Vol 21, No 3 (2024)

BACKGROUND: Serum tryptase levels are used as a diagnostic marker in mastocytosis and are considered an indicator of clonal mast cell load.

AIM: To analyze tryptase levels in children with different clinical types of cutaneous mastocytosis.

MATERIALS AND METHODS: A single-center prospective study was conducted for the period 2022–2024. The results of examination of 202 children aged from 6 months to 17 years with a diagnosis of cutaneous mastocytosis, who were undergoing outpatient treatment at the Moscow scientific and practical Center of dermatovenereology and cosmetology, were analyzed. Serum tryptase levels were determined by enzyme immunoassay.

RESULTS: The median basal tryptase level for the entire group (n=202) was 5.09 μg/L (range 1.3–57.0 μg/L). Serum tryptase values exceeding 11.0 μg/L were detected in 17.3% of patients, and 20.0 μg/L in 7.4%. The mean enzyme level in the control group was 2.55 μg/L. There were significant differences in tryptase concentrations between the maculopapular cutaneous mastocytosis and control groups (p=0.05). The median tryptase level in children with maculopapular cutaneous mastocytosis was 8.7 μg/L. The most pronounced differences were observed between the enzyme levels in children with the monomorphic type of maculopapular cutaneous mastocytosis and in the control group (p=0.01). Analysis of tryptase levels depending on gender and clinical type of maculopapular cutaneous mastocytosis showed that children and adolescents with the monomorphic type of maculopapular cutaneous mastocytosis had higher enzyme levels.

CONCLUSION: Further studies are needed to determine which tryptase levels in children are indicative of mast cell activation and primary mast cell diseases. A detailed understanding of tryptase levels in children may facilitate the development of new specific therapeutic approaches to the management of various clinical forms of cutaneous mastocytosis.

Considering the rapid, often unpredictable and unfavorable course of anaphylaxis, early recognition of the signs of this emergency and immediate provision of first aid by the victims themselves or by the witnesses before the arrival of emergency medical services are of exceptional and sometimes of vital importance. Trends towards an increase in the incidence of anaphylaxis and the actual increase in the number of hospitalizations due to complications of severe allergic reactions indicate the high relevance of the problem and the need to establish a reliable organizational basis to ensure the widespread practice of providing first aid for anaphylaxis in the Russian Federation.

This research aimed to review current international and national guidelines on the provision of first aid for anaphylaxis and to identify possible areas for improving the organization of first aid for anaphylaxis in Russia. According to the authors’ opinion, the application of evidence-based provisions presented in the current first aid guidelines to real-life first aid practice can significantly improve outcomes in anaphylaxis, but at the same time requires some basic organizational changes. The priority directions in organizing the provision of first aid for anaphylaxis in the Russian Federation are the development and approval by the Ministry of Health of Russia of a uniform procedure for the provision of first aid for anaphylaxis, including a list of measures for the provision of first aid and the sequence of their application; creating conditions for standardized training of the population in the principles and methods of providing first aid; and ensuring the general availability of epinephrine in the form of autoinjectors as a first-line treatment to prevent life-threatening complications of anaphylaxis.

Glucocorticoids are the most commonly used anti-inflammatory drugs for the treatment of inflammatory diseases, despite the fact that the resistance to them is increasing due to various circumstances. glucocorticoids insensitivity is an important clinical issue that is associated with life-threatening disease progression. Currently, there is no reliable biomarker that can be used to define steroid resistance. According to several studies, increased expression of the glucocorticoid receptor beta (GR-β) isoform is associated with steroid resistance.

In this review authors analyse the correlation between GR-β expression and the occurrence of steroid therapy-resistant diseases and to determine its potential as a biomarker. This review summarizes studies that point out increased GR-β expression in steroid-resistant groups of patients compared to steroid-sensitive groups. Articles included in the review focus on asthma, nasal polyposis, ulcerative colitis, and other chronic inflammatory diseases in which resistance to steroid therapy is observed. GR-β levels have also been shown to be elevated in patients with allergic rhinitis, Crohn’s disease and rheumatoid arthritis. In most studies, higher levels of GR-β were detected in peripheral blood mononuclear cells by reverse transcription polymerase chain reaction. Increased expression of GR-β is mostly associated with steroid resistance.

Thus, it can potentially be used as a biomarker of steroid resistance-related disorders.

Currently, the issue of COVID-19 infection remains. The potential consequences of acute infection are not yet fully understood. Dermatological manifestations of COVID-19, including urticarial vasculitis, which is poorly responsive to standard therapy, are increasingly reported.

Urticarial vasculitis is a rare dermatological manifestation of COVID-19, characterized by long-lasting welts with angioedema or without it, and histopathological features of leukocytoclastic vasculitis. Leukocytoclastic vasculitis, in turn, is a common form of small vessel vasculitis, with an inflammatory infiltrate consisting of neutrophils, fibrinoid necrosis, and nuclear debris fragmentation (leukocytoclasis). Proper nosology and classification of urticarial vasculitis based on clinical presentation and histological data are crucial.

We described a clinical case of a 54-year-old woman with urticarial vasculitis, who had no systemic diseases. The lack of response to treatment with antihistamines in combination with corticosteroids led to the initiation of colchicine as an alternative therapy. Colchicine suppresses granulocyte mitotic activity, inhibits the formation of leukotriene B4, and reduces leukocyte migration to the site of inflammation, preventing neutrophil degranulation. After treatment with colchicine at a dose of 0.5 mg twice a day for 2 months, remission and improvement of clinical symptoms were achieved.

Post-COVID syndrome with dermatological manifestations is a common issue today. Skin biopsy serves as an important tool in the differential diagnosis of chronic urticaria and urticarial vasculitis after a COVID-19 infection. This highlights the importance of studying dermatological manifestations of COVID-19 for more effective diagnosis and treatment of this disease.

Smoking electronic cigarettes, or vaping, is considered by many to be a safe alternative to smoking tobacco. It was only relatively recently that reports began to appear about the development of lung damage caused by electronic cigarettes ― EVALI syndrome. Currently, the morphological manifestations of this syndrome have not been studied sufficiently due to the small number of cases described in the literature. These facts, as well as the fact that the changes that occur are not specific, and may vary in different patients, make it difficult to diagnose the disease in a timely manner.

We have described a clinical case of death in a patient who has repeatedly received inpatient treatment for interstitial lung disease, who was admitted to the pulmonology department with signs of decompensated respiratory failure after prolonged and intensive use of electronic cigarettes. The autopsy revealed morphological signs and justified the clinical symptoms of chronic hypersensitive pneumonitis in combination with EVALI syndrome.

The article also provides a brief review of the literature on EVALI syndrome and hypersensitive pneumonitis with their diagnostic criteria. Like EVALI, hypersensitive pneumonitis is difficult to recognize and diagnose by doctors, which is often associated with low awareness, as well as the absence of pathognomonic symptoms.