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.

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.

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.