Validation of the Behçet's Syndrome Overall Damage Index (BODI) for Retrospective Studies and a Proposal for Modification.

OBJECTIVE: Assessment of damage accrual over time is important for evaluating and comparing long-term results of treatment modalities and strategies. Retrospective studies may be useful for assessing long-term damage, especially in rare diseases. We aimed to validate Behçet's Syndrome Overall Damage Index (BODI) for use in retrospective studies by evaluating its construct validity, reliability, and feasibility in retrospectively collected data. Additionally, we aimed to determine missing items by evaluating Behçet's syndrome patients with different types of organ involvement and long-term follow-up. METHODS: We included 300 patients who had at least 2 clinic visits at 1-year intervals. The construct validity for use in retrospective trials was assessed by comparing BODI scores calculated from patient charts and during face-to-face visits. BODI was additionally scored using retrospective chart data by 2 different observers and by the same observer six months apart, in a blinded manner. The time for filling BODI was evaluated to assess feasibility. Additionally, damaged items that were missing from BODI were identified. RESULTS: There was a good correlation between the retrospective and face-to-face evaluation of BODI (ICC 0.99; %95 CI 0.99-0.99). Inter-observer and intra-observer agreement were good (ICC 0.96 and 1, respectively). The main damage items that BODI did not capture were hypertension, liver failure, lung parenchymal involvement, glaucoma, and lymphedema. CONCLUSION: BODI seems to be a reliable and feasible instrument for assessing damage in retrospective studies. Modifying BODI using the additional damage items identified in this study may make it an even better scale.

De novo manifestations during adalimumab treatment in Behçet's syndrome.

OBJECTIVES: Treatment response may be variable across organ manifestations of Behçet syndrome (BS). We aimed to determine the frequency of de novo manifestations during adalimumab treatment. METHODS: We conducted a chart review of all BS patients who received adalimumab in our center between 2008 and 2023. Demographic data, reasons for initiating adalimumab, concurrent medications, previous treatments, and outcomes were recorded. We defined de novo manifestations as new BS manifestations that occurred for the first time during treatment with adalimumab. For patients with vascular involvement, a new vascular event at another vessel was also considered as a de novo manifestation. RESULTS: Among the 335 patients, a de novo manifestation developed in 14 (4%) patients. De novo manifestations were vascular involvement in 5 patients, arthritis in 3, anterior uveitis in 2, nervous system involvement in 2, gastrointestinal involvement in 1, and epididymitis in 1 patient. The primary reasons for adalimumab treatment were vascular involvement in 5 patients, uveitis in 4, arthritis in 3, mucocutaneous involvement in 1, and epididymitis in 1 patient. Upon the development of de novo manifestation, adalimumab was switched to another biologic in 4 patients, dose was intensified in 3, colchicine, conventional immunosuppressives, and/or glucocorticoids were added in 5, and topical eye drops were added in 2 patients, leading to remission of de novo manifestations in all patients. CONCLUSION: De novo manifestations were infrequent (4%) among BS patients treated with adalimumab. Of these, 57% were major organ involvement, mainly vascular involvement. None of the patients developed posterior uveitis.

Infliximab for vascular involvement in Behçet's syndrome.

OBJECTIVE: Vascular involvement is an important cause of morbidity and mortality in patients with Behçet's syndrome (BS). We aimed to survey the efficacy and safety of infliximab (IFX) in BS patients with vascular involvement followed in a dedicated tertiary center. METHODS: Charts of all BS patients who used IFX for vascular involvement between 2004 and 2022 were reviewed. Primary endpoint was remission at Month 6, defined as lack of new clinical symptoms and findings associated with vascular lesion, lack of worsening of the primary vascular lesion and a new vascular lesion on imaging, and CRP < 10 mg/L. Relapse was defined as development of a new vascular lesion or recurrence of the preexisting vascular lesion. RESULTS: Among the 127 patients (102 men, mean age at IFX initiation: 35.8 ± 9.0 years) treated with IFX, 110 (87%) had received IFX for remission induction and 87 of these (79%) were already on immunosuppressives when the vascular lesion requiring IFX developed. The remission rate was 73% (93/127) at Month 6 and 63% (80/127) at Month 12. Seventeen patients experienced relapses. Remission rates were better among patients with pulmonary artery involvement and venous thrombosis compared to patients with non-pulmonary artery involvement and venous ulcers. Fourteen patients had adverse events leading to IFX discontinuation and 4 had died due to lung adenocarcinoma, sepsis, and pulmonary hypertension-related right heart failure due to pulmonary artery thrombosis (n = 2). CONCLUSION: Infliximab seems to be effective in majority of BS patients with vascular involvement, even in those who are refractory to immunosuppressives and glucocorticoids.

Emergence of new manifestations during infliximab treatment in Behçet's syndrome.

OBJECTIVES: Infliximab (IFX) is increasingly being used for the treatment of severe manifestations of Behçet's syndrome (BS). However, emergence of new manifestations has also been occasionally reported during IFX treatment. We aimed to assess the frequency of new manifestations in our BS patients treated with IFX. METHODS: A chart review was conducted to identify all BS patients treated with IFX in our clinic between 2004 and 2020. Demographic data, indications for IFX initiation, concomitant treatments and outcomes were recorded. A new manifestation was defined as the emergence of a new organ involvement or mucocutaneous manifestation developing for the first time during IFX treatment or within 12 weeks after the last infusion of IFX. RESULTS: Among our 282 patients who used IFX, 19 (7%) patients had developed a total of 23 new manifestations during a mean follow-up of 20.0 (15.3) months. Patients with vascular involvement were more likely to develop a new manifestation (12/19, 63%). Initial manifestations that required IFX were in remission at the time of new manifestation in 14/19 patients. IFX treatment was intensified (n = 6) and/or glucocorticoids, immunosuppressives or colchicine was added to IFX (n = 21). IFX was switched to another agent for the remaining manifestations (n = 8). These treatment modifications led to remission in 17/19 patients. CONCLUSION: New manifestations developed during IFX treatment in 7% of our patients with BS. They could be managed by intensifying IFX treatment or adding other agents in the majority of these manifestations.

Can rituximab be used in the treatment of pemphigus vulgaris during the COVID-19 pandemic?

Rituximab is a monoclonal antibody that targets CD20, a B-lymphocyte antigen; that leads to a decline in the B-cell counts for at least a year. The patients who have received rituximab treatment in the previous 5 years with the diagnosis of pemphigus group of diseases at Cerrahpasa Medical Faculty were questioned for COVID-19 infection. A total of 48 patients were included in this study; only one male patient had COVID-19 infection which had a mild course. There is no significant difference in the total number of lymphocytes between patients who have received rituximab within the previous 5 years or last year. The number of lymphocytes is independent of the number of courses of rituximab treatment received. Therefore, we suggest that all pemphigus patients who have received rituximab treatment within the previous 5 years should be careful of the preventive measures against the COVID-19 infection irrespective of the number of treatment courses or the number of years which has passed since the treatment. The disease course was mild in the only infected patient. Thus, rituximab may be used in the treatment of pemphigus vulgaris during the COVID-19 pandemic if its use is necessary.

Swabs Containing Tea Tree Oil and Chamomile Oil Versus Baby Shampoo in Patients With Seborrheic Blepharitis: A Double-Blind Randomized Clinical Trial.

PURPOSE: The comparison of the efficacy of swabs containing tea tree oil and chamomile oil and baby shampoo (BS) in the treatment of seborrheic blepharitis was aimed. METHODS: This randomized, double-blind, parallel-group, active control, multicenter clinical trial included patients with seborrheic blepharitis using block randomization (BS, n=23; swabs, n=26). Patients were treated with BS or swabs for 8 weeks followed by 4 weeks of treatment withdrawal. Change in Blepharitis Symptom measure (BLISS), Demodex count, Ocular Surface Disease Index (OSDI) score, Schirmer test, tear breakup time (TBUT), noninvasive TBUT (NI-TBUT), corneal staining, and meibography at different visits (baseline, fourth, , and 12th week) were the main outcome measures. RESULTS: Patients in both groups showed similar baseline parameters (P>0.05). Patients using swabs showed significantly lower BLISS scores compared with patients using BS at the 4th, 8th, and 12th week visits (3.6±6.1 vs. 6.3±4.5 P=0.011; 1.1±2.8 vs. 6.6±6.7, P<0.001; 0.9±2.8 vs. 5.7±6.6, P=0.002, respectively). Patients using swabs showed improvement in OSDI scores after 8 weeks of treatment compared with the baseline visit (P<0.001). Despite a similar Demodex reduction effect in both treatment arms even after 4 weeks of treatment (P<0.001), both treatment modalities did not show any effect on the other parameters. CONCLUSION: Although both swabs and BS showed efficacy for the treatment of seborrheic blepharitis in terms of the Demodex reduction and symptomatic improvement, swabs may provide better symptomatic improvement. Four weeks of treatment discontinuation may not cause any recurrence in the symptoms or Demodex infestation.

[Clinical Characteristics of Mild-Moderate COVID-19 Patients and Risk Factors for the Development of Pneumonia]. / Hafif-Orta Klinik Seyirli COVID-19 Hastalarinin Genel Özellikleri ve Pnömoni Gelisimi için Risk Faktörler.

Limited data exists to date on the predictors for the development of pneumonia in patients with mild and moderate coronavirus (COVID-19). In this study, it was aimed to evaluate the demographic characteristics and clinical findings of mild and moderate COVID-19 and to determine the risk factors for the development of COVID-19 pneumonia in patients admitted to the pandemic outpatient clinic of a university hospital. A total of 414 patients with laboratory confirmed COVID-19 were included. Of these, 220 (53.1%) were male, the mean age was 38.3 ± 12.7. Median duration of hospital admission from the onset of symptoms was three days (0-11). Of the confirmed COVID-19 cases, 154 (37.2%) had a history of family contact and the most common symptoms were weakness (68.4%), myalgia (61.8%), headache (56.5%), loss of smell (45.2%), loss of taste (43.2%) and anorexia (42.8%). Among females, weakness (p= 0.016), headache (p= 0.008), sore throat (p= 0.032), nausea (p= 0.003), anorexia (p= 0.045), loss of taste (p= 0.005) and loss of smell (p<0.001) were more common. Loss of taste (47.6% vs. 25%, p<0.001) and loss of smell (50% vs. 26.3%, p<0.001) were more common in patients with under the age of 50 and cough (43.4% vs. 29.3%, p= 0.003) was more common in patients with above the age of 40. Among 46 (11.1%) patients with asymptomatic COVID-19, there was no significant difference (p= 0.500) between the genders. Pneumonia was detected in 150 (43.8%) of 339 patients who underwent thorax computed tomography. In the univariate analysis; advanced age (p<0.001, odds ratio (OR)= 1.44), obesity (p<0.001 OR= 2.5), not being actively smoking (p<0.001, OR= 6.19), fever at first admission (p= 0.002, OR= 2.02), cough (p<0.001, OR= 3.26), shortness of breath (p<0.001, OR= 23.37), weakness (p= 0.042, OR= 1.63), anorexia (p= 0.009, OR= 1.79) and elevation of D-dimer (p= 0.014, OR= 1.92) were associated with the development of pneumonia. In multivariate analysis, obesity (p= 0.005, OR= 2.69), not being actively smoking (p<0.001, OR= 5.43), cough at first admission p= 0.017, OR= 2.16) and shortness of breath (p= 0.008, OR= 16.22) was determined as an independent risk factor for the development of pneumonia. CRP (p<0.001), D-dimer (p<0.001), ferritin (p<0.001) values among 108 (26.1%) patients with a body-mass index(BMI) >30 were high, and 60.9% of the patients had pneumonia (p<0.001) . CRP (p<0.001), D-dimer (p= 0.010) values were low, lymphocyte count (p= 0.001) was high among 106 (25.6%) active smokers, and 15.6% of the patients had pneumonia (p<0.001). Of the patients reported with persistent symptoms, 25.9% had loss of smell, 25% had weakness, and 23.1% had loss of taste on the seventh day; 21.1% had loss of smell, 21.1% had myalgia, and 19.7% had loss of taste on the 14th day. During their follow-up, the COVID-19 polymerase chain reaction (PCR) test was studied in 286 patients for control purposes. The median time of being negative for COVID-19 PCR test was eight days (3-56). In conclusion, symptoms may last longer than 14 days in 20- 30% of patients presenting with mild-moderate clinical findings. In addition, obesity should be considered as an important risk factor for COVID-19 pneumonia.

A survey of bullous diseases in a Turkish university hospital: clinicoepidemiological characteristics and follow-up

Background/aim: Autoimmune bullous diseases, if left untreated, are life-threatening conditions affecting primarily skin and mucous membranes. These blistering disorders are characterized by epidermal or subepidermal detachment. Autoimmunity plays a key role in pathogenesis; therefore, immunosuppressive agents are the treatment of choice. The aim of this study is to document relative frequencies of different autoimmune bullous diseases, patient characteristics, treatment options, and side effects in patients presenting to our bullous skin disease center at Istanbul University, Cerrahpasa, Cerrahpasa Medical Faculty. Materials and methods: Medical files were examined retrospectively for all patients with autoimmune bullous diseases who were followed up between 2003 and 2019 at the Bullous Skin Disease Center at Istanbul University, Cerrahpasa. Results: A total of 346 patient files were examined. Pemphigus vulgaris was the most frequent autoimmune bullous disease, followed by bullous pemphigoid and pemphigus foliaceus, according to our study. There is a general female predominancy for all autoimmune bullous diseases. The most commonly preferred treatment options were high-dose daily corticosteroids. Conclusion: This retrospective study summarizes the patient characteristics, comorbidities, treatment choices, and side effects during 16 years of clinical practice.

Treatment of alopecia areata with 2940-nm fractional erbium:yttrium-aluminum-garnet laser.

Various laser therapies have been used for the treatment of alopecia areata (AA). Enhanced hair regrowth was reported with the use of ablative fractional 2940-nm erbium:yttrium-aluminum-garnet (Er:YAG) laser in a murine model. However, clinical effectiveness of fractional Er:YAG laser for hair disorders has not been investigated in human studies. The aim of our study was to assess the effectiveness of 2940-nm fractional Er:YAG laser in the treatment of chronic AA that is unresponsive to conventional therapies. Twenty-five AA patients underwent three sessions, each 4 to 6 weeks apart, of fractional Er:YAG laser. Clinical evaluation was done at baseline and 1 month post-treatment (follow-up) by photographic assessment. Subjective evaluation by patient satisfaction score was also performed. Photographic evaluation of the patients revealed a mean percent change in SALT score of 17.4 ± 3.5% at follow-up. A total of 16 patients who were treated for patchy AA of the scalp showed 27.8 ± 31.3% regrowth. Five patients who were treated for AA of the beard had 39 ± 34.2% regrowth. Fractional Er:YAG laser might be a therapeutic alternative for patients with patchy AA of the scalp and beard that is unresponsive to conventional therapies.

Inpatient dermatology consultations during COVID 19 pandemic in a tertiary referral center.

Since the COVID-19 infection first appeared in December 2019, patient profile of outpatient and inpatient clinics has changed. Various cutaneous findings associated with COVID-19 have been reported in the literature. The main objective of this study was to describe and analyze the profile of the consultations requested from dermatology department during the COVID pandemic. Retrospective, cohort study. In this study, we observed the dermatology consultations of the hospitalized patients over a period of 2 months, corresponding to the peak of COVID outbreak in a tertiary care hospital in Turkey. We reviewed the inpatient dermatology consult database retrospectively. Both pediatric and adult dermatology inpatient consultations were evaluated. A total of 166 inpatient dermatology consultations were requested from dermatology department during March-May, 2020. The mean age of the patients was 53.12 (1-89) years. Almost 32.5% (n = 54) of dermatology consultations were requested from the COVID wards and the COVID intensive care unit. The second most common consultations were requested from internal medicine departments (n = 46, 27.7%). The most common indications for the consultations were cutaneous infections (36%), followed by inflammatory disorders (32%), and urticaria (11%). Dermatology consultations have an essential role on the management of hospitalized patients, especially at that pandemic time. Careful dermatological examination improves diagnostic accuracy in skin disorders and skin manifestations of COVID-19 infection that provides an early diagnosis and treatment, helps to improve the quality of the patient care and management.

Cutaneous manifestations in hospitalized patients diagnosed as COVID-19.

Cutaneous manifestations of COVID-19 disease have not yet been fully described. To describe cutaneous manifestations of COVID-19 disease in hospitalized patients. We examined the cutaneous manifestations of 210 hospitalized patients. Cutaneous findings were observed during COVID-19 infection in 52 of the patients. Lesions may be classified as erythematous scaly rash (32.7%), maculopapular rash (23%), urticarial lesions (13.5%), petechial purpuric rash (7.7%), necrosis (7.7%), enanthema and apthous stomatitis (5.8%), vesicular rash (5.8%), pernio (1.9%), and pruritus (1.9%). Cutaneous manifestations were observed statistically significantly more in certain age groups: patients of 55 to 64 and 65 to 74 years of age complained of more cutaneous manifestations than the other age groups. As for gender, there was no significant difference between male and female patients in terms of cutaneus findings. The relationship between comorbidity and dermatological finding status was statistically significant. The relationship increases linearly according to the comorbidities. According to the statistical results, the patients who were hospitalized in the intensive care unit had a higher risk of having cutaneous findings due to COVID-19 infection. With this study, we may highlight the importance of overlooked dermatological findings in patients that are hospitalized.

Relationship between fecal calprotectin level and disease activity in patients with hidradenitis suppurativa.

BACKGROUND: Hidradenitis suppurativa is a chronic, inflammatory, recurrent disease with recurrent abscesses, and sinus tract formation leading to scarring. Calprotectin has immunomodulatory, antimicrobial, and antiproliferative properties and is a calcium-binding protein primarily found in the neutrophil cytoplasm. In recent years, a significant relationship between the activity of various diseases and the level of calprotectin has led to the conclusion that there may be a similar relationship in hidradenitis suppurativa. OBJECTIVE: To determine the relationship between disease activity and fecal calprotectin levels in patients with hidradenitis suppurativa. METHODS: Fifty patients with hidradenitis suppurativa (case group) who present to the Dermatology and Venerology Department between December 6, 2017, and April 6, 2018, and 36 healthy volunteers (control group) were enrolled in our study. Fecal calprotectin levels we requantitatively calculated using enzyme-linked immunosorbent assay. RESULTS: In patients with active hidradenitis suppurativa, the level of stool calprotectin was higher than that of patientsin remission, and this difference was statistically significant (p < .001). There was no statistically significant correlation between disease stage and fecal calprotectin levels in patients with hidradenitis suppurativa (p = .14). Age, sex, smoking and alcohol use, anti-TNF-α treatment, and fecal calprotectin levels were not significantly correlated. In our study, fecal calprotectin levels in patients with active hidradenitis suppurativa were higher than inpatients in remission (p < .001). CONCLUSION: Fecal calprotectin can beused as a marker of disease activity in hidradenitis suppurativa.

Assessment of treatment efficacy of diphenylcyclopropenone (DPCP) for alopecia areata

Background/aim: Alopecia areata (AA) is an inflammatory disease with a genetic and autoimmune basis. Herein, it was aimed to study the efficacy and safety of an immunomodulatory therapeutic agent, diphenylcyclopropenone, while manifesting its association with histopathological features, prognostic factors, and side effects. Materials and methods: In this retrospective study, 98 patients (60 males, 38 females) with alopecia, who were referred to the Hair Disease Polyclinic at the Department of Dermatology, between 2011 and 2015, were included. Together with medical histories and dermatological examinations, a skin biopsy for histopathological examination was conducted for all of the patients prior to therapy. Therapeutic success was evaluated on the basis of the hair regrowth percentage. Results: Regarding the overall treatment success, 33 (34%) patients had complete response, 16 (16%) had partial response (between 50% and 99%), 27 (28%) had minimal response (between 1% and 49%), and 22 (22%) were nonresponders. Both sexs were equally represented in the outcome. Conclusions: There was a significant relation between the severity of alopecia and the treatment outcome (P = 0.038). Patients with AA had significantly better response when compared to those with alopecia totalis and universalis. There was no statistically significant relation with other parameters, such as disease duration, age, sex, atopy history, age of onset, and histopathological features.

Patients' experiences with Behçet's syndrome: structured interviews among patients with different types of organ involvement.

OBJECTIVES: Behçet's syndrome (BS) is a multisystem variable-vessel vasculitis with significant life impact. The aim of this study was to explore the perspectives of patients with BS with different types of organ involvement. METHODS: Semi-structured qualitative interviews were conducted with 20 patients with BS with different types of organ involvement. Interviews were audio-recorded, transcribed, and translated into English. A Grounded Theory approach was employed in thematic analysis of translated interviews. RESULTS: Interviews with participants yielded four themes, including symptoms (skin problems, pain, vision problems, fatigue/sleep disturbances, and gastrointestinal/weight loss), impact on function (impact on speech and vision, mobility, energy for tasks, adaptations, and self-care), psychological impact (emotions and emotional management techniques), and social impact (ability to socialize generally and impact on familial relationships). CONCLUSIONS: Patients with BS identified several domains, including physical functioning, psychological state, and social identity that are significantly modulated by the symptoms of BS. Those are inter-related with physical symptoms, reflecting the multi-system character of BS, and impair patients' function impacting on psychological and social identities. This work advances an understanding of BS, and will be useful in developing patient-oriented outcome measures for use in studying BS.

Biosimilar infliximab for Behçet's syndrome: a case series.

OBJECTIVES: The efficacy and safety of biosimilar infliximab (bio-IFX) was shown in randomised controlled trials and it was approved for all indications of the reference product in several countries. However, a previous case series of 3 patients with Behçet's syndrome (BS) reported disappointing results. We aimed to share our experience with bio-IFX treatment in different types of organ involvement in patients with BS. METHODS: We reviewed the charts of all BS patients who were prescribed reference infliximab (ref-IFX) or bio-IFX in our BS clinic. Among the 181 BS patients who were prescribed IFX since 2003, 6 (3%) were prescribed bio-IFX due to refractory disease despite conventional immunosuppressives. RESULTS: A total of 6 patients (mean age: 32.1±6.2, mean disease duration: 5.3±1.8 years, 5 men and 1 woman) received bio-IFX for uveitis, nervous system, vascular and joint involvement. Four of the 6 patients obtained remission and stayed in remission during the 16±6.5 months they used bio-IFX. Among the 4 patients who obtained remission, 2 were switched to ref-IFX due to unavailability of bio-IFX infusion set and did not experience adverse events or loss of efficacy. However, relapses occurred during tapering. The other 2 patients are still in remission with bio- IFX. Among the remaining 2 patients, one had to be switched to ref-IFX after the first infusion, due to a change in the reimbursement policy and the other was non-responsive. CONCLUSIONS: Our limited experience showed that bio-IFX may be a safe and effective alternative for patients with BS, refractory to conventional immunosuppressives.

Metabolic syndrome in dermatology: Treatment and Management for Dermatologists.

Metabolic syndrome, a commonly observed pathology and a global health issue, is closely related to the skin and thus dermatologists. The skin contributes to the development of metabolic syndrome and it is an end organ for it. Metabolic dysfunction is related to many skin disorders, some of which with high association and some of which with lower association. The diseases of highest association will be discussed in this article. These diseases are acne vulgaris, acanthosis nigricans, hidradenitis suppurativa, and psoriasis vulgaris.

Cutaneous foreign body granulomas associated with lipolytic cocktail: Who is the enemy, mesotherapy or drugs injected?

Mesotherapy is widely used for its lipolytic effect as an alternative procedure to surgical methods. Although many benefits of lipolytic mesotherapy have been observed, numerous side effects have also been reported. Here, we report a case of cutaneous foreign body granulomas that occurred after lipolytic mesotherapy.

The Pathergy Test as a Diagnostic Tool.

The pathergy test produces a nonspecific hyperreactive lesion in Behçet's disease (BD), a finding that has been known since 1937. Pathergy refers to the development of new skin lesions or the aggravation of existing ones after trivial trauma. In clinical practice, the pathergy test induces a skin response by needleprick, with positive reactions manifesting as a papule or pustule developing by 48 hours. The pathergy test is one of the major features and diagnostic criteria of the disease. It is very similar to the erythematous papules or pustules that appear spontaneously in patients with BD. There is no standardized method for conducting the pathergy test. Intradermal, intravenous, and subcutaneous applications are used. There is no generally accepted opinion on which form of the test yields a higher positivity rate. The pathergy reaction is also reported in pyoderma gangrenosum, and has been noted in other neutrophilic dermatoses such as Sweet syndrome. The overall objective of this contribution is to provide a review of the available information, literature, and research relating to the pathergy test.