The Role of Caspases in Melanoma Pathogenesis.

Melanoma (malignant melanoma, MM) is an aggressive malignant skin cancer with an increasing incidence rate. The complete pathogenesis of MM in not clear. Due to DNA damage, mutations, dysregulation of growth factors, inactivation of tumor suppressor genes, and activation of oncogenes, excessive uncontrolled growth of abnormal melanocytes occurs in melanomas. Caspases are a group of proteolytic enzymes that participate in several processes important in regulating mechanisms at the cellular level. They play a role in cell homeostasis and programmed cell death (apoptosis) and in the regulation of non-apoptotic cell death processes. Dysregulation of caspase activation plays a role in the etiology of cancers, including melanoma. Caspases can initiate and execute apoptosis and are involved in regulating cell death and controlling tumor growth. These enzymes also inhibit tumor growth by cleaving and inactivating proteins that are involved in cell proliferation and angiogenesis. Moreover, caspases are involved in the activation of immune processes through the processing and presentation of tumor antigens, which facilitates recognition of the tumor by the immune system. The role of caspases in melanoma is complex, and they may inhibit melanoma growth and progression. This work aims to review the current knowledge of the role of individual caspases in melanoma pathogenesis.

The elastography of distal ulnar nerve branches in cyclists.

INTRODUCTION: Forced elbow flexion and pressure during bicycling result in ulnar nerve traction and pressure exerted in Guyon's canal or the nerve's distal branches. The compression of the nerves causes a change in their stiffness related to edema and eventually gradual fibrosis. PURPOSE: This study aimed to evaluate the elastography of terminal branches of the ulnar nerve in cyclists. STUDY DESIGN: Cross-sectional study. METHODS: Thirty cyclists, 32 healthy individuals, and 32 volunteers with ulnar nerve entrapment neuropathies participated in the study. Each participant underwent a nerve examination of the cubital tunnel, Guyon's canal and the deep and superficial branches of the ulnar nerve using shear wave elastography. The cyclist group was tested before and after a 2-hour-long workout. RESULTS: Before cycling workouts, the ulnar nerve stiffness in the cubital tunnel and Guyon's canal remained below pathological estimates. Cycling workouts altered nerve stiffness in the cubital tunnel only. Notably, the stiffness of the ulnar terminal branches in cyclists was increased even before training. The mean deep branch stiffness was 50.85 ± 7.60 kPa versus 20.43 ± 5.95 kPa (p < 0.001) in the cyclist and healthy groups, respectively, and the mean superficial branch stiffness was 44 ± 12.45 kPa versus 24.55 ± 8.05 kPa (p < 0.001), respectively. Cycling contributed to a further shift in all observed values. DISCUSSION: These observations indicate the existence of persistent anatomical changes in the distal ulnar branches in resting cyclists that result in increased stiffness of these nerves. The severity of these changes remains, however, to be determined. CONCLUSIONS: These data show elastography values of the ulnar terminal branches in healthy individuals and cyclists where despite lack of clinical symptoms that they seem to be elevated twice above the healthy range.

Elastography of nerves in the wrists of cyclists.

Purpose: This study aimed to investigate changes in the elasticity of the median and ulnar nerves in cyclists. Material and methods: The study included 30 cyclists and 2 non-biking reference groups that included 32 healthy volunteers and 32 individuals with ulnar nerve entrapment neuropathies. All participants underwent physical, ultrasonographic, and elastographic examinations including assessment of nerve cross-sectional area (CSA) and stiffness (SWE). The cyclists' group was tested before and after a 2-hour workout. Results: The values of ulnar nerve CSA and stiffness in Guyon's canal in resting cyclists were 5.30 ± 1.51 mm2 and 49.05 ± 11.18 kPa, respectively. These values were significantly higher than in the healthy volunteers, but not higher than in the nerve entrapment group. Median nerve CSA and stiffness at rest were 9.10 ± 2.61 mm2 and 38.54 ± 14.87 kPa, respectively. Both values were higher than respective values in the healthy group. Cycling induced an increase in all these parameters, although the increase in nerve stiffness was more noticeable than in CSA. Conclusions: The elasticity of the median and ulnar nerve in cyclists remains within normal limits, questioning the belief that cyclists are at risk of nerve palsy in Guyon's canal. However, cycling workout does exert compression, resulting in transient oedema of both nerves. The dynamics of changes was more noticeable in SWE examination than in conventional ultrasound, which may depend on SWE sensitivity.

The Essential Role of microRNAs in Inflammatory and Autoimmune Skin Diseases-A Review.

The etiopathogenesis of autoimmune skin diseases is complex and still not fully understood. The role of epigenetic factors is emphasized in the development of such diseases. MicroRNAs (miRNAs), a group of non-coding RNAs (ncRNAs-non-coding RNAs), are one of the important post-transcriptional epigenetic factors. miRNAs have a significant role in the regulation of the immune response by participating in the process of the differentiation and activation of B and T lymphocytes, macrophages, and dendritic cells. Recent advances in research on epigenetic factors have provided new insights into the pathogenesis and potential diagnostic and therapeutic targets of many pathologies. Numerous studies revealed a change in the expression of some microRNAs in inflammatory skin disorders, and the regulation of miRNA expression is a promising therapeutic goal. This review presents the state of the art regarding changes in the expression and role of miRNAs in inflammatory and autoimmune skin diseases, including psoriasis, atopic dermatitis, vitiligo, lichen planus, hidradenitis suppurativa, and autoimmune blistering diseases.

In the Pursuit of Metabolic Markers of Systemic Sclerosis-Plasma Adiponectin and Omentin-1 in Monitoring the Course of the Disease.

Systemic sclerosis (SSc) is a connective tissue disease leading to cutaneous and visceral fibrosis. Pathological features of SSc include immune dysregulation, vasculopathy, and impaired angiogenesis. Adipokines act as cytokines and hormones and are involved in various pathological processes, including metabolic disorders, inflammation, vasculopathy, and fibrosis. This study aimed to determine the level of omentin-1 and adiponectin to evaluate their potential role in the pathogenesis of SSc. We assessed serum omentin-1 and adiponectin as well as metabolic parameters in 58 patients with SSc and 30 healthy controls. The follow-up was performed in SSc individuals. Omentin-1 levels were significantly higher in SSc individuals as compared to the controls. In post-hoc analysis, omentin-1 was higher in the group with disease duration ≥7 years than in the control group. A positive correlation was noted between disease duration and both adipokines and increased with longer disease duration. However, there were no correlations between selected adipokines and metabolic parameters. Enhanced omentin-1 levels and higher levels of omentin-1 in patients with longer disease duration may suggest that omentin-1 is involved in the pathomechanisms of SSc as its concentrations are not directly related to BMI, age, and insulin resistance.

Psychological consequences of hospital isolation during the COVID-19 pandemic - research on the sample of polish firefighting academy students.

Currently, a very important thread of research on COVID-19 is to determine the dimension of the psychopathological emotional reactions induced by the COVID-19 pandemic. A non-experimental online research project was designed to determine the predictors of the severity of psychopathological symptoms, such as depression and PTSD symptoms, and the nature of the feedback mechanism between them in groups of men, remaining in hospital isolation due to infection and at-home isolation during the COVID-19 epidemic. The presence of symptoms of depression, post-traumatic stress disorder (PTSD) and a sense of threat due to the pandemic were assessed using the following screening tests: IES-R by Weiss and Marmar, PHQ-9 by Spitzer et al., and a self-constructed sliding scale for assessing COVID-19 anxiety. The study was carried out on a group of 57 firefighting cadets, hospitalized in a COVID-19 isolation room (Mage = 23.01), staying in isolation due to SARS-CoV-2 virus infection and a control group of 57 healthy men (Mage = 41.38) staying at home during quarantine and national lockdown. COVID-19 pandemic causes many psychopathological reactions. The predictive models revealed that the predictors of symptoms of PTSD in isolated patients included depression and the experienced sense of COVID-19 threat resulting from the disease, while in the control group the symptoms of depression were the only predictor of PTSD. PTSD experiences are usually associated with depression. It may also be a form of the re-experiencing process or the effect of high affectivity, indirectly confirmed by the participation of hyperarousal in the feedback loop. Our findings highlight the importance of mental health aspects in patients treated during the COVID-19 pandemic. The COVID-19 pandemic requires social distancing, quarantine and isolation, which may cause psychopathological symptoms not only in affected people, but also in the general population. Moreover, the need for greater psychological support can be emphasized for both: the sick and the general population.

Hidradenitis suppurativa - biologic therapy and other available treatment options.

Hidradenitis suppurativa (HS) is a chronic recurrent inflammatory skin disease which is characterized by painful, recurrent nodules and abscesses. The overall prevalence of HS is estimated to be 11 per 100 000 individuals in the United States and 4% of the world's population. Women are three times more affected than men, especially patients between 18 and 29 years. Similarly to acne vulgaris, HS is primarily associated with follicular occlusion, which results from a number of biological processes, including follicular epithelial hyperplasia and hyperkeratinization. There are numerous available treatment options for cutaneous lesions in the course of HS. A combination of conservative therapy and appropriate surgical treatment conducted by an experienced surgeon ensures the best possible clinical outcomes. Presently, biologic therapy is the most effective pharmacological treatment in patients with a moderate-to-severe course of the disease. Numerous ongoing clinical trials provide hope for greater availability of new biologic therapy methods.

Is biologic therapy the future of granuloma annulare treatment?

Granuloma annulare (GA) is a benign inflammatory skin disease that presents with erythematous papules and annular plaques. The pathogenesis of GA remains unknown and may potentially involve type 1 T helper cell (Th1)-mediated delayed-type hypersensitivity reaction. GA is associated with many conditions such as malignancy, trauma, thyroid disease, diabetes mellitus, and viral infection. The role of biological treatment is under investigation. In this article, we present a case of GA development following systemic treatment of psoriatic arthritis in a 59-year-old patient; golimumab therapy resulted in the successful treatment of both psoriatic arthritis and GA. This is the first case report describing GA therapy using golimumab.

Comparative effectiveness of adalimumab versus ustekinumab in the treatment of severe chronic plaque psoriasis: The results based on data from the program "treatment of moderate and severe forms of plaque psoriasis (B.47)" of the National Health Fund in Poland.

Biological agents: TNF-α inhibitors, IL-12, and IL-23 blockers, IL-17 inhibitors are used in the treatment of plaque psoriasis. Adalimumab (ADA) is an antibody that binds to TNF-α. Ustekinumab (UST) blocks IL-12 and IL-23. The data obtained from medical records is of exceptional value. The aim of the study was to evaluate the efficacy of ADA and UST during a single 40-week period of biological treatment of patients under the drug program "Treatment of moderate and severe form of plaque psoriasis." The group of 620 adult patients with moderate to severe form of plaque psoriasis, who were unresponsive or had contraindications to the standard treatment were qualified to the drug program. In the evaluated group, 50.64% patients were treated with UST, 49.36% with ADA. The efficacy of treatment was assessed during weeks 0, 4, 16, 28, and 40. At week 16th, PASI75 reached 80.72% patients in ADA treated group, PASI ≥90 54.88%, PASI100 19.6% of patients. In the UST group (week 16th) PASI75 reached 70.38%, PASI90 44.26%, PASI100 15.6% of patients. At week 28th PASI90 and PASI100 were more pronounced in the ADA group than in UST. In addition, the total percentage of PASI improvement was significantly higher in the ADA group (p = 0.0006). The percentage of PASI improvement in week 40 was statistically higher in ADA group compared to UST (p = 0.015). Compared to UST, ADA was clinically more effective during a 40-week observation. Patients receiving ADA achieved PASI75, PASI90, and PASI100 more frequently and faster than those treated with UST. Additionally, ADA improved the quality of life of psoriatic patients more substantially compared to UST.

Effect of Vitamin D Concentration on Course of COVID-19.

BACKGROUND The course of COVID-19 disease is associated with immune deregulation and excessive release of pro-inflammatory cytokines. Vitamin D has an immunomodulatory effect. We aimed to assess the possible correlation between the incidence and severity of SARS-CoV-2 infection and serum vitamin D concentration. MATERIAL AND METHODS A total of 505 successive patients admitted to a COVID-19-dedicated hospital were included in the retrospective analysis. Serum 25-hydroxyvitamin D (25-OHD) levels and SARS-CoV-2 RT-PCR throat swab test results were determined for each patient. The course of COVID-19 was assessed on the basis of the serum Vitamin Modified Early Warning Score (MEWS), which includes respiratory rate, systolic blood pressure, heart rate, temperature, and state of consciousness), as well as number of days spent in the intensive care unit (ICU) and need for oxygen therapy. RESULTS There was no difference in 25-OHD concentration between COVID-19-confirmed and negative results of the PCR tests. No correlation was found between serum 25-OHD in the COVID(+) group and the need for and time spend in the ICU, as well as the MEWS score. Multivariate analyses showed a positive correlation between need for oxygen therapy and lower 25-OHD concentration, as well as older age (P<0.001) and similar positive correlation between need for ventilation therapy with lower 25-OHD concentration, as well as older age (P=0.005). CONCLUSIONS Our findings do not support a potential link between vitamin D concentrations and the incidence of COVID-19, but low vitamin D serum level in COVID-19 patients might worsen the course of the disease and increase the need for oxygen supplementation or ventilation therapy.

Dermatological manifestations of the Coronavirus disease 2019 in children: a systemic review.

Introduction: An increasing number of publications describing dermatoses associated with Coronavirus disease 2019 (COVID-19) have shown differences in the morphology and incidence of dermatoses in children compared to the general population. Material and methods: Among 22 selected full-text articles, dermatoses associated with COVID-19 have been described in 196 children (average age: 12.57 years). Dermatoses were the only symptom in most patients (n = 134; 68.4%). RT-PCR tests of nasopharyngeal swabs confirmed SARS-CoV-2 infection in only 18 cases (n = 18/69; 26.1%). Results: Dermatoses described include chilblain-like lesions (n = 173; 88.3%), maculopapular rash (n = 16; 8.2%), erythema multiforme-like lesions (n = 12; 6.1%), varicella-like exanthema (n = 1; 0.5%), and urticaria (n = 1; 0.5%). Conclusions: This publication aims to summarise existing data on dermatoses and to draw attention to how identification of dermatological symptoms in paediatric patients can provide a quick and reliable indicator of COVID-19.

Elastography as a possible useful method of assessment of skin involvement in systemic sclerosis.

Introduction: Scleroderma (Sc) is a connective tissue disorder associated with internal organ involvement, increased mortality, and unknown pathogenesis. It has been found that the more extensive the skin involvement the more severe internal organ manifestations and increased disability. The Rodnan skin score (RSS) is one of the established methods to examine skin thickness among patients with Sc. Due to RSS limitations, for instance, lack of detection of subclinical changes, a new tool is needed for the evaluation of Sc. In recent studies, shear wave elastography (SWE) has been examined as a potential tool to assess skin involvement through the evaluation of skin strain. Aim: To verify whether elastography is a reliable method to examine Sc progression and possibly provide one useful site to perform the examination - as an easy, cheap, and reliable examination tool. Material and methods: Forty Sc patients were examined, and 28 healthy individuals were recruited for the control group. Among the patients and control group, skin thickness was assessed using the RSS and skin strain measurements using elastography in 20 body locations. Results: SWE in the right-hand finger can be treated as an important diagnostic indicator of the severity of Sc. Conclusions: SWE is a reliable method for evaluating skin involvement among patients with systemic sclerosis (SSc). Right finger measurements correlate positively with Rodnan's results and can be a predictor of the severity of SSc. This study found SWE to be a reliable method for examining SSc progression and possibly one useful site for the examination.

Skin manifestations of neuroendocrine neoplasms: review of the literature.

Neuroendocrine neoplasms (NENs) are a heterogeneous group of rare tumours derived from peptidergic neurons and specialized neuroendocrine cells capable of secreting various peptides or amines. These cells may be present in endocrine tissue or diffused in the tissues of the digestive or respiratory system. The article reviews the characteristic features of NENs, with particular emphasis on skin manifestations, such as necrolytic migratory erythema (NME), tongue inflammation, angular cheilitis, venous thrombosis and alopecia in glucagonoma; "flushing", "lion face", pellagra skin symptoms, "scleroderma-like features without Raynaud's phenomenon" in carcinoid tumours. The paper also presents the clinical picture of the neuroendocrine tumour of the skin - Merkel cell carcinoma. The aim of this study was to draw attention to the need for precise and comprehensive diagnosis of the patients, with particular emphasis on skin lesions as a revelator of neuroendocrine tumours. This management allows for the early implementation of appropriate treatment.

Effectiveness of infliximab biosimilars in the treatment of moderate to severe chronic plaque psoriasis: experience of real-world data from the register of the program "Treatment of moderate and severe forms of plaque psoriasis (B.47)" of the National Health Fund in Poland.

Introduction: Infliximab (IFX) is a monoclonal antibody that binds to and neutralizes TNF-α. IFX (Remicade) was approved by the U.S. Food and Drug Administration in 2006 for the treatment of severe plaque psoriasis. In 2013 two infliximab biosimilars: Remsima and Inflectra were also registered. The introduction of biosimilar drugs is associated with a significant reduction in treatment costs. Aim: To evaluate the efficacy of treatment with biosimilar IFX with non-medical switch option in patients with plaque psoriasis under the drug program "Treatment of moderate and severe plaque psoriasis" of the Ministry of Health in Poland. Material and methods: The group of 91 adult patients with moderate to severe plaque psoriasis, unresponsive or with contraindications to the standard treatment were qualified to the drug program (in 2016-2018). Efficacy of treatment with biosimilar IFX was evaluated using the Psoriasis Area and Severity Index, body surface area and Dermatology Life Quality Index scoring performed at week 0, 14, 46 and 94. Results: The mean change in PASI, DLQI, and BSA scores at week 14 was 89.92%, 93.75% and 90.91%, respectively. By week 14, 83.52% of patients achieved PASI75, 49.45% PASI ≥ 90 and 26.37% PASI100. At week 46, 84.62% of patients achieved PASI75, 54.95% PASI ≥ 90, and 21.98% PASI100. At week 94 of therapy, 80.22% of patients achieved PASI75, 48.35% PASI ≥ 90, and 18.68% PASI100. At week 94 of therapy, PASI100 was maintained by 37.5% of patients who achieved PASI100 at week 14. Conclusions: 94-week therapy with biosimilar infliximab results in high and sustained clinical efficacy in patients with moderate to severe psoriasis.

Real-world evidence on time to relapse of plaque psoriasis after discontinuation of biologic treatment in Poland.

The aim of this study is analysis of time to relapse after discontinuation of biologic treatment and identification of factors associated with risk of relapse. The analysis used real-world data of 705 patients treated with biologic drugs (adalimumab [ADA], ustekinumab, infliximab, and etanercept) in Poland in 2013-2019. Time to relapse was analyzed by Kaplan-Meier estimator. Data was stratified by the number of prior relapses. Determinants of risk to relapse were analyzed with Prentice-Williams-Peterson model. Kaplan-Meier estimate of time to the first relapse was 276 days, to the second relapse was 246 days, to the third relapse was 218 days, and to the fourth relapse was 178 days. In multidimensional analysis statistically significant variables affecting risk of relapse were the following: biologic naivety (hazard ratio [HR] 0.707), ADA (HR 0.787), psoriasis area and severity index at the last follow-up visit (HR 1.049), abnormal hemoglobin level (HR 0.794), and abnormal lymphocyte counts (HR 1.278). The findings of this study suggest that periods to relapse after discontinuation of biologic drugs become shorter with the number of prior relapses experienced by the patient. Ninety-five percentage of observed relapses occurred within 613 days of the end of the first treatment cycle, within 478 of the second cycle and within 351 days of the third cycle.

Features of classic lichen planopilaris and frontal fibrosing alopecia in reflectance confocal microscopy: A preliminary study.

BACKGROUND: Classic lichen planopilaris (LPP) and frontal fibrosing alopecia (FFA) are primary lymphocytic cicatricial alopecia. In patients with ambiguous clinical presentation, reflectance confocal microscopy (RCM) a new noninvasive skin imaging technique, could be a helpful diagnostic tool. The aim of our study was to describe the characteristic features of classic LPP and FFA using RCM. MATERIALS AND METHODS: Ten patients with classic lichen planopilaris and two with frontal fibrosing alopecia were examined with RCM. RESULTS: Lichenoid inflammatory infiltrate around the hair follicle was observed in three cases of classic LPP and FFA (3/12; 25.0%). Extensive perifollicular fibrosis was seen in nine patients (9/12; 75.0%) with classic LPP and FFA. An increased number of white, ill-defined, coarse dermal fibers at the level of the superficial dermis were visible in seven cases (7/12; 58.3%). Moreover, dilated blood vessels were present in seven patients with classic LPP and FFA (7/12; 58.3%). CONCLUSION: Summing up, reflectance confocal microscopy allows to visualize major key diagnostic features of classic lichen planopilaris and frontal fibrosing alopecia in the real time. The value of RCM examination in scarring alopecia needs to be further evaluated, but it appears to be a useful adjuvant tool for the initial diagnosis of classic LPP and FFA.

High-resolution ultrasound imaging of skin involvement in systemic sclerosis: a systematic review.

To collect evidence on the application of ultrasound in skin assessment in patients with systemic sclerosis (SSc). The authors carried out a review of the literature via Pubmed MEDLINE database. The search terms were: skin imaging in systemic sclerosis, ultrasound skin imaging in patients with systemic sclerosis. The selection and analysis of articles were performed by two independent evaluators. The authors analyzed 10 studies characterizing 470 patients with systemic sclerosis. The patients were young adults, mainly women. The described methods of ultrasound were: ultrasound elastography (7.14%), ultra-high-frequency (7.14%) and B-mode ultrasonographic imaging (21.43%), high-frequency ultrasonography (21.43%), shear-wave elastography (21.43%) and others (21.43%). Skin measurements reported in the analyzed studies were: skin ultrasound in all studies, skin thickness (8 studies), skin elasticity (5 studies), skin stiffness (2 studies), subcutaneous tissue thickness (1 study). Ultrasound measurements were compared to different types of scales and measurements used in the description of disease progression. Ultrasound may be used in the clinical assessment of skin involvement in SSc. To the best of our knowledge, articles currently reporting the use of ultrasound in skin imaging show interesting ideas and provide basis for further research. Skin involvement in SSc assessed with ultrasound should be compared to skin biopsy. It is necessary to develop guidance for conducting skin measurements using ultrasound in patients with scleroderma. Currently, skin imaging in SSc is of limited clinical use due to a variety of methods and the lack of a standard operating procedure. The authors of analyzed studies suggested that high-frequency ultrasound provided a quantitative and reliable evaluation of dermal thickness in patients with SSc.

The Role of Epigenetic Factors in Psoriasis.

Psoriasis is a chronic, systemic, immune-mediated disease with an incidence of approximately 2%. The pathogenesis of the disease is complex and not yet fully understood. Genetic factors play a significant role in the pathogenesis of the disease. In predisposed individuals, multiple trigger factors may contribute to disease onset and exacerbations of symptoms. Environmental factors (stress, infections, certain medications, nicotinism, alcohol, obesity) play a significant role in the pathogenesis of psoriasis. In addition, epigenetic mechanisms are considered result in modulation of individual gene expression and an increased likelihood of the disease. Studies highlight the significant role of epigenetic factors in the etiology and pathogenesis of psoriasis. Epigenetic mechanisms in psoriasis include DNA methylation, histone modifications and non-coding RNAs. Epigenetic mechanisms induce gene expression changes under the influence of chemical modifications of DNA and histones, which alter chromatin structure and activate transcription factors of selected genes, thus leading to translation of new mRNA without affecting the DNA sequence. Epigenetic factors can regulate gene expression at the transcriptional (via histone modification, DNA methylation) and posttranscriptional levels (via microRNAs and long non-coding RNAs). This study aims to present and discuss the different epigenetic mechanisms in psoriasis based on a review of the available literature.

Cutaneous manifestations of coronavirus disease 2019 - review of literature and case reports.

Primary clinical manifestations of coronavirus disease are respiratory tract infections, ranging from an asymptomatic carrier state to severe interstitial pneumonia. The most common symptoms are fever, dry cough, fatigue, nausea, diarrhea, and loss of smell or taste. Recent reports indicate the possibility of several nonspecific dermatological symptoms. These include urticaria, maculopapular lesions, vascular lesions involving petechiae, purpura and livedo reticularis. The onset of cutaneous lesions may precede full-blown COVID-19 or remain the only manifestation of the disease (especially in young patients). Focusing on dermatological symptoms may be crucial for preventing the spread of SARS-CoV-2. However, unambiguous assessment of cutaneous manifestations of COVID-19 requires more research and case studies conducted by dermatologists.

Familial localized scleroderma with paediatric onset: a review.

Localized scleroderma is an inflammatory disease causing sclerosis of the skin. The aetiology and pathogenesis of localized scleroderma remain unclear. Localized scleroderma is considered a genetically driven disease. It is not well understood if genetic factors or environmental exposure individually can cause its development or if their interaction is needed to trigger the disease. Some authors postulate that familial clustering is evidence of a hereditary disease. Familial localized scleroderma has been rarely reported and is a case worth studying. We present the review of literature on this subject with 3 additional cases of familial localized scleroderma with paediatric onset.