A Review of Primary Immunodeficiency Diseases with Skin Manifestations

Introduction: Primary immunodeficiencies (PID) are rare heterogeneous disorders with defects in which one or more components of the immune system are malfunctioning. Clinical presentations of the patients according to type of immunodeficiency are variable. The majority of these patients are susceptible to infections depending on the type of disorder. In these patients, one of the most important and common symptoms is a skin manifestation that in many cases helps to diagnose the disease. Skin symptoms can include infectious-inflammatory-autoimmune-allergic manifestations and malignancies. In some cases, skin involvement can be the initial manifestation of immunodeficiency diseases, so understanding the relationship between the type of primary immunodeficiency and the type of skin involvement is very important in diagnosing the disease. The majorities of skin diseases are not pathogenomonic in primary immunodeficiencies and may be seen in other diseases with normal levels of immunity. However, there are numerous skin findings that are so characteristic of immunodeficiency diseases that it is necessary to evaluate the immune system. Conclusion: Skin is an organ that may be involved in many diseases, including primary immunodeficiency. Sometimes skin is the first organ involved in immune deficiencies. Therefore, recognizing skin manifestations in these patients is one of the most important factors in early diagnosis of these people.

Approaches to treatment of recurrent eczema in practice of general practitioner (clinical example)

The article presents a clinical case of recurrent eczema. Despite treatment lasting more than 5 years, the patient observed only a short-term effect of the therapy. The cause of the continuous course of severe eczema was malnutrition and hypovitaminosis of vitamins A and E, due to a lack of fish and seafood in the diet. Despite the relatively rare occurrence of eczema in the outpatient practice of a general practitioner, on average from 2 to 5 % of the adult population in the world suffers from this disease. Among the main methods of treating eczema is long – term antihistamine and anti-inflammatory therapy. However, these methods of treatment are not always enough. The reason for the continuous course of eczema was irrational nutrition with a lack of fat-soluble vitamins A and E. Correction of the diet using a sorbent based on the herb Zostera marina allowed to achieve significant improvement and remission of the disease.Conclusions. 1) A thorough analysis of anamnestic data is required to determine the treatment regimen in cases of prolonged persistence of eczema symptoms, with resistance to standard therapy algorithms. 2) Patient’s refusal of certain types of food can lead to hypovitaminosis, which is accompanied by skin symptoms. 3) The appointment of retinol, tocopherol acetate and vitamin D 1 will contribute to a rapid and effective improvement of the condition of the skin in cases with insufficient consumption of products containing fat-soluble vitamins. Preparations made from the sea grass Zostera marina help to improve digestion, sorb and remove unwanted biologically active substances, reducing the severity and relieving skin manifestations.

Graves’ Disease

Graves’ disease (GD) is an autoimmune disorder characterized by presence of TSH receptor autoantibody. It is most common cause of hyperthyroidism worldwide. Though GD can occur any age but peak incidence is seen during adulthood in between 20 to 50 years of age. GD is more commonly seen in female. GD is primarily disease of thyroid gland but affects multi organ system i.e. heart, liver, muscle, eye and skin. Symptoms and signs are result from hyperthyroidism or a consequence of underlying autoimmunity. Weight loss, fatigue, heat intolerance, tremor, and palpitations are the most common symptoms. Diffuse goiter presents in most of younger patients with thyrotoxicosis but less common in older patients. Graves’ ophthalmopathy and pretibial myxedema are extrathyroidal manifestations of GD which results from action of TSHR autoantibodies on TSHR present onfibroblast, adipocyte and T cells in extrathyroidal tissue. Treatment of GD remains in between antithyroid drugs, radioiodine or surgery. In this review we discuss the diagnosis and management of GD.

Dermatomyositis Identified During Palliative Care Management for Chemotherapy-Associated Pain: A Case Report

Introduction: Skin disorders and neuropathy often occur as side effects of chemotherapy. We encountered a patient who was treated for drug-induced skin symptoms, but the symptoms did not improve, and he was eventually diagnosed as having dermatomyositis. Case presentation: A 71-year-old man underwent chemotherapy with regorafenib in February 2020 for the postoperative recurrence of sigmoid colon cancer, but treatment was discontinued after about 2 months owing to the appearance of skin symptoms, which were thought to be side effects of regorafenib. Subsequently, his symptoms further worsened, and he was hospitalized 3 weeks after the appearance of the initial skin symptoms, and a palliative care team was asked to relieve his back pain caused by the drug-induced skin symptoms. Erythema was widely observed on the lower back and limbs, and he experienced needle stick-like pain. Furthermore, the patient demonstrated difficulty in lifting both upper limbs. As acetaminophen was effective for his pain, the dose was slowly increased with careful observation. The cause of the patient’s muscle weakness was unclear, and after careful discussion of the possible causes among specialists in dermatology, neurology, and rheumatoid arthritis, a diagnosis of dermatomyositis associated with the malignant tumor was made about 10 days after his admission. The patient’s symptoms gradually improved with steroid pulse treatment (methylprednisolone 1 g/day for 3 days) followed by high-dose gamma globulin treatment (2.5 g/day for 5 days), and the patient was discharged 48 days after admission. Discussion: Because this patient was referred to a palliative care team for the purpose of relieving pain caused by skin symptoms associated with chemotherapy, a crucial point is the symptoms were treated as side effects of the chemotherapy from the beginning. As neuropathy can occur as a result of chemotherapy, the pain and muscle weakness could be explained at the time; however, the symptoms continued to worsen even after the chemotherapy was stopped. Because the symptoms were not typical of polymyositis/dermatomyositis, diagnosis of the patient was delayed, even though he was treated in each specialized department. Our present case indicates that paraneoplastic syndrome should always be kept in mind when treating cancer patients.

Relaxing Effect of Novel Cosmetic Ingredient using Lactobacillus gasseri HDB1102 on Skin Problems Caused by Particulate Matter

In this study, we developed Dermabiotics HDB1102 using Lactobacillus gasseri HDB1102 to relieve skin irritation caused by particulate matter (PM). L. gasseri HDB1102 was provided from cell bank and identified by 16S ribosomal RNA gene sequencing. Dermabiotics HDB1102 was manufactured by heating, centrifuging, and filtering culture medium of L. gasseri HDB1102. When 0-2.5%(v/v) Dermabiotics HDB1102 was treated, cytotoxicity on normal human epidermal keratinocytes (NHEKs) and human fibroblast was not observed by using MTT assay. The mRNA expression levels of cytochrome P450 1A1 (CYP1A1), interleukin (IL)-1β, and IL-8 on Dermabiotics HDB1102 treated cells decreased compared to PM-treated cells. Conversely, the mRNA expressions of aquaporin-3 (AQP-3), CD-44, and collagen type 1 (COL-1) on Dermabiotics HDB1102 treated cells were dose-dependent higher than those of non-treated cells. These results indicated that Dermabiotics HDB1102 have anti-inflammatory, moisturizing, and anti-wrinkle effects and could be used as a potential cosmetic ingredient to alleviate skin symptoms caused by PM.

Management and outcome of suspected and confirmed COVID-19 (SARS-CoV-2) vaccine hypersensitivity.

Systemic allergic reactions to vaccines are very rare. In this study we assessed the management and outcome suspected SARS-CoV-2 vaccine hypersensitivity. We present data of 219 individuals, who experienced symptoms suspicious for an allergic reaction after the first (n=214) or the second vaccination (n=5). 195 reactions occurred after the first application of mRNA-based vaccines (157 Comirnaty®, and 38 Spikevax®) and eighteen reactions were reported after first application of a vector vaccine (Vaxzevria®). Of these 162 experienced immediate symptoms. Skin symptoms occurred in 91 cases. The most frequent cutaneous symptom was angioedema (n=45), followed by generalized urticaria (n=36) and generalized erythema/flush (n=20). 70 patients had cardiovascular symptoms, 45 showed respiratory symptoms and gastrointestinal symptoms were recorded in 14 patients. The allergological assessment of 334 individuals (219 with reactions after COVID vaccination and 115 with a history of vaccine related reactions) showed in 17% a suspicion of sensitization against the SARS-CoV-2 vaccine and/or their ingredients defined as one positive skin test and/or BAT. The majority of the SPT/IDT with the vaccines were negative. Of the 214 patients with suspected allergic symptoms after the first vaccination, 67/67 patients tolerated the re-vaccination. In this study, 334 individuals of a cohort resembling >2000 persons presenting for an allergy workup regarding SARS-CoV-2 vaccination only 45 were diagnosed in concordance with the anaphylaxis definition of the Brighton collaboration with anaphylactic immediate hypersensitivity reaction after SARS-CoV-2 vaccination. Identifiable characteristics of these patients with suspected, but also diagnosed SARS-CoV-2 vaccine hypersensitivity were female gender and the symptom angioedema. Overall, IgE-mediated hypersensitivity towards SARS-CoV-2 vaccines is extremely low and not increased in comparison to the reported hypersensitivity for other vaccines.

The Role of Bradykinin Receptors in the Etiopathogenesis of Chronic Spontaneous Urticaria

Background and Objectives: Chronic spontaneous urticaria (CSU) is a distressing skin condition, which manifests as red, swollen, itchy, and sometimes painful hives or wheals appearing on skin. Recently, CSU has been associated with bradykinin release, which was previously discovered to be the main trigger of hereditary angioedema attacks. To study the role of bradykinin receptors 1 (BR1) and 2 (BR2) in the etiopathogenesis of CSU. Materials and Methods: A total of 60 individuals, 30 patients with CSU and 30 healthy subjects, were recruited to the study. CSU was diagnosed in accordance with the standardized protocol of dermatological assessment of skin symptoms. The level of bradykinin receptors was determined in populations of CD3+, CD4+, and CD8+ lymphocytes as well as in CD14++CD16−, CD14++CD16+ and CD14+CD16+ monocytes. In addition, urticaria activity score summed over 7 days (UAS-7) was assessed and correlated with BR1 and BR2 expression. Results: A statistically significant higher concentration of BR1 expression in lymphocytes was found in patients with CSU, compared to the control group (p < 0.001). Moreover, a statistically significant positive correlation was observed between UAS-7 and BR1/BR2 expression in CD14++CD16− cells (p = 0.03, R = 0.4). Conclusions: Bradykinin receptors are elevated in selected populations of lymphocytes in symptomatic CSU patients compared to healthy controls, indicating their role in the etiopathogenesis of the disease.

Modern approach to the treatment of patients with androgen-dependent dermatopathies

Introduction. About 10–20% of patients among women of reproductive age suffer from signs and symptoms of androgen excess in hyperandrogenism. Its skin symptoms may include hirsutism, acne, seborrhea and alopecia.Aim. To assess the effect of cyproterone acetate on skin symptoms of hyperandrogenism and improvement in the patients’ quality of life after treatment.Materials and methods. The study included 120 patients with skin symptoms of hyperandrogenism. The patients ranged in age from 18 to 35 years. The inclusion criteria were the main complaints of the patients as follows: acne, hirsutism and alopecia. The patients underwent a comprehensive clinical and laboratory examination. The drug of choice for the treatment of androgendependent dermatopathies was cyproterone acetate at a dose of 50–100 mg daily.Results and discussion. All patients were divided into three groups: 44 (37%) patients with hirsutism, 55 (53%) with acne, 12 (10%) with alopecia. Moreover, 32 (27%) patients had a mix of symptoms at the same time. Such patients were assigned to one of the groups depending on the predominant complaint. All patients were prescribed cyproterone acetate at a dose of 50-100 mg once daily, depending on the severity of symptoms from day 1 to day 10 of the menstrual cycle, and 54 (52%) patients were prescribed cyproterone acetate 20 mg and ethinyl estradiol 35 mg from day 1 to day 21 of the menstrual cycle. The study showed stabilization of the psychoemotional state, relief of anxiety, depressive disorders, enhancement of self-esteem and sociability, and improvement of the quality of life in its various manifestations in the majority of patients after the treatment.Сonclusion. When assessed using the dermatology life quality index questionnaires, all patients reported the stabilization of the psychoemotional state and the improvement in the quality of life after the treatment. The anxiety and depressive disorders decreased in 109 (91%) patients, which favourably influenced various areas of quality of life, enhanced self-esteem and sociability with a positive effect on personal relationships.

Sexuality in Persons With Hidradenitis Suppurativa: Factors Associated With Sexual Desire and Functioning Impairment

Hidradenitis Suppurativa (HS) is a chronic skin disease involving intimate and sensitive areas and affecting physical and mental health. We investigated the prevalence of sexual desire and functioning impairment, and their associations with quality of life, anxiety, depression, minor psychiatric disorders (MPD), and clinical features (e.g., disease severity) in 77 patients with HS who completed self-report measures and answered to questions assessing socio-demographic characteristics, lifestyle habits, and hindered sexuality due to HS. The majority of patients reported hindered sexuality, and poor sexual functioning, while showing good levels of dyadic and solitary sexual desire. No associations were found between clinical severity and sexuality measures. Multivariate analyses showed significant associations of sexual outcome measures with alcohol consumption, low Body Mass Index, family history of HS, and severe skin symptoms. Moreover, we found that the presence of negative psychological factors (i.e., MPD, anxiety, poor mental status) increased the risk of sexual impairment. These findings underline the important role of psychological and sexual aspects in HS patients and suggest that physicians should consider the effect of disease burden on patients' sexual health.

Oral and Skin Lesions Among Yemeni Khat Abusers While Chewing and After Quitting：Crosse-Sectional Survey

Background: Khat chewing is a routine practice in Yemen. Associations between Khat chewing and various adverse oral-dental health conditions have been recorded, however, adverse skin health evidence is still lacking. Objective: To investigate the difference between oral and skin symptoms and signs while chewing Khat and after quitting chewing among Yemeni khat abusers. Method: A descriptive cross-sectional study was conducted on a sample of 300 Yemeni Khat ex-chewer participants in china. Khat chewing status data, a range of oral and skin symptoms and signs, and other pertinent variables were collected using self-administered questionnaires by WeChat. The mean differences between oral and skin symptoms and signs while chewing Khat and after quitting chewing were investigated using the Paired Samples t-test. Result: Of the 300 Yemeni who participated in the study, 255(85%) were men and 45(15%) were women. The oral symptoms and signs mean while chewing was 0.26, 0.022 (standard deviation [SD], whereas that after quitting chewing Khat was 0.10 (mean), 0.269 (standard deviation [SD], t (12.00), P=0.00. The skin symptoms and signs mean while chewing was 0.13, 0.28 (standard deviation [SD], whereas that after quitting chewing Khat was M=0.06, 0.21(standard deviation [SD], t (9.407), P=0.00. Conclusions: There is a statistically significant difference between the mean oral and skin symptoms and signs while chewing and after quitting, whereas long-term Khat chewing negatively affects oral and skin health.