Evaluation of the Quality of Life and the Demographic and Clinical Characteristics of Patients With Pemphigus With Oral Mucosal Involvement: A Multicenter Observational Study.

INTRODUCTION: Pemphigus vulgaris (PV) is an autoimmune disease primarily affecting the oral mucosa. OBJECTIVES: This study aimed to determine the demographic, clinical and treatment characteristics of PV patients with oral mucosal involvement and to assess the impact on their quality of life. METHODS: We conducted a prospective observational study among 106 patients diagnosed with PV and presenting oral mucosal involvement. Demographic data, clinical and treatment characteristics, and quality of life questionnaires were recorded. RESULTS: The study included 106 patients, 55 (51.89%) were male and there was a predominance of the mucocutaneous subtype in 83 individuals (78.38%). Oral mucosa was the initial site of manifestation in 44 patients (41.51%). Bilateral buccal mucosa was the most frequently affected site. The predominant symptom reported was a burning sensation, noted in 91 patients (85.85%). Oral mucosal examination revealed erosions in 85.85% of the patients. Systemic steroids were the most commonly administered treatment, and rituximab was used in 18 patients (16.98%). A positive and significant correlation was found between pemphigus severity and Oral Health Impact Profile-14, Dermatology Life Quality Index and Dermatological Quality of Life Scale scores (P < 0.05). The presence of superficial ulcers, flaccid bullae, lesion diameter ≥1 cm, and >10 lesions were factors that markedly diminished quality of life. Complete response to treatment was noted in all patients administered rituximab. CONCLUSIONS: The most common area of involvement was bilateral buccal mucosa, and the severity of PV closely correlated with a decline in quality of life measures. These results highlight the need for careful clinical oversight of PV, taking into account its effects on patients quality of life.

Analysis of clinical characteristics and factors affecting treatment responses in patients with pyoderma gangrenosum: a multicenter study of 239 patients☆.

BACKGROUND: Pyoderma Gangrenosum (PG) is a chronic disease characterized by recalcitrant skin ulcers. OBJECTIVE: We aimed to evaluate the demographic, clinical characteristics, treatments and factors affecting the treatment responses of patients with PG. METHODS: We performed a multicenter study of 12 tertiary care centers. We analyzed the data of the patients who were followed up with a diagnosis of PG between the years 2012‒2022 retrospectively. RESULTS: We included a total of 239 patients of whom 143 were female and 96 were male, with an average age of 54.2 ±â€¯17.4 years. The most common treatment was systemic steroids (n = 181, 75.7%). Among these patients, 50.8% (n = 92) used systemic steroids as the sole systemic agent, while 49.2% (n = 89) used at least one adjuvant immunosuppressive agent. The independent factors determined in regression analysis to influence response to systemic steroids positively were disease onset age ≥ 30-years, negative pathergy, absence of leukocytosis, negative wound culture, presence of a single lesion, and absence of upper extremity involvement. Biological agents were used in 18.4% (n = 44) of the patients in the present study. We also analyzed pathergy positive PG and early onset (onset age < 30) PG separately due to their distinct clinical features which were revealed during statistical analysis. STUDY LIMITATIONS: Retrospective nature of the present study. CONCLUSIONS: Analyses of the factors influencing treatment responses are addressed in this study. Also, we concluded that investigation for accompanying autoinflammatory diseases of pathergy positive PG and early onset PG is necessary and the patients in these two groups are more resistant to treatment, necessitating more complicated treatments.

S2k guidelines on the management of paraneoplastic pemphigus/paraneoplastic autoimmune multiorgan syndrome initiated by the European Academy of Dermatology and Venereology (EADV).

BACKGROUND: Paraneoplastic pemphigus (PNP), also called paraneoplastic autoimmune multiorgan syndrome (PAMS), is a rare autoimmune disease with mucocutaneous and multi-organ involvement. PNP/PAMS is typically associated with lymphoproliferative or haematological malignancies, and less frequently with solid malignancies. The mortality rate of PNP/PAMS is elevated owing to the increased risk of severe infections and disease-associated complications, such as bronchiolitis obliterans. OBJECTIVES: These guidelines summarize evidence-based and expert-based recommendations (S2k level) for the clinical characterization, diagnosis and management of PNP/PAMS. They have been initiated by the Task Force Autoimmune Blistering Diseases of the European Academy of Dermatology and Venereology with the contribution of physicians from all relevant disciplines. The degree of consent among all task force members was included. RESULTS: Chronic severe mucositis and polymorphic skin lesions are clue clinical characteristics of PNP/PAMS. A complete assessment of the patient with suspected PNP/PAMS, requiring histopathological study and immunopathological investigations, including direct and indirect immunofluorescence, ELISA and, where available, immunoblotting/immunoprecipitation, is recommended to achieve a diagnosis of PNP/PAMS. Detection of anti-envoplakin antibodies and/or circulating antibodies binding to the rat bladder epithelium at indirect immunofluorescence is the most specific tool for the diagnosis of PNP/PAMS in a patient with compatible clinical and anamnestic features. Treatment of PNP/PAMS is highly challenging. Systemic steroids up to 1.5 mg/kg/day are recommended as first-line option. Rituximab is also recommended in patients with PNP/PAMS secondary to lymphoproliferative conditions but might also be considered in cases of PNP/PAMS associated with solid tumours. A multidisciplinary approach involving pneumologists, ophthalmologists and onco-haematologists is recommended for optimal management of the patients. CONCLUSIONS: These are the first European guidelines for the diagnosis and management of PNP/PAMS. Diagnostic criteria and therapeutic recommendations will require further validation by prospective studies.

Frequency of skin cancer and evaluation of risk factors: A hospital-based study from Turkey.

OBJECTIVES: Skin cancers are the most common type of cancer with a significantly increasing incidence. The purpose of the study was to uncover the one-year frequency of melanoma and non-melanoma skin cancers (NMSC) and to determine the risk factors in the development of skin cancer. METHODS: The study included 7396 people from all age groups admitted to the dermatology clinic between October 2020 and 2021. The sociodemographic characteristics, sun protection habits, chronic diseases, and drug and vitamin use were evaluated. Lesions with clinical suspicion of skin cancer were excised. RESULTS: The frequency of skin cancer was found to be 2.7%, basal cell cancer (BCC) 1.2%, squamous cell cancer (SCC) 1.1%, malignant melanoma (MM) was 0.4%. Daily black tea consumption was found to be a risk factor for three type of skin cancer, BCC (p = 0.021), SCC (p = 0.006), and MM (p = 0.002), respectively. Obesity was observed as a risk factor for BCC (p = 0.005) and MM (p = 0.008). We found that having a history of alcohol use were an independent risk factor for all skin cancer types and BMI <30 for SCC. Vitamin D and supplemental drugs intake were observed as protective factors for BCC (p = 0.035, p = 0.007, respectively). Daily coffee consumption was determined as a protective factor for SCC (p < 0.001) and MM (p = 0.049). CONCLUSION: This study estimates the frequency of NMSC and melanoma. Also provides evidence to determine the risk factors and probably protective factors for the development of skin cancers.

Retrospective analysis of methylprednisolone treatment alone and in combination with methotrexate in patients with extensive alopecia areata.

Alopecia areata (AA) is a chronic autoimmune disorder that primarily affects the hair follicle. Systemic corticosteroids and methotrexate (MTX) are among the therapeutic options in severe cases. This study aimed to show whether the combination therapy of methylprednisolone (MP) and MTX was superior to MP alone in the management of extensive AA. A total of 26 patients with extensive AA, 14 treated with MP alone and 12 treated with the combination of MP and MTX, were retrospectively evaluated in terms of gender, age, severity of disease, clinical characteristics, disease duration, dose and duration of medications, therapy response, and side effects. Of the 26 patients with extensive AA, 14 were male and 12 were female, and the average age was 17.02 ± 10.70 years. All patients had more than 50% hair loss, 23 had extensive multifocal AA, and three had alopecia totalis. A total of 14 patients were treated with MP alone (starting dose: 0.3-0.5 mg/kg, maximum 32 mg/day), and 12 were treated with MP + MTX (starting dose: 5-15 mg/week, maximum 20 mg/week). A total of 12 of the 14 patients (85.7%) who were treated with MP alone showed a complete response, with the response rate of the patients who showed more than 50% response being 92.85%. Seven of the 12 patients (58.3%) who were treated with MP + MTX achieved complete healing, and all patients on this regimen had more than 50% treatment response. Our results showed that the combination therapy of MP and MTX was not superior to MP alone in the management of extensive alopecia areata.

Isotretinoin-induced inflammatory back pain and sacroiliitis in patients with moderate-to-severe acne vulgaris.

INTRODUCTION: Isotretinoin has been reported to induce inflammatory back pain (IBP) and sacroiliitis in the patients with acne vulgaris. The aim of this study is to investigate the incidence of IBP and sacroiliitis in patients receiving isotretinoin treatment compared with oral antibiotics for acne vulgaris. MATERIALS AND METHODS: A total of 201 patients with moderate-to-severe acne vulgaris who received isotretinoin (n = 100) or oral antibiotics (n = 101) were included in the study. All patients were monthly questioned for IBP symptoms during their treatment. Patients described IBP were also evaluated for sacroiliitis by c-reactive protein, sedimentation rate, HLAB27, and sacroiliac magnetic resonance imaging (MRI). Isotretinoin was discontinued in all patients diagnosed as sacroiliitis, and these patients were reevaluated after 3 months. RESULTS: IBP was observed in 21 (10.4%), and sacroiliitis was detected in 11 (11%) patients on isotretinoin treatment; in oral antibiotic group, we did not observe IBP or sacroiliitis. The incidence of IBP and sacroiliitis differed significantly between the isotretinoin and oral antibiotic groups (p < 0.0001, p = 0.02). Complete regression was observed in the great majority of patients following cessation of isotretinoin. CONCLUSIONS: Our study is the largest prospective controlled study that investigated the incidence of sacroiliitis in patients receiving isotretinoin and compared with patients using oral antibiotics.

Incidence and Prognosis of COVID-19 in Patients with Psoriasis: A Multicenter Prospective Study from the Eastern Black Sea Region of Turkey.

COVID-19 infection can have a poor prognosis, especially in patients with chronic diseases and those receiving immunosuppressive or immunomodulating therapies. This study aimed to investigate the severity of COVID-19 infection in patients with psoriasis and compare the infection severity for systemic treatments and comorbidities. We conducted a study in the dermatology clinics of five different centers in the Eastern Black Sea region of Turkey. Four hundred and eighty-eight patients were included, and 22.5% were confirmed as having COVID-19 infection. In our study, the frequency of hospitalization rates due to COVID-19 infection were similar (15.4%, 25.9% respectively) in patients receiving biological treatment and receiving non-biological systemic treatment (P=0.344). Hospitalization rates were higher in patients with hypertension, androgenetic alopecia, and acitretin use (P=0.043, P=0.028, P=0.040). In conclusion, current biologic treatments and non-biologic systemic treatments in patients with psoriasis did not appear to increase the risk of the severe form of COVID-19, except for acitretin.

A multicentre prospective analysis of the incidence of pemphigoid diseases in Turkey.

BACKGROUND: The differentiation between the pemphigoid diseases is essential for treatment and prognosis. In Turkey, data on the incidence of these diseases are insufficient. Our aim in this study is to determine the incidence, demographics and clinical characteristics associated with diseases of the pemphigoid group. METHODS: We prospectively analysed 295 patients with pemphigoid who visited dermatology clinics of tertiary referral hospitals in 12 different regions of Turkey within a year. The diagnosis was based on clinical, histopathological, direct immunofluorescence (DIF) and serological (multivariant enzyme-linked immunosorbent assay [ELISA], indirect immunofluorescence and mosaic-based BIOCHIP) examinations. Clinical and demographic findings, aetiological factors and concomitant diseases observed in the patients were recorded. RESULTS: A total of 295 (female/male ratio: 1.7/1) patients with pemphigoid were diagnosed in 1-year period. The overall incidence rate of pemphigoid diseases was found to be 3.55 cases per million-years. The ratio of pemphigoid group diseases to pemphigus group diseases was 1.6. The most common pemphigoid type was bullous pemphigoid (BP, 93.2%). The others were epidermolysis bullosa acquisita (3.1%), pemphigoid gestationis (2.4%), linear IgA disease (1%) and mucous membrane pemphigoid (0.3%). The most common (26.8%) possible trigger of the bullous pemphigoid was gliptin derivative drugs. The most common concomitant diseases with pemphigoid were cardiovascular (27.8%) and neurological diseases (23.7%). CONCLUSIONS: This study showed that the increased frequency of bullous pemphigoid reversed the pemphigoid/pemphigus ratio in Turkey. Further studies are warranted regarding the reasons for this increase.

Prevalence and Dermoscopic Patterns of Acral Melanocytic Nevi in Turkey.

Although nevi are frequently encountered in the acral region, very limited studies have reported their prevalence in specific populations. We aimed to determine the prevalence of acral nevi, their dermoscopic patterns, and evaluate patient awareness in a Turkish population. We prospectively examined 2644 patients admitted to the outpatient dermatology clinics between October 2016 and October 2017. The characteristics of the detected acral nevi and dermatoscopic images were recorded. A questionnaire of demographic characteristics was completed from all patients. Two hundred six of the 2644 patients had at least one acral nevus. Two hundred sixty nevi were examined. The general prevalence of acral nevi was 7.8%. Women were more likely to have acral nevi than men (8.7% vs. 6.3%; P=0.028). Moreover, darker-skinned patients were also had significantly more acral nevi (8.6% in skin type III-IV vs. 6.0% in skin type I-II; P<0.001). The prevalence of acral nevi was 9.4% before the age of 20, 9.5% in patients aged 20-40 years, and 4.6% after the age of 40. In addition, 51.5% of all nevi exhibited a parallel furrow, 13.5% were lattice-like, and 7.7% had a homogeneous pattern. The overall nevus awareness rate was 73.8% and was significantly higher in women at 78.3%. Our study is the first large-scale study of that showed the prevalence of acral nevi in Turkey. According to our study, the prevalence of acral nevi was higher in patients with female sex and darker skin type. We also found that the prevalence of acral nevi decreased over 40 years of age. The general awareness of nevi was higher in women.

Evaluation of factors that may affect disease development and severity in childhood atopic dermatitis.

BACKGROUND: Atopic dermatitis is a chronic, relapsing, inflammatory disease. Data concerning the role of perinatal conditions in the development of the disease are few and inconsistent. The purpose of this study was to investigate the effects of skin care on the severity of the disease, also to evaluate the relations of perinatal conditions with disease development and severity. METHODS: Seventy-five patients with atopic dermatitis aged 2-10 years, their parents and 65 healthy age-compatible volunteers and their parents were included in the study. The Scoring Atopic Dermatitis (SCORAD) score was used to assess the severity of the disease. RESULTS: Emollient use (regular/irregular) was higher in patients with severe disease compared to those with mild and moderate disease, but no significant difference was determined in regular use rates (P=0.029, and P=0.504, respectively). Topical corticosteroid use and sleep disturbance rates in the previous three months in the subjects regularly using emollients were low (P=0.032, and P=0.005, respectively). The incidence of severe disease was higher in the patients born in the winter (P=0.033). Severe disease rates were significantly high in the subjects in the patient group with advanced maternal age, a history of cesarean delivery and incubator use (P=0.017, P=0.046, and P=0.025, respectively). CONCLUSIONS: Low topical corticosteroid requirement and sleep disturbance rates in the subjects regularly using moisturizers emphasize the importance of emollient use in treatment. The association of cesarean delivery, history of incubator, and birth in winter with severe disease suggests that disease severity is related to less exposure to environmental allergens in the hygiene hypothesis.

Drug survival of biologic treatments in Turkish patients with psoriasis.

Psoriasis is a chronic, immune-mediated disease deriving from a polygenic predisposition. Treatment paradigm of the moderate to severe psoriasis has changed in the last two decades as biologics have been developed. Real-world data concerning biologics in the treatment vary from country to country. We aimed to investigate the efficacy and drug survival of biologic agents in patients treated in our clinic in Turkey. Data for 211 biologic protocols were examined for 125 patients treated with a biologic at least once for a minimum duration of 16 weeks at our department. The drug survival rate for 3 years was highest among ustekinumab (UST) users. The primary failure rate in the present study was higher among tumor necrosis factor-alpha inhibitors compared to UST (P < .0001). The higher primary failure rate was found in etanercept users and the highest secondary failure rate was observed in infliximab, while the lowest primary and secondary failure rates were determined in UST users. UST use and articular involvement emerged as significant positive predictors of drug survival in naïve patients. Our study is the first study presenting real-life data and biologic survival in the treatment of psoriasis in Turkey, and UST exhibited significantly higher drug survival scores, particularly in naive patients.

Sun-protection habits and knowledge of patients with vitiligo.

INTRODUCTION: Vitiligo is a common chronic depigmentation disease. Patients are generally advised to protect themselves from the sun. METHODS: One hundred fifty-three patients diagnosed with vitiligo and 106 healthy volunteers with no additional dermatological disease were included in the study. We evaluated the sun-protection habits of patients with vitiligo and controls, and also assessed their knowledge and attitudes toward sun exposure. RESULTS: Rates of sunscreen use, high-factor sunscreen use, and remaining in the shade were significantly higher among patients (p = 0.004, p = 0.028, p = 0.040). We found significantly higher rates of modifying vacation habits, high sun-protection factors (SPF) sunscreen use, and preferring to remain in the shade among patients using phototherapy (p = 0.016, p = 0.019, p = 0.028). We showed higher rates of modification of vacation habits, wearing sunglasses, and remaining in the shade among patients with longer disease durations (p = 0.026, p = 0.001, and p = 0.017). CONCLUSIONS: We determined higher rates of sunscreen use, high-SPF sunscreen use, and tendencies to remain in the shade in vitiligo patients compared to the general population. We also determined that disease duration and treatment with phototherapy alters sun-protection habits, but the presence of generalized disease and lesions in visible areas such as the face does not alter patients' habits.

Impairment of myocardial functions and arterial stiffness in patients with lichen planus.

BACKGROUND: Lichen planus is a chronic inflammatory mucocutaneous disease. Recent studies have suggested that it is associated with an increased risk of cardiovascular comorbidities. OBJECTIVE: The purpose of this study was to assess and compare arterial stiffness and cardiovascular hemodynamics in patients with lichen planus and a healthy control group. METHODS: Fifty-five patients with lichen planus and 42 healthy controls were enrolled. All patients underwent echocardiographic examination, and arterial stiffness was measured using applanation tonometry. RESULTS: No statistically significant difference was determined between the patient and control groups in terms of arterial stiffness, but stiffness was markedly higher in patients with erosive lichen planus compared to the control group and other patients (p=0.006, and p=0.023, respectively). Moderate positive correlation was determined between duration of disease and arterial stiffness. Impairment of systolic and diastolic functions was also determined in patients with lichen planus compared to the control group (p<0.001, and p=0.005, respectively). STUDY LIMITATIONS: Relatively low number of patients. CONCLUSION: The positive correlation observed between duration of disease and arterial stiffness in patients with lichen planus suggests that these patients should be followed-up in terms of cardiovascular risk in the presence of resistant and long-term disease, particularly in case of erosive lichen planus.

Diagnosis and management of pemphigus: Recommendations of an international panel of experts.

BACKGROUND: Several European countries recently developed international diagnostic and management guidelines for pemphigus, which have been instrumental in the standardization of pemphigus management. OBJECTIVE: We now present results from a subsequent Delphi consensus to broaden the generalizability of the recommendations. METHODS: A preliminary survey, based on the European Dermatology Forum and the European Academy of Dermatology and Venereology guidelines, was sent to a panel of international experts to determine the level of consensus. The results were discussed at the International Bullous Diseases Consensus Group in March 2016 during the annual American Academy of Dermatology conference. Following the meeting, a second survey was sent to more experts to achieve greater international consensus. RESULTS: The 39 experts participated in the first round of the Delphi survey, and 54 experts from 21 countries completed the second round. The number of statements in the survey was reduced from 175 topics in Delphi I to 24 topics in Delphi II on the basis of Delphi results and meeting discussion. LIMITATIONS: Each recommendation represents the majority opinion and therefore may not reflect all possible treatment options available. CONCLUSIONS: We present here the recommendations resulting from this Delphi process. This international consensus includes intravenous CD20 inhibitors as a first-line therapy option for moderate-to-severe pemphigus.

Mycosis Fungoides: A 10-year Turkish Experience.

Mycosis fungoides is the most common primary cutaneous T cell lymphoma, characterized by erythematous patches and plaque lesions with slow progression to cutaneous tumors or extracutaneous involvements in some patients. We aimed to evaluate the clinical characteristics, treatment responses, disease courses, and mortality rates of our MF cases. The data of 100 patients with MF were retrospectively examined from medical records in our clinic between January 2005 and January 2015. Demographic and clinical characteristics of the patients, disease stage, treatment protocols, response to treatment, recurrence, progression, and mortality rates were recorded. The male to female ratio in patients was 1.2. Mean age at onset of disease was 46, and duration of disease ranged from one to 42 years. At time of diagnosis 31 patients were at stage 1A, 31 at stage 1B, 30 at stage 2A, 2 at stage 2B, 1 at stage 3, and 5 at stage 4. Stable disease was observed in 35% of patients, progression in 10%, relapse in 27%, and complete response in 28%. Large cell transformation was found in 3 patients and additional malignity in 11. Thirty-seven patients (37%) were still surviving disease-free. 10 patients had died, three of them due to disease-related conditions. The most common first-line therapy in our study was phototherapy. It was applied to 87% of patients from stage 1A. Our results are generally consistent with current literature, but disease progression and disease-specific mortality rates were significantly lower than the literature, probably due to early phototherapy.

High levels of endothelial progenitor cells and circulating endothelial cells in patients with Behçet's disease and their relationship to disease activity.

BACKGROUND: Behçet's disease is a multisystemic vasculitis, associated with vascular endothelial dysfunction. Currently, the prognosis is unpredictable, because there is still no valid laboratory marker indicating the disease activity in Behçet's disease. Endothelial progenitor cells and circulating endothelial cells are newly introduced hematological markers which are presumed to take part in the pathogenesis of vasculitis. OBJECTIVES: To evaluate the levels of endothelial progenitor cells and subtypes and circulating endothelial cells in patients with Behçet's disease and to describe their relationship with the disease activity. METHODS: A total of 45 patients with Behçet's disease and 28 healthy controls were included in the study. Endothelial progenitor cells (CD34+CD133+KDR+ as early endothelial progenitor cells and CD34+KDR+ as late endothelial progenitor cells), and circulating endothelial cells (CD34+CD133+) were measured by flow cytometry. RESULTS: The mean plasma level of endothelial progenitor cells and circulating endothelial cells, vascular endothelial growth factor, matrix metalloproteinase-9, C-reactive protein, and erythrocyte sedimentation rate were significantly higher in patients with Behçet's disease. All of these parameters except circulating endothelial cells were also found to be higher in patients with active disease than in patients with inactive disease. Early endothelial progenitor cells showed significant correlations with C-reactive protein and circulating endothelial cells. STUDY LIMITATIONS: The cross-sectional nature of the study and patient characteristics such as being under treatment, which can affect endothelial progenitor cells numbers. CONCLUSION: The increase in endothelial progenitor cells may play an essential role in the repair of endothelial injury in Behçet's disease, especially in the active period of the disease. Thus, endothelial progenitor cells can indicate the disease activity. In addition, endothelial progenitor cells and circulating endothelial cells can be used as endothelial repair and injury markers for Behçet's disease, respectively.