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Abstract Introduction Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are life-threatening acute mucocutaneous disorders usually triggered by drugs. In this study, we aimed to evaluate the factors affecting mortality in patients with SJS-TEN. Methods Our study is a retrospective cohort study, analyzing data collected from a total of 12 tertiary care centers between April 2012 and April 2022. Results The study included 59 males and 107 females, a total of 166 patients, with an average age of 50.91 ± 21.25 years. Disease classification was TEN in 50% of cases, SJS in 33.1%, and SJS-TEN overlap in 16.9%. The average SCORTEN within the first 24 h was 2.44 ± 1.42. Supportive care was provided to 99.4% of patients. The most commonly used systemic immunomodulatory treatments were systemic steroids (84.3%), IVIG (intravenous immunoglobulin) (49.3%), and cyclosporine (38.6%). Plasmapheresis was administered to five patients. While 66.3% of patients were discharged, 24.1% resulted in exitus. Our comparative analysis of survivors and deceased patients found no effect of systemic steroids, IVIG, and cyclosporine treatments on mortality. Univariate analysis revealed that the SCORTEN scores on days 1 and 3 as well as the rates of detachment at the onset and during follow-up were significantly higher in deceased patients compared to survivors. The rates of fever, positive blood cultures, and systemic antibiotic use were higher in deceased patients compared to survivors. The presence of comorbidities, diabetes, and malignancy were significantly more common in deceased patients. Multivariate regression analysis indicated that over SCORTEN 2, the mortality risk exponentially rose with each SCORTEN increment, culminating in an 84-fold increase in mortality at SCORTEN 5–6 (odds ratio [95% confidence interval]: 13.902–507.537, p

Children who have lymphatic malformations frequently experience functional limitations and aesthetic abnormalities that have a significant impact on their quality of life and may pose a threat to their lives. Conventional treatments such as surgery or sclerotherapy are rarely curative, demonstrating the great need for new treatment modalities. Recently, oral or topical administration of sirolimus has successfully treated lymphatic malformations. We report the 6-month treatment outcome of a 7-year-old boy with lesions consistent with lymphangioma circumscriptum on the left side of the neck since birth who was successfully treated with topical sirolimus.

Background and Design: The onset chronology of disease symptoms in patients with hidradenitis suppurativa (HS) and their gender differences have not yet been fully elucidated. We aimed to define the onset chronology of the disease symptoms and the socio-demographic and clinical characteristics. Materials and Methods: This single-center case series study included 100 consecutive patients. The disease symptoms for each patient were recorded in the time order of manifestations. Results: HS was more common in males (72%); 70% of the patients were overweight or obese. Smoking and alcohol consumption was significantly higher in males. The axilla was the most common onset area, followed by the inguinal and gluteal regions. The incidence of disease onset in the axilla in male and disease onset in the gluteus in female patients was significantly higher. Also, involvement of the pubis, perianal region, neck, back, and behind the ear was more common in males. The disease followed a more severe course in patients with the involvement of axilla, perianal, and inframammary areas (p

Background and Design: The human papillomavirus (HPV) is a non-enveloped DNA virus infecting skin and mucosal surfaces and causes one of the most common sexually transmitted infections worldwide. Thus, physicians must know about HPV infections, associated cancers, and immunization for public health. This study aimed to determine the knowledge of medical faculty students about HPV infections, related cancers, and vaccines and examine related variables. Materials and Methods: This descriptive study included all students in the first to fifth grades of the faculty of medicine. Data were collected with a two-part questionnaire consisting of 37 questions. Questionnaires were filled voluntarily. Data were analyzed with SPSS version 18.0. Results: The study included 250 medical students, and 52.4% (n=131) were men. While 95.6% (n=239) stated that they were aware of HPV, only 39.2% thought that they had sufficient knowledge. The most important sources of information were lectures and the Internet. Only 3.6% (n=9) of the students had been vaccinated against HPV. The most important reason (58%) for not being vaccinated was not having heard of the HPV vaccine before or not knowing someone who had it, and the other important reason was economics. Nearly 80% of the students thought that HPV vaccines should be included in routine vaccination. While 60% of the students stated that they would get an HPV vaccine if a free vaccine is provided, the lack of sufficient information was cited as the most important reason (45%) against the widespread use. Conclusion: The knowledge of medical school students about HPV infection and vaccines was not very sufficient, and the vaccination rate was low. Our results suggest that HPV-related courses in medical education and reimbursement of vaccines by health authorities require some adjustments in the core education curriculum and national health policies.

Morphea is an inflammatory immune-mediated disease associated with many factors such as genetic predisposition, environmental triggers such as bacterial and viral infections, as well as intrinsic factors such as hormonal and immunological dysregulation, although its pathogenesis is largely unknown. Here, we report the first case of morphea relapse after the mRNA coronavirus disease-2019 (COVID-19) vaccine. Our case along with recent reports suggest that new associations might appear due to COVID-19 infection and vaccines.

Dermatofibromas are common skin tumors although there are some different clinical forms, which are seen rarely. Giant dermatofibromas are benign, pedunculated lesions that are larger than 5 cm with typical dermatofibroma histopathologic features. Typically, plaque-like dermatofibromas are considered a subtype of giant dermatofibromas without a pedicule. Here, we aimed to report a case of plaque-like dermatofibroma seen in a 5-year-old girl due to its rarity and to review this clinical picture together with all the cases in the literature.

Background Rituximab appears to be effective for treating pemphigus, although there are limited long-term data. Methods This retrospective single-center study evaluated patients with conventional treatment-resistant pemphigus who received rituximab during September 2010–December 2019. The first rituximab cycle was based on the rheumatoid arthritis protocol in all patients except one patient, and additional single doses (500 mg or 1000 mg) were administered after clinical and/or serological relapse. The consensus definitions were used for complete remission off therapy, complete remission on minimal therapy, and clinical relapse. Serological relapse was defined as a progressive ≥2-fold increase in anti-desmoglein titers (vs. previous the measurement). Results The study included 52 patients with pemphigus vulgaris and 1 patient with pemphigus foliaceus. The mean number of infusions was 5 and the average follow-up after the first infusion was 56 months. The average time to clinical and/or serological relapse was 12 months. Complete remission was achieved in 84.9% of patients, including after the first rituximab cycle in 25 patients (47.1%). Two patients died during the follow-up period. Conclusion Additional rituximab cycles may help achieve and prolong remission in patients with moderate-to-severe pemphigus resistant to conventional therapies. However, prospective trials are needed to identify the optimal dosing protocol.

Coronavirus disease-2019 (COVID-19) is a serious cause of respiratory tract infection, and its severe course has been associated with some risk factors, including malignancies and immunosuppressive treatments. Primary cutaneous lymphomas (PCL) are a heterogeneous group of immune system neoplasms, which are subclassified as indolent and aggressive types according to their survival rates. PCL treatment ranges from skin-based therapies to systemic treatments, of which immunosuppressive effects occur in some. During the COVID-19 pandemic, patients with PCL should be protected from possible COVID-19 complications, and the optimal treatment should be provided to control the disease taking into account the treatment-related risks. Therefore, recommendations about the management of patients with PCL during the COVID-19 pandemic were overviewed in light of the literature. Topical treatments can generally be considered low-risk therapies and can be continued without interruption. Phototherapy, skin radiotherapy, and total skin electron beam therapy increase the risk of COVID-19 exposure due to hospital visits. Moderate-risk therapies like interferons, systemic retinoids, methotrexate, and systemic corticosteroids might be used with caution. Advanced-stage patients with COVID-19 related comorbidity and who previously received immunosuppressive therapy should be carefully evaluated. Biological agents and systemic chemotherapeutics, which are considered high-risk, should not be delayed when needed. However, increasing intervals between treatments or switching to alternative therapies may be preferable in stable diseases. Most importantly, all patients with PCL should be ensured to comply with general protection measures as long as the pandemic continues.

Background and Design: Atopic dermatitis (AD) is a common chronic, recurrent, and itchy inflammatory skin disease. Various therapeutic agents are available, but severe side effects may limit their usage. Recently, dupilumab, a human monoclonal antibody that targets the interleukin-4 (IL-4) receptor alpha subunit of heterodimeric IL-4 and IL-13 receptors, is approved and might be used in patients with resistant AD, with the permission of the Ministry of Health. Materials and Methods: This study aimed to retrospectively evaluate the clinical characteristics of patients and dupilumab treatment responses in our center. This study included patients with AD who were unresponsive to conventional treatments and treated with dupilumab. Sociodemographic, laboratory data, previous treatments, and responses along with disease severity scores [eczema area and severity index (EASI)] before and after dupilumab were evaluated through the electronic files of patients. Results: A total of 21 patients (13 males and 8 females) between June 2019 and March 2021 were identified. The mean age of patients was 40.57±15.21 years. The mean duration of dupilumab treatment was 6.59±5.88 months. The mean EASI score at the beginning of dupilumab was 15.35±8.03, whereas 4.6±2.9 after treatment. A 70-100% improvement was found in approximately 75% of the patients. No side effects were observed that required treatment discontinuation or dose changes. Conclusion: Our study has the highest number of reported patients in our country, which revealed that dupilumab is highly effective and safe for conventional treatment-resistant AD.

Omalizumab is a humanized monoclonal antibody which is an FDA-approved treatment of severe allergic asthma and inhibits IgE binding to FcεRI. According to increasing evidence of IgE inhibition, omalizumab was suggested as a therapeutic approach for bullous pemphigoid (BP). Rituximab has been reported to be effective in various autoimmune diseases, including autoimmune bullous dermatoses. A specific protocol for the use of rituximab to treat BP patients is not yet available. There are only small case series and case reports about the efficacy and safety of rituximab in BP. Here we present a young BP patient who responded well to rituximab therapy and was refractory to conventional and omalizumab therapies although he had elevated IgE levels and eosinophilia. Our case supports the knowledge about the effectiveness and safety of rituximab not only in pemphigus but also in BP. On the other hand, although it did not work in our case, omalizumab may be a potentially effective agent in some carefully selected patients with certain subtypes of BP.

Lymphomatoid papulosis (LyP) is a benign papulonodular skin eruption with histologic features of malignant lymphoma. A new variant of LyP which was termed “type E” was recently described with similar clinical and histological features to angiocentric and angiodestructive T-cell lymphoma. LyP type E is characterized with recurrent papulonodular lesions which rapidly turn into hemorrhagic necrotic ulcers and spontaneous regression by leaving a scar. None of the available treatment modalities affects the natural course of LyP. For therapy various modalities have been used such as topical and systemic steroids, PUVA, methotrexate, bexarotene, and IFN alfa-2b. Here we present a severe and devastating case with a very rare variant of LyP type E, which is, to our knowledge, the first case successfully treated with IFN alfa-2a. Now disease has been maintaining its remission status for six months.

Background and Design: Drug reaction with eosinophilia and systemic symptoms (DRESS) is an uncommon, but potentially fatal, adverse drug reaction. Despite the alarming statistics regarding morbidity, mortality, and hospitalizations, epidemiological data on DRESS are insufficient. In this investigation, we sought to determine the etiology, clinicopathological characteristics, and prognosis of DRESS cases at our institution. Materials and Methods: In this retrospective, single-center study, 23 patients with DRESS examined between January 2014 and September 2020 were included according to the European Registry of Severe Cutaneous Adverse Reactions (RegiSCAR) scoring system. Patients were examined between January 2014 and September 2020. Descriptive statistics, Shapiro-Wilk test, Kolmogorov-Smirnov test, Mann-Whitney U test, Pearson chi-square test, and Fisher's exact test were performed. Results: The most frequently detected culprit drug category was anticonvulsants. Maculopapular eruption (100%) and elevated liver function tests (82%) were the most prevalent cutaneous and laboratory findings, respectively. Patients with elevated liver enzymes were more likely to exhibit facial erythema/edema and lymph node enlargement than those without (p=0.021 and p=0.103, respectively). The predominant pathological features were sparse vacuolization of the dermal-epidermal junction and superficial perivascular lymphohistiocytic inflammation with eosinophils. Two patients died during the period of follow-up, three patients were lost to follow-up, and eighteen patients recovered completely. Conclusion: Our research demonstrated that facial erythema/edema and lymph node enlargement are more prevalent in patients with elevated liver enzymes. Cyclosporine may be a treatment option in the fragile age group to prevent systemic corticosteroid complications. Early diagnosis and treatment that balances benefits and risks remain the most important determinants of prognosis

Background: Alopecia is a complication of autoimmune blistering diseases (AIBDs) that affects patients’ quality of life; however, it has generally been overlooked in patients with severe disease because it is regarded as a cosmetic issue. Objective: To study the epidemiologic data and clinical presentations of alopecia in our cohort of patients with AIBDs. Methods: Forty-one patients with AIBDs were assessed in this cross-sectional study. An assessment tool to collate patient information, including AIBD scalp involvement, trichoscopic findings, and Severity of Alopecia Tool II scores, was used. Results: More than 70% of patients in our cohort had at least 1 type of alopecia, with 10% presenting with a nonspecific (end-stage) scarring alopecia. Elevated Dsg1 ratios were predictive of hair loss in pemphigus vulgaris (P

Background: Glucocorticoids are the mainstay of treatment for autoimmune blistering diseases (AIBDs). The Glucocorticoid Toxicity Index (GTI) is a novel, outcome-based glucocorticoid-induced adverse effects monitoring instrument. Objective: To investigate whether the GTI score was able to accurately quantify the glucocorticoid-induced toxicity in patients with AIBDs. Methods: The prospective cohort study included patients with confirmed diagnoses of AIBDs (group1, currently receiving glucocorticoids; and group 2, had glucocorticoids ceased earlier). Data were collected minimally at baseline (V1) and 3 months (V2). Further data from patients who were able to complete the follow-up visits at 6 months (V3) and 12 months (V4) amid the COVID-19 pandemic were also included. GTI scores were calculated after data collection. Results: Analysis of data from V1 and V2 found a linear correlation between GTI score and prednisone doses (P

Livedoid vasculopathy (LV) is a rare, chronic, and occlusive disease of the veins supplying the upper parts of the skin. The pathogenesis of the disease is not precisely understood, and its attacks are often unpredictable but tend to worsen during the summer. LV affects women more often. This increased risk for LV in women might be related to sex-specific physiological conditions, such as pregnancy, or a higher incidence of LV-associated conditions, such as connective tissue diseases, hypercoagulable states, and venous stasis in women. The typical clinical appearance of LV consists of three main findings: livedo racemose, atrophie blanche, and skin ulcers. The purpose of this comprehensive review was to analyze LV in all aspects and mainly focus on early diagnosis for successful clinical management with a holistic and multidisciplinary approach. A detailed history, dermatological examination, and laboratory testing are essential for a diagnosis of LV. When LV is clinically suspected, a skin biopsy should be taken to confirm the diagnosis. Another critical step is to investigate the underlying associated conditions, such as connective tissue diseases, hypercoagulable states, thrombophilia, and malignancy. Unfortunately, no associated conditions can be detected in approximately 20% of all cases (idiopathic LV) despite all efforts. The diagnosis of the disease is delayed in most patients. Thus, irreversible, permanent scars appear. Early and appropriate treatment reduces pain and prevents the development of scars and other complications. Antiplatelet drugs and anticoagulants can be preferred as the first-line treatments along with general supportive measures. Other therapeutic options might be considered in unresponsive cases. Preference for refractory cases is based on availability, clinical experience, and patient-related factors (comorbidities, age, sex, and compliance). These include anabolic steroids, intravenous immunoglobulin, hyperbaric oxygen therapy, psoralen-ultraviolet A, vasodilators, fibrinolytics, immunomodulators, and immunosuppressives.

Leishmaniasis is one of eight neglected tropical diseases currently endemic in 102 countries/areas around the world. In recent years, cutaneous leishmaniasis (CL) has been increasingly observed among migrants, travelers, ecotourists, and military personnel. Because of its great capacity to mimic other dermatoses, CL is one of the great imitators and can mislead practitioners, which can result in untreated lesions that cause scars. CL is a disfiguring disease, especially for women, and often leaves scars on visible body sites, causing psychological, social, and economic problems. CS is a challenge, especially in nonendemic regions, such as Australia, because experience with diagnosis and management of the disease is limited. Keywords: Cutaneous leishmaniasis, neglected diseases, disfiguring diseases, stigma, women's health

Bullous pemphigoid is an autoimmune subepithelial disease characterised by pruritus followed by urticarial plaques and finally bullae on the skin and mucosa. Drug-associated bullous pemphigoid (DABP) is a term used to describe instances of bullous pemphigoid demonstrating clinical, histological, or immunopathological features identical or similar to those of the idiopathic form of bullous pemphigoid, associated with the systemic ingestion, or topical application of particular drugs. In this study, we conducted a comprehensive search of the literature according to PRISMA guidelines and a total of 170 publications were included in the final qualitative analysis. In conclusion, 89 drugs were implicated in DABP. The strongest evidence for DABP is seen with gliptins, PD-1/PD-L1 inhibitors, loop diuretics, penicillin and derivatives. An appreciation of the medications associated with bullous pemphigoid enables clinicians to identify potential cases of DABP earlier and cease the offending medication.

A 69-year-old woman affected by multiple sclerosis for 35 years was diagnosed with bullous pemphigoid (BP) and treated successfully with dimethyl fumarate (DMF) at a dose of 120 mg twice per day for 7 days and then increased to 240 mg twice per day after first-line therapies of BP. DMF is now under evaluation with an investigator-initiated prospective controlled trial in patients with BP to determine the efficacy and safety of adjuvant DMF. To our knowledge, this is the first case of BP successfully treated with DMF in the literature. Keywords: Bullous pemphigoid, dimethyl fumarate, multiple sclerosis, therapy