarity. The diagnostic methods described in the literature include many studies, including a blood test for HHV-8, as well as routine histological examination and immunohistochemistry.

The article presents a rare clinical case of Kaposi's sarcoma in a young girl with a long history of the disease.

Polymorphic photodermatosis is the most common, acquired skin disease characterized by an abnormal, recurrent, and delayed response to sunlight.

In this article, modern views on the etiology of pathogenesis are described clinical picture, differential diagnosis, principles of treatment and prevention of polymorphic photodermatosis. The pathogenesis of this disease is a form of delayed-type hypersensitivity reaction to photoantigens. The abnormal immune response has been attributed to the immunosuppressive effects of sunlight due to a breakdown in allergen tolerance and a disruption in the mechanisms of apoptosis.

The main symptoms of the disease are erythema and highly itchy elements of skin lesions, which are very diverse, most often represented by macules, papules and vesicles, or vesiculo-bullous, urticarial, hemorrhagic rashes, hence the name of the disease polymorphic photodermatosis. Usually one morphology prevails in one patient. The article presents a clinical case and the results of their own observations.

Studies of the pathogenesis of polymorphic photodermatosis have confirmed the presence of an abnormal immune response. The exact mechanism of immunosuppression caused by ultraviolet radiation and the relative contribution of UV-B and UV-A in healthy people are still unclear, but the expression of TNF-alpha, IL-4 and IL-10 and the depletion of Langerhans cells appear to be key phenomena.

Diagnosis of the disease based on the clinical picture. The need for differential diagnosis of various forms of polymorphic photodermatosis is due to their clinical and sometimes histological similarity with other skin formations. For more accurate verification of the diagnosis, a biopsy of the examined skin area is possible, followed by histology. The histology of polymorphic photodermatosis is nonspecific, in accordance with the polymorphic clinical picture, as well as depending on the timing of the biopsy.

Further studies of the pathological mechanisms underlying polymorphic photodermatosis may allow us to develop a more targeted approach to conservative treatment and prevention.

On June 20, 2023, the 1153rd meeting of the Moscow Society of Dermatovenerologists and Cosmetologists named after A.I. Pospelov took place.

The meeting was held in a "face-to-face" format. There were 86 participants in total. Applications for membership in the Moscow Society of Dermatovenerologists and Cosmetologists named after A.I. Pospelov were not submitted. Elections of delegates to the XXIII Congress of Dermatovenerologists and Cosmetologists of Russia (Corresponding member of the Russian Academy of Sciences, Professor O.Y. Olisova, Professor E.S. Snarskaya, Professor O.B. Tamrazova, Associate Professor A.B. Yakovlev, assistant I.S. Maksimov, doctor V.V. Savenkov) and nomination of candidates for the position of the President of the All-Russian Public Organisation "Russian Society of Dermatovenerologists and Cosmetologists" were held (Academician of the Russian Academy of Sciences A.A. Kubanov).

Two reports are presented in the clinical part of the meeting: (1) paraneoplastic bullous dermatoses (Sechenov University); (2) Otto–Moncorps keratosis (Sechenov University).

Reports were made in the scientific part of the meeting: (1) mycobacterial infection combined with skin mycoses in patients with HIV infection: diagnostic issues (Sechenov University, Pirogov Russian National Research Medical University); (2) under the "mask" of a scar (Sechenov University).

Genodermatoses are skin diseases caused by the inheritance of a pathological gene responsible for the formation of a particular skin structure.

Hereditary diseases can be monogenic, polygenic and chromosomal. Genodermatoses follow Mendelian inheritance (autosomal dominant, autosomal recessive or sex-linked). Some genodermatoses appear as a result of genetic mosaicism (parts of two different chromosomes exchange their fragments).

Autosomal dominant disorder manifest in every generation in women and men with the same frequency. Recessive genodermatoses are usually more severe and rarer. In this case, both parents are carriers of the pathological gene, but are clinically (phenotypically) healthy. The risk of developing of autosomal recessive pathology increases highly in closely related marriages.

We present a gallery of the hereditary dermatoses.

Neurofibromatosis is a neurocutaneous syndrome characterized by the formation of benign tumors in the skin, soft tissues, nervous system and internal organs.

Congenital epidermolysis bullosa is a group of genodermatoses with the development of intraepidermal blisters that arise spontaneously or as a result of minor trauma.

Tuberous sclerosis (Bourneville–Pringle disease) is a hereditary disease characterized by hyperplasia of ectodermal and mesodermal derivatives, leading to disruption of embryonic differentiation. It damages the skin, central nervous system and other organs.

Pseudoxanthoma elasticum is characterized by calcification of elastic fibers of the skin with damage to the vision organ, cardiovascular system and reproductive function.