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Abstract Introduction Data on generalized pustular psoriasis (GPP) in Poland are limited. Therefore, this study aimed to evaluate the demographic and clinical characteristics of Polish patients with GPP. In addition, the effect of the frequency of disease flares and the concomitant presence of psoriasis vulgaris (PV) on patient characteristics was evaluated. Methods In this retrospective study, medical records of patients hospitalized for GPP in dermatology departments of the Polish Generalized Pustular Psoriasis Group were analyzed. Results A total of 90 patients from 14 dermatology centers were included. The median age of patients was 59 (range, 5–85) years, and most patients were female (68.9%). The most common comorbidity was hypertension (43.3%), followed by cardiovascular diseases, diabetes, and hyperlipidemia. The median duration of GPP was 3 years, and the median number of flares was 2. Hospitalization was required in 67% of flares. The main triggering factors were drugs (20.5%) and infections (19.3%). Women and men with GPP differed in terms of age (p

Hidradenitis suppurativa is a chronic, recurrent, inflammatory skin disease that affects hair follicles, typically developing after puberty. It is characterized by painful, deep-seated inflammatory lesions that appear in anatomical regions where apocrine glands are located, mainly in the axillar, inguinal, and anogenital areas. The pathogenesis of hidradenitis suppurativa is multifactorial, with follicular occlusion representing the initial stage of the disease and subsequently leading to the formation of neutrophilic abscesses and infiltration by various inflammatory cells. The treatment of hidradenitis suppurativa requires a multidisciplinary approach, involving dermatologists and dermatologic surgeons, as well as physicians of other medical specialties. The primary pharmacological treatments for hidradenitis suppurativa involve antibiotics and biologic disease-modifying drugs, which can be used either in monotherapy or in combination with surgical interventions. The following guidelines provide up-to-date information on the management of patients with hidradenitis suppurativa.

Background. Systemic sclerosis (SSc) is an autoimmune disease characterized by inflammation, progressive vasculopathy, and fibrosis of skin and internal organs. The aim of the study was to evaluate extended inflammatory parameters (EIP) in patients with SSc in comparison to the control group of healthy subjects. Methods. A total of 28 patients with SSc and 29 healthy controls (HCs) were included in the study. The following EIP parameters were analyzed: neutrophil reactive intensity (NEUT-RI), neutrophil granularity intensity (NEUT-GI), antibody-synthesizing lymphocytes (AS-LYMP), and reactive lymphocytes (RE-LYMP). Results. Patients with SSc showed significantly higher values of parameters determining neutrophil reactivity and neutrophil granularity when compared to HCs (respectively, 49.16 FI vs. 44.33 FI, p

Rosacea is a chronic inflammatory skin disease characterised by periods of remission and subsequent relapses typically provoked by environmental factors. Eruptions located on the face are easily visible and may be difficult to conceal, which leads to a decrease in patients’ self-esteem, quality of life, and general well-being. Since the aetiology of rosacea is multifactorial and not entirely understood, and the clinical characteristics and course of the disease are heterogeneous, therapy may be challenging. While a complete cure is not possible, in the majority of cases clinical improvement and long-term remission are satisfying both to the patient and physician. In recent years, the therapeutic approach has been significantly modified. Currently, the focus is placed on the need to personalise the treatment process and adapt it to the patients’ phenotypic characteristics. Combination therapies and new variants of existing topical and systemic drugs are increasingly being recommended. The mainstays of therapy are antiinflammatory agents. Proper skin care – along with the identification and avoidance of factors known to trigger consecutive episodes of rosacea – are also essential.

Rosacea is a chronic, relapsing, inflammatory, but not infectious, skin disease. The prevalence of rosacea is relatively high in the adult population of Northern Europe. Its characteristic symptoms include recurrent flushing, erythema, telangiectasia, papules and pustules, localized on cheeks, nose, chin and forehead. At present, four, not mutually exclusive subtypes of acne are classified: erythematotelangiectatic, maculopapular, hypertrophic and ocular. The clinical presentation of this dermatosis is heterogeneous. Clinical symptoms and their severity may vary individually. For this reason, treatment should be individualized and take into consideration the type of cutaneous lesions, presence of comorbidities, patient’s occupation, impact of the disease on the quality of life, and patient’s preferences. In addition to topical and systemic medications, adequate skin care is important.

Psoriasis is a chronic inflammatory skin disease which concerns approx. 1-3% population worldwide. In children psoriasis is less common, with the peak of morbidity in teenagers. Because of its more common prevelance in children in last decade, especially the disease with moderate and severe course, it is necessary to remind its clinical features and comorbidities. The aim of the recommendation of Polish Dermatological Society was to give the newest data on the psoriasis treatment in pediatric population which may be extremely important for everyday dermatological treatment. We described both topical and systemic treatments in details, including biological therapies.

Dermatomyositis is an autoimmune disease characterized by skin lesions and/or symptoms of myositis. In addition to the so-called classic dermatomyositis the following forms of dermatomyositis are distinguished based on the clinical presentation: pediatric, paraneoplastic, drug-induced and amyopathic. A number of disease-specific autoantibodies are identified in dermatomyositis (including anti-Mi2, anti-TIF1, anti-NXP2, anti-SAE or anti-MDA5), presence of which may be associated with a specific clinical phenotype. The diagnosis of the severity of muscle involvement is currently based mainly on physical examination, deviations in results of laboratory investigations, electromyographic examination and imaging examination, mainly with the use of magnetic resonance imaging. Systemic glucocorticosteroids administered as monotherapy or in combination with other immunosuppressants remain the mainstay of dermatomyositis treatment, and in the absence of satisfactory improvement, intravenous immunoglobulins are used. In addition, in case of interstitial lung disease, the use of cyclophosphamide may be necessary. The choice of a therapy, as well as the rate of dose reduction, depend on the dynamics of the disease, symptoms, diagnosed immunological disorders, as well as comorbidities and the drugs used. Each diagnostic and therapeutic decision must take into account the individual clinical data of the patient and current scientific reports.

Actinic keratosis, a type of squamous cell carcinoma in situ, is characterised by hyperkeratotic focal lesions formed on the ultraviolet-damaged skin. Usually, there are a few to several so foci of actinic keratosis, and less often a single lesion is present. The treatment depends on the number, size, duration, and location of lesions. This study discusses some options of combined treatment of actinic keratosis and the possible risk of cancer associated with the use of photodynamic therapy in combination with laser therapy, topical medications (imiquimod, 5-fluorouracil, diclofenac, topical retinoids, ingenol mebutate, calcipotriol), and microneedling. Combined treatment is worth considering in cases of numerous hyperkeratotic foci, particularly in difficult-to-treat locations. Effective photoprotection is essential for all persons with actinic keratosis.

The basis for development of atopic dermatitis are genetic, immunological and microbiological abnormalities as well as epidermal barrier defects. The course of the disease includes periodic exacerbations and remissions. Its development and course are influenced by numerous environmental and individual factors. In recent decades, in industrialized countries, a threefold increase in the incidence of atopic dermatitis was observed. There also is an increasing number of cases resistant to topical treatment. Effective treatment of atopic dermatitis should provide control of clinical symptoms, prevent exacerbations and improve the quality of life of patients. The multifactorial etiopathogenesis and various endotypes and phenotypes of the disease justify the tendency to optimize and personalize the therapy. Currently, we recommend the use of dupilumab for the treatment of patients from 12 years of age with moderate and severe atopic dermatitis, who do not respond to topical treatment.

Scars are formed when a defect in the dermis is replaced with fibrous connective tissue. Sometimes scars limit the range of motion as a result of contractures. In addition, they are a source of severe subjective complaints and a significant cosmetic defect. People with visible scarring have been shown to have a reduced quality of life due to a sense of shame, anxiety, and the lack of public acceptance. The therapy of scars can be challenging, and may be associated with a risk of recurrence. Fractional laser treatments are currently a widely used therapeutic approach for scar management, both in paediatric and adult patients. The observed improvement in skin colour, decrease in tissue tension, and reduction of itching and pain experienced by the patient, combined with a low risk of adverse effects, determine the efficacy and safety of this treatment modality, and contribute to the satisfaction of patients with the final cosmetic result, thus improving their mental and emotional well-being.

Localized scleroderma is an autoimmune disease affecting the dermis and underlying tissues (subcutaneous tissue, fascia, muscles, bones). The disease mainly occurs in women aged 20–50 years; however, children aged 7–10 years represent about 15% of all cases. Clinical course of juvenile localized scleroderma is classified with regard to its activity/severity as well as extent and depth of lesions. Some clinical subtypes of juvenile localized scleroderma, characterized by severe course, need to be monitored regularly to prevent irreversible sequelae. The treatment of juvenile localized scleroderma depends on the clinical subtype of the disease, its severity/activity and extent of skin lesions.

Psoriasis is one of the most common chronic inflammatory dermatoses. We present the second part of the diagnostic and therapeutic guidelines on psoriasis. The second part discusses the principles of treatment of plaque psoriasis, with a particular emphasis on systemic drugs. The second part of the guidelines constitutes an integral whole with the first part which addresses the general principles of diagnostic management, prevention of cardiovascular complications, therapeutic goals, and selected specific forms of psoriasis. The guidelines have been updated to take account of access to new therapies which have recently been approved in Poland, ongoing improvements in the efficacy of available therapeutic methods, and changing needs of patients. We hope that the current guidelines will assist physicians in making diagnostic and therapeutic decisions, and contribute to the improvement of medical care provided to patients with psoriasis.

Psoriasis is a chronic inflammatory disease affecting approximately 1–3% of the general population. Although skin lesions are the primary clinical manifestation of the condition, psoriasis – especially moderate to severe – is currently regarded as a systemic disease, not only associated with the development of psoriatic arthritis but also linked to a range of other health consequences including cardiovascular complications. In view of new data on the pathogenesis of psoriasis, very rapid progress in the development of new therapeutic approaches, and in particular the registration of new drugs, a need arose to update the guidelines for the diagnosis and treatment of this nosological entity. The aim of the recommendations of the Polish Dermatological Society is to summarise the most recent literature and formulate guidelines for the treatment of psoriasis that may be useful in daily dermatological practice. However, the final decision regarding the diagnostic and therapeutic procedure should always be made individually for each patient based on the patient’s current clinical status and the most up-to-date scientific reports.

From 5% to 40% patients with psoriatic skin lesions develop psoriatic arthritis, but 15% to 30% of patients with psoriasis suffer from undiagnosed psoriatic arthritis. The key role in development o-f psoriasis is played by T-lymphocytes and release of proinflammatory cytokines, interferon and adhesive proteins. Infection may exert an influence on psoriasis vulgaris and psoriatic arthritis development. The presence of antibodies against the antigens of Yersinia spp., Campylobacter spp. and Chlamydia trachomatis was more frequently observed in the serum of patients with psoriatic arthritis. Diagnosis of psoriatic arthritis may be based on CASPAR criteria. Diagnostic imaging (CT, MRI, USG) are routine diagnostic procedures in psoriatic arthritis. Several sub-types of psoriatic arthritis have been identified. Questionnaires to identify early stages of the disease have been constructed. Genetic factors, cigarette smoking, alcohol abuse, infections, stress and environmental factors may affect clinical course and results of treatment of psoriasis vulgaris and psoriatic arthritis. In severe cases, the disease may lead to disability.

The action of fractional lasers is based on the principle of fractional photothermolysis, which consists in generating in the skin a mesh of microthermal treatment zones surrounded by healthy, undamaged tissue. The most important factors determining the effectiveness of the procedure and the risk of developing adverse effects are: depth, size and density of microthermal treatment zones. The laser beam energy set by the operator is responsible for the depth of microthermal treatment zones penetration into the skin. The amount of intact, healthy tissue remaining between the micro damage zones and their depth depends on the number of laser beam passes. This draws attention to the need for careful selection of treatment parameters, which can only be performed by a person with appropriate qualifications. Fractional laser treatments are an effective form of therapy for many skin diseases and they have a low risk of side effects, most of which are short-term and harmless.

The aim of this study was to evaluate the nailfold videocapillaroscopic examination results from patients with pseudoexfoliative glaucoma (XFG) and to assess the relationship between the results of this examination and the patient’s clinical status in the XFG group. Material and Methods: The studied group consisted of 39 Caucasian patients with XFG and 32 patients in a control group. The patients were classified into two subgroups: the hypertensive pseudoexfoliative glaucoma (hXFG) subgroup and the normotensive pseudoexfoliative glaucoma (nXFG) subgroup. The nailfold videocapillaroscopy (NVC) was performed on all participants. The results of each NVC were classified as having a normal or abnormal pattern. Results: There was no statistical difference between the results of an abnormal NVC pattern in the study group vs. the control group (p = 0.8773). Microhemorrhages were shown in 30.0% of patients with nXFG vs. the control group (6.25%) (p = 0.0520). Microhemorrhages tended to be more frequent in the XFG group (p = 0.1221). A prevalent number of tortuous capillaries was observed in hXFG patients with advanced glaucomatous neuropathy. Dilatation in the capillaries and microbleedings were observed in the group of patients with lower IOP values. Tortuosity in the capillaries was significantly more frequent in PEXG patients (XFG vs. control: p = 0.0386). No relationships between the results of NVC and age, c/d, BCVA, time of treatment, and visual field defect were found. Conclusions: Specific features of NVC examination differentiate nXFG from hXFG patients. Some capillaroscopic features may correlate with the patient’s clinical status of XFG.

The treatment goal in atopic dermatitis is eliminating clinical symptoms of the disease, preventing exacerbations and complications, as well as improving patients’ quality of life. In cases of severe atopic dermatitis and lack of response it is recommended to introduce systemic therapy. Patients ofter require multi-specialist consultations, and occasionally hospitalization. It is not recommended to use acupuncture, acupressure, bioresonance, homeopathy, or Chinese herbs in the treatment of atopic dermatitis.

Localized scleroderma (morphea) is a chronic autoimmune disease of the connective tissue. Its etiopathogenesis is unknown. Most often the disease affects the skin, but may also involve the subcutis, muscles, and the osteoarticular system. The clinical presentation and the course of disease may be diverse. This article presents clinical characteristics of various types of the disease, the classification of its subtypes, recommendations for differential diagnosis and treatment.

Atopic dermatitis is a chronic and recurrent inflammatory dermatosis with concomitant intensive pruritus, and is diagnosed both in children and adults. Atopic dermatitis -patients are predisposed to have bacterial, viral and fungal skin infections; they also suffer from an increased risk of developing food allergies (especially, at an infantile age), allergic rhinitis, or bronchial asthma (a so-called atopic march). Currently, an increasing atopic dermatitis incidence constitutes a serious medical problem that regards not only dermatology and allergology, but also paediatrics, and family medicine. The basis for atopic dermatitis treatment and prophylaxis is restoration of epidermal barrier functions by means of tailored emollients. Atopic dermatitis therapies should effectively eliminate clinical symptoms of the disease, prevent exacerbations as well as complications, and improve patients’ quality of life.

Introduction Psoriasis and psoriatic arthritis (PSA) are chronic, inflammatory, systemic diseases characterized by metabolic abnormalities, including an increased cardiovascular risk and an oxidative imbalance. This study assessed blood parameters of lipid metabolism and markers of oxidative stress in patients with psoriasis and PSA. Material and methods The study included 93 patients with psoriasis (31 patients with PSA and psoriasis, 62 patients with psoriasis vulgaris), and 60 healthy, age-matched controls. Serum concentrations of the glucose and the following lipid metabolism parameters were measured: triglycerides (TG), total cholesterol (TC), low-density lipoproteins (LDL), very low-density lipoproteins (VLDL), high-density lipoproteins (HDL), and apolipoproteins A and B (ApoA, ApoB). Oxidative status was determined as serum concentrations of ox-LDL/MDA Adduct. The Psoriasis Area and Severity Index (PASI) was used to determine disease severity. Results Among the three studied groups, controls had the highest HDL concentration (p < 0.001), patients with PSA had the highest ApoB concentration (p < 0.05), ApoA : ApoB ratio (p < 0.05), ox-LDL/MDA adduct concentration (p < 0.001), and TC: HDL and LDL : HDL ratios (accordingly p < 0.05, p < 0.01). In patients with psoriasis or PSA, oxidative status correlated positively with TC and ApoB concentrations. Conclusions In line with previous research, among patients with psoriasis and PSA, we found lipid metabolism abnormalities and an oxidative imbalance, which might be due to chronic inflammation in these conditions. Effective treatment of patients with psoriasis or PSA could reduce the risk of cardiovascular diseases.

Sulodexide is a highly purified mixture of glycosaminoglycans composed of fast mobility heparin (80%) and dermatan sulfate (20%). Glycosaminoglycans constitute a group of heteropolysaccharides consisting of repeating disaccharide units, which contain amino sugar combined with uronic acid or a hexose. Glycosaminoglycans build glycocalyx covering endothelium of blood vessels, and thus, play an important role in vascular homeostasis. Multi-directional activity of sulodexide including antithrombotic, fibrinolytic, anti-aggregation, anti-inflammatory, anti-proliferative, and anti-oxidative qualities, as well as a high safety profile have allowed using the drug in numerous entities. Advantageous results of sulodexide therapy are observed in diseases involving endothelial damage, prothrombotic or inflammatory states. Thanks to its pleiotropic effects, sulodexide can be also applied in treatment of skin diseases.

Vitiligo is an idiopathic chronic dermatosis manifested by depigmented patches of skin due to the loss of normal pigment-producing cells (melanocytes) and pigment (melanin). The disease develops more commonly in genetically predisposed individuals affected by various adverse external and internal factors inducing cellular stress in melanocytes. Cellular stress activates autoimmune and autoinflammatory mechanisms leading to melanocyte destruction. The disorder affects 0.5–4.0% of the world population, with no predilection for either sex. Manifestations can appear at any age, however in more than half of patients vitiligo starts before the age of 20 years. The course of the disease is varied – from stable cases with small isolated vitiligo patches to the rapid progression of lesions resulting in large areas of the skin being affected. There are multiple methods of treating vitiligo including pharmacological and surgical modalities, and phototherapy. Another essential aspect is patient education. Available treatment methods do not always yield the expected therapeutic effect. The Recommendations contain information about the diagnosis of vitiligo, and describe the recommended therapeutic methods.

(1) Background: Pemphigus is a blistering autoimmune disease of the skin and/or mucous membranes, characterised by the presence of specific autoantibodies directed against structural proteins of the human skin. Recent reports indicate that new haematological parameters, termed Extended Inflammation Parameters (EIP), can be used to assess the activation of immune cells during active inflammation. These include parameters assessing both neutrophil activation (NEUT-RI, NEUT-GI) and the number of activated lymphocytes (RE-LYMP). The aim of this study was to investigate the relationship between changes in NEUT-RI, NEUT-GI and RE-LYMP and the disease activity in patients with pemphigus. (2) Results: The study involved 32 patients with diagnosed different types of pemphigus. Neutrophil activation parameters (NEUT-RI and NEUT-GI) and lymphocytes (RE-LYMP) were significantly higher in these patients compared to the parameters in healthy participants (respectively p = 0.0127, p = 0.0011 and p = 0.0033). The increased quantity of activated lymphocytes (RE-LYMP) also correlated significantly with the extent of skin and/or mucosal lesions in patients assessed by the PDAI scale (p < 0.02). (3) Conclusions: The NEUT-RI, NEUT-GI and RE-LYMP parameters proved to be appropriate markers of inflammation severity in pemphigus, also in relation to local lesions, which was not possible with the inflammation markers (CRP, ESR) used so far on a routine basis.

Psoriasis is an inflammatory, autoimmune disease that affects approximately 2% of the population. The inflammation in psoriasis can be systemic, so despite a predominantly cutaneous manifestation, it also affects the internal organs. The diagnosis and monitoring of the disease are based on the clinical picture. To assess the disorders of other organs, additional tests need to be performed. Recently, the examination of blood morphology has been enriched with modern haematological parameters, i.e., Extended Inflammation Parameters (EIP), which include RE-LYMPH (activated lymphocytes), AS-LYMPH (antibody-producing B lymphocytes), and NEUT-RI and NEUT-GI (activated neutrophils). In the study, higher values of new haematological parameters were observed in individuals with psoriasis than in healthy controls. A higher EIP value was noted in the group of individuals with plaque psoriasis than in the group of individuals with psoriatic arthritis. Implementation of these parameters into routine laboratory analysis will likely make it possible to estimate the severity of the inflammation and improve its assessment.

Metalloproteinases (MMPs) and cytokines have a great impact on the pathogenesis of psoriasis. Cytokines, as key mediators of inflammation and autoimmune processes, play a crucial role in the regulation of MMP expression in different cell types. Parallel, MMPs have an influence on cytokine production. This interaction was not well recognized in psoriatic patients. Our study is aimed at assessing the selected serum MMP levels and their correlations with cytokine levels in the serum of psoriatic patients. We observed a significantly elevated level of pro-MMP-1 and MMP-9 in psoriatic patients’ serum in comparison to the control group. We did not observe any statistically significant differences of MMP-3 and pro-MMP-10 between the psoriatic patients and the control group. We did not observe any statistically significant differences in all the studied MMP levels between the patients with and without psoriatic arthritis (PsA). MMP-3 level correlated positively with proinflammatory cytokines, i.e., IL-12p/70, IL-17A, and TNF-α as well as MMP-3 and pro MMP-1 correlated positively with IL-4 in the psoriatic patients. In the control group, a positive correlation between pro-MMP-1 and TNF-α was found. These results confirm MMPs and Th1 and Th17 cytokine interaction in the inflammatory regulation in psoriasis.

Photodynamic therapy (PDT) is safe and effective in the treatment of patients with actinic keratosis (AK). The aim of the study was to assess the efficacy, tolerability and cosmetic outcome of topical PDT in the treatment of AKs with three forms of photosensitizers: 5-Aminolevulinic acid hydrochloride (ALA-HCl), 5-Aminolevulinate methyl ester hydrochloride (MAL-HCl) and 5-Aminolevulinate phosphate (ALA-P). The formulations were applied onto selected scalp/face areas. Fluorescence was assessed with a FotoFinder Dermoscope 800 attachment. Skin areas were irradiated with Red Beam Pro+, Model APRO (MedLight GmbH, Herford, Germany). Applied treatments were assessed during the PDT as well as 7 days and 12 weeks after its completion. Ninety-four percent of patients rated obtained cosmetic effect excellent. The efficacy of applied PSs did not differ significantly. However, pain intensity during the PDT procedure was significantly lower in the area treated with ALA-P (5.8 on average) in comparison to the areas treated with ALA-HCl or MAL-HCl (7.0 on average on 0–10 scale). Obtained results show that ALA-P may undergo more selective accumulation than ALA-HCl and MAL-HCl. Our promising results suggest that PDT with the use of ALA-P in AK treatment may be an advantageous alternative to the already used ALA-HCl and MAL-HCl.

Psoriatic arthritis (PsA) is a seronegative arthropathy with many clinical manifestations, and it may affect nearly a half of patients with psoriasis. PsA should be diagnosed as early as possible to slow down joint damage and progression of disability. To improve the diagnosis of PsA, physicians should look for peripheral inflammatory pain, axial inflammatory pain, dactylitis, and buttock and sciatic pain. In most patients with PsA, pharmacologic treatment with non-steroidal anti-inflammatory drugs, disease-modifying anti-rheumatic drugs, and biologic agents is effective. However, when pharmacological treatment fails, patients with PsA may benefit from orthopedic surgery, which can improve both joint function and quality of life. Total hip arthroplasty, total knee arthroplasty, and arthroscopic synovectomy of the knee are the most common surgical procedures offered to patients with PsA. The management of PsA requires the care of a multidisciplinary team, which should include dermatologists, rheumatologists, physiotherapists, and orthopedic surgeons.

Background: Eyebrow loss in the course of frontal fibrosing alopecia (FFA) is becoming a growing issue among older females. It has a considerable negative impact on patients’ quality of life. Since there is no standardized treatment, photobiomodulation with light-emitting diodes (LEDs) could be an option. Here we assess, for the first time, the efficacy of LED therapy in the treatment of eyebrow loss in females with FFA. Methods: 16 female patients with FFA aged 60–74 years were enrolled in the study. LED therapy was performed once a week for a 10-week session. The LEDs’ effectiveness was assessed at the baseline, after 10 irradiations, and 6 months after the end of treatment during a follow-up visit. Results: The therapy was well tolerated. After 10 irradiations, the total eyebrow hair count increased significantly, as did the number of thick hairs and mid-thick hairs (p = 0.002, p = 0.002, and p = 0.044, respectively). During the follow-up visit, the total number of eyebrow hairs remained significantly higher than before treatment (p = 0.002). Conclusion: The study revealed that LED therapy seems to be a novel and promising therapeutic option for eyebrow loss in patients with FFA. It is safe and well tolerated and leads to clinically and cosmetically acceptable improvement.

Advances in genotypic technologies enable identification of possible associations between genetic variants of certain genes and increased risk of developing plaque psoriasis or psoriatic arthritis. The aim of the study was to analyze the NOTCH3 (6746T>C) (rs1044009) and PSMA6 (-8C>G) (rs1048990) polymorphisms and their role in genetic susceptibility to psoriasis. The study included 158 psoriatic patients and 100 healthy controls. The frequencies of the NOTCH3 genotypes differed between the psoriatic patients and healthy controls (p = 0.050). No differences were found in the distribution of PSMA6 genotypes and alleles between the psoriatic patients and healthy controls. The studied psoriatic patients presented a higher frequency of the CC genotype of PSMA6 compared to the healthy controls (8.8% vs. 2%, respectively). Psoriatic arthritis was more frequent among patients with the CC genotype of PSMA6 (p = 0.059). CC homozygosity of NOTCH3 was more commonly observed in the studied psoriatic patients than in the healthy controls (OR = 4.76, p= 0.032). The obtained data suggest that genetic variants of NOTCH3 (6746T>C) and PSMA6 (-8C>G) genes may play significant roles in psoriatic patients. Further studies are necessary to unequivocally determine their role as genetic risk factors of psoriasis development.

Systemic sclerosis is an immune-mediated disease characterized by a chronic progressive course. The complex and diverse clinical features seen in patients require case-by-case approach and cooperation of multiple specialists both at the stage of diagnosis and treatment. Critical factors in systemic sclerosis include early diagnosis, assessment of internal organ involvement, identification of patients at potential risk of organ complications, assessment of disease dynamics and activity, and implementation of optimal therapy. Treatment decisions should be made individually for each patient after careful consideration of such aspects as the severity of skin lesions, duration and activity of the disease, manifestations and possible internal organ involvement. On account of potential organ dysfunctions, patients require a multidisciplinary therapeutic approach and coordinated treatment by experienced specialists or in specialist medical centres. Part 2 presents current guidelines for the treatment of systemic sclerosis.

Localized scleroderma (morphea) is a connective tissue disease that affects the skin, subcutaneous tissue and underlying tissues including the fasciae, muscles and bones. The treatment of localized scleroderma depends on the clinical form of the disease, its severity/activity, extent of skin lesions and patient’s age. Patients with limited skin involvement caused by morphea require topical therapy with glucocorticoids, calcineurin inhibitors, calcipotriol or UVA/PUVA phototherapy. More severe variants of localized scleroderma with extensive tissue involvement require immediate systemic therapy with glucocorticoids and/or methotrexate, or mycophenolate mofetil. According to recent reports, attempts have also been taken, with variable clinical outcomes, to treat localized scleroderma topically with pirfenidone and imiquimod, systemically with tacrolimus and cyclosporine; and with fractional CO 2 laser, photodynamic therapy and autologous fat transfer. The article presents current therapeutic methods of localized scleroderma.

Systemic sclerosis is an immune-mediated disease characterized by a chronic and progressive course. It often leads to multiorgan failure and patient disability, and contributes to significant reduction in the quality of life. Systemic sclerosis affects the skin, subcutaneous tissue, muscles, osteoarticular system and internal organs. The complexity and diversity of clinical presentations require an individual approach and multidisciplinary collaboration both at the stage of diagnosis and treatment. Critical factors in systemic sclerosis include early diagnosis, assessment of internal organ involvement, identification of patients at potential risk of organ complications, assessment of disease dynamics and activity, and implementation of optimal therapy. Part 1 presents current recommendations for the diagnostics and monitoring of patients with systemic sclerosis. Attention is given to classification criteria, clinical forms of systemic sclerosis, assessment of skin thickness and systemic sclerosis microangiopathy, significance of antinuclear antibodies, diagnosis of interstitial lung disease and pulmonary arterial hypertension, renal crisis and cardiac abnormalities, and evaluation of the gastrointestinal tract and osteoarticular and muscular systems.

Psoriasis is a long-lasting inflammatory skin disease with an autoimmunological and immunometabolic background. The latest European data indicate that the occurrence of psoriasis in children ranges from 0.7% to 2.1%. Despite distinct clinical characteristics, this disease continues to cause diagnostic difficulties and is often incorrectly diagnosed. The goal of this article is to present the course of psoriasis in children, basic diagnostic techniques, including dermoscopy, influence of the disease on the quality of life and risk of co-morbidity development. The therapeutic approcach to childhood psoriasis is presented.

Hidradenitis suppurativa (acne inversa) is a chronic, inflammatory, recurrent, debilitating disease of hair follicles manifested by painful, deepseated, inflamed lesions localized in intertriginous areas. Establishing a correct diagnosis seems straightforward enough, as it is based on clinical criteria, but a comparative analysis of published papers on hidradenitis suppurativa epidemiology easily reveals substantial discrepancies in the estimation of the obtained results for the general population. Data on the prevalence of hidradenitis suppurativa in European countries and in the United States of America differ by up to four orders of magnitude, ranging from 0.00033% to 4.1%. Based on data obtained from the Polish National Health Fund and HS reference centres, the prevalence of hidradenitis suppurativa in Poland was estimated at 0.001%, which classifies hidradenitis suppurativa as a rare disease.

Systemic sclerosis is a chronic connective tissue disease characterized by microvascular abnormalities, immune disturbances and progressive fibrosis of the skin and internal organs. Skin involvement may result in contractures, leading to marked loss of hand mobility, adversely affecting the performance of daily activities and decreasing the quality of life. Face involvement not only causes functional loss, but also lowers the self-esteem of patients. Increasing attention has recently been focused on the need to rehabilitate patients with systemic sclerosis in order to prevent the development of joint contractures and loss of mobility. The study presents a review of the current literature on rehabilitation possibilities in patients with systemic sclerosis, with a special focus on physiotherapy methods.

Localized scleroderma is a rare autoimmune disorder affecting the dermis, subcutaneous tissue and deeper structures. The course of localized scleroderma includes three stages: early inflammation, progressive sclerosis and atrophy. The active stage of the disease is characterized by erythema (‘lilac ring’), skin induration, and appearance of new or expansion of pre-existing skin lesions during the preceding month. The most recent localized scleroderma classification recognizes five types of the disease: localized, generalized, linear, deep and mixed. Various methods have been proposed for the evaluation of disease activity or severity and tissue damage, including ultrasonography, thermography, durometry, computed tomography and magnetic resonance imaging. However, all of them have their limitations, as they only make it possible to assess one component of the disease: activity or severity or tissue damage. The LS Cutaneous Assessment Tool (LoSCAT) questionnaire is a promising method of patient evaluation because of its availability, repeatability of results and sensitivity to changes resulting from treatment.

Chronic kidney disease causes skin changes which may sometimes be the first sign of kidney failure. Specific skin changes include acquired perforating dermatosis, porphyria cutanea tarda, pseudoporphyria, calcinosis and nephrogenic systemic fibrosis. The majority of patients present with cutaneous manifestations which are classified as non-specific, including xerosis, pruritus, pigmentation disturbances, nail plate abnormalities, uraemic frost and gynaecomastia. Treatment improving kidney function (dialysis therapy or kidney transplantation) also leads to the resolution of skin lesions.