An Anti-Interleukin-17A Monoclonal Antibody, Ixekizumab, in the Treatment of Resistant Hidradenitis Suppurativa: A Case Series.

Introduction: Although adalimumab is the only approved biologic for the treatment of hidradenitis suppurativa (HS), the treatment response may not be satisfactory in all patients. Recently, many other biological agents, including interleukin 17 inhibitors such as ixekizumab, have shown promise. Case Presentations: Five severe HS (Hurley stage III) patients resistant to conventional treatments and adalimumab for at least 3 months were recruited. Patients were prescribed ixekizumab with a scheme approved for psoriasis (160 mg once, followed by 80 mg at weeks 2, 4, 6, 8, 10, and 12.) The primary outcome measure was achieving the Hidradenitis Suppurativa Clinical Response (HiSCR) score following 12 weeks. Secondary outcome measures included the patient-reported Dermatology Life Quality Index (DLQI) and visual analog scale (VAS). Four of 5 patients (80%) achieved HiSCR. While improvement was observed in the VAS and DLQI scores of 4 patients, the decline was limited in 1 patient. No adverse event was recorded related to ixekizumab. Conclusion: The result of our observation suggests that ixekizumab may be effective for HS, especially in challenging cases.

Certolizumab pegol in the treatment of severe hidradenitis suppurativa after adalimumab failure: A real-life experience.

There exists an unmet need to treat hidradenitis suppurativa (HS) despite several approved therapeutic agents for its treatment. We sought to investigate the role of certolizumab pegol in severe, recalcitrant HS patients unresponsive to adalimumab. This retrospective cohort includes HS patients over 18 years of age who had a history of unresponsiveness to adalimumab and whose treatments were switched to certolizumab pegol with dosing similar to psoriasis (400 mg every 2 weeks). For subjects who achieved a hidradenitis suppurativa clinical response (HiSCR) following 12 and 24 weeks of treatment, dermatological life quality index (DLQI), abscess, inflammatory nodule count (AN count), and International Hidradenitis Suppurativa Severity Score System (IHS4) were evaluated as outcome measures. Eleven severe, recalcitrant HS patients with Hurley stage III HS were enrolled for this study. All patients were male and had a history of prior adalimumab exposure. Only three (27.2%) patients also had a history of using biologic agents other than adalimumab for HS. Six of 11 patients (54.5%) achieved HiSCR at week 12. However, two among these six responders lost response at week 24 despite continued therapy (HiSCR at week 24: 33.3%). The decrease in DLQI (p: 0.017 and 0.021) and IHS4 (p: 0.008 and 0.007) scores of the patients at weeks 12 and 24 showed a significant difference compared to the baseline. Certolizumab pegol is a promising treatment option for severe, recalcitrant HS patients who are unresponsive to adalimumab.

Pemphigus vulgaris after SARS-CoV-2 vaccination: A case with new-onset and two cases with severe aggravation.

Pemphigus may be induced or aggravated by certain drugs and vaccines. People worldwide are currently vaccinated with several SARS-CoV-2 vaccines which may be associated with increased number of aggravated or triggered autoimmune bullous diseases in subjects with an underlying genetic predisposition. Herein, a case of new-onset pemphigus vulgaris (PV) and two cases with aggravation of PV after vaccinations for SARS-CoV-2 are reported.

Patients With Hidradenitis Suppurativa Negatively Perceive Both Medical and Euphemistic Appellations of Their Disease: A Study From Turkey.

BACKGROUND: The use of medical terms and folk names (euphemisms) affect a patient's understanding of diseases and perceptions of severity. OBJECTIVES: We determine the psychological effects on patients with hidradenitis suppurativa of medical and folk names of their disease. METHODS: This was a cross-sectional and exploratory study conducted at a tertiary referral university hospital in Turkey. A questionnaire on the medical and folk names of hidradenitis suppurativa was administered to 31 males and 25 females. RESULTS: The patients expressed that they found the medical term hidradenitis suppurativa to be incomprehensible because it is a foreign term. When hearing it for the first time, it evoked negative responses such as confusion and worry about their health. Half of the patients preferred their doctors to use a more understandable and pronounceable name. More than 80% of patients expressed feeling depressed and stigmatized by the folk name of their disease. They preferred the terms boils, abscesses, or hidradenitis when referring to their disease. CONCLUSION: Both medical and folk names for hidradenitis suppurativa have negative effects on patients, and most patients feel stigmatized by either term.

Family physicians' awareness and general knowledge of hidradenitis suppurativa: A survey research.

RATIONALE, AIMS, AND OBJECTIVE: Medical consultations of family physicians play a key role in early diagnosis and proper management of hidradenitis suppurativa (HS). This study aimed to investigate family physicians' disease awareness, general attitudes, and knowledge of HS. MATERIAL-METHODS: This cross-sectional descriptive study was conducted with 211 family physicians and residents through a web-based survey. The questionnaire consisted of 25 questions about the demographic characteristics of the participants as well as their awareness and general knowledge of HS. RESULTS: While 90% of physicians know that the diagnosis of HS is made clinically without any need for a laboratory examination, only 23.7% of them felt confident performing such a diagnosis. About 63% of participants defined HS as an infectious disease of the apocrine glands, contradictive to the real pathophysiology. About 21% of them had not been medically trained in HS, and 3% of them even had never heard the name of HS. Only 33% were aware of the role of biological agents. Most of the family physicians (57.4%) were willing to participate in postgraduate education programmes about HS. CONCLUSION: The family physicians' awareness of HS is limited. Increased practice in medical education and postgraduate education programmes may help increase the knowledge of family physicians.

Rapid Response to Certolizumab Pegol in Hidradenitis Suppurativa: A Case Report.

INTRODUCTION: Hidradenitis suppurativa (HS) is a chronic inflammatory skin disease of hair follicles which usually affects patients' quality of life negatively. Although there are various therapeutic approaches including topical and systemic antibiotics, retinoids, biological agents, and also surgical modalities, there is no curative treatment option and clinical management of HS is still challenging. CASE PRESENTATION: Herein, we present a 43 year-old man with HS who had used different treatments in 15 years. The patient showed improvement initially with adalimumab, then exhibited secondary unresponsiveness after 18 months. While worsening of HS was recorded due to increased discharge under secukinumab, no additional benefit was observed with methotrexate. He was not willing to undergo surgical procedures due to previous surgical experience. Finally, certolizumab pegol has been started to patient with similar dosing to psoriasis patients. Sartorius, 10-point visual analogue scale (VAS), and dermatological life quality index (DLQI) scores regressed from 171 to 105, 9/10 to 3/10, and 27 to 19 after 3 months with certolizumab pegol treatment. DISCUSSION/CONCLUSION: Certolizumab pegol is a humanized antigen-binding fragment of a monoclonal antibody which binds to TNF-alpha. However, certolizumab pegol is only approved for plaque psoriasis in dermatology; it is likely to be a promising effective agent for HS, especially in challenging cases.

Disease severity and poor mental health are the main predictors of stigmatization in patients with hidradenitis suppurativa.

Hidradenitis suppurativa (HS) causes significant psychological distress in patients. We aimed to investigate the stigmatization level of HS patients and to determine the related physical, social, and psychological factors. A questionnaire with 22 questions about the physical and psychosocial problems related to HS was administered to 29 male and 34 female patients between the ages of 18 and 66 to evaluate the thoughts, emotions, attitudes, and behaviors of patients. Each patient filled Dermatology Life Quality Index, Beck Depression Inventory, Liebowitz Social Anxiety Scale, Body Image Scale, and 6-item Stigmatization Scale. Hurley classification was used to assess severity of disease. Patients with HS feel stigmatized depending on physical and psychosocial problems caused by the disease. Higher stigmatization scores correlated with higher scores of depression (r: .437, P < .001), social anxiety (r: .263, P: .03), and worse life quality (r: .522, P < .001), and body image perception (r: .696, P < .001). The degree of stigmatization was closely associated with the presence of painful lesions and disruption of socializing (P: .021 and .033, respectively). Disease severity (ß: 5.12, P: .003 for moderate disease) and reporting feeling psychologically negatively affected due to HS (ß: 4.26 P: .007) were the two main predictors of stigmatization in patients with HS. Cross-sectional nature of the study is the limitation. In conclusion, the stigmatization level of patients with HS is strongly related to disease severity and poor mental health.

Nicolau syndrome following subcutaneous glatiramer acetate injection: A case report and review of the literature.

Nicolau syndrome is a rare serious drug reaction associated with the administering various injectable medications. It is often characterized by an acute and severe pain accompanying erythema that tends to rapidly evolve into the livedoid reticular or hemorrhagic patches and less commonly to ulcers and skin necrosis. Herein, we report a 34-year-old woman who presented with painful, tender discoloration over her abdominal skin following subcutaneous glatiramer acetate injection. Since the patient was diagnosed with multiple sclerosis 18 months ago, she had been on treatment with subcutaneous glatiramer acetate injections thrice weekly. The patient was diagnosed with Nicolau syndrome clinically and histopathologically. After 15-day treatment with topical betamethasone valerate and mucopolysaccharide polysulfate cream twice daily, the lesion completely regressed with only minimal hypopigmented irregular scarring. Nicolau syndrome should be considered in patients with severe pain, tenderness, and redness localized at the injection site following glatiramer subacetate.

Medical and socioeconomic challenges of private dermatocosmetology clinics during COVID-19 pandemic: A survey from Turkey.

BACKGROUND: Coronavirus 2019 (COVID-19) disease has rapidly spread worldwide with a multitude effects on daily life. Since the transmission risk increases with close contact, some cosmetic procedures are considered high risk and majority of them had to be postponed or canceled in private dermatocosmetology clinics especially during the heavy period of the outbreak. AIMS: We aimed to document the medical and socioeconomic problems emerged in dermatocosmetology clinics in Turkey caused by COVID-19 pandemic and to discuss the management strategies taken by dermatologists. PATIENTS/METHODS: This survey research was conducted with 100 dermatologists who work in private dermatocosmetology clinics. The survey included 38 questions about office re-arrangements including patient admission and office environment, safety precautions taken for cosmetic procedures, management of clinic staff, and financial impact of the pandemic. RESULTS: A remarkable decrease in major cosmetic interest was reported in private clinics; meanwhile, there was an increase in applicants for noncosmetic dermatological complaints. The most avoided cosmetic procedures were application of skin care devices, lasers, chemical peeling, and thread lifting, while botulinum toxin injection was the most performed procedure. Nearly half of the participants had severe financial damage. Of the participants, 55% reported that they worked anxiously during this period and 60% believed that they managed the early period of the pandemic successfully. CONCLUSION: Private dermatocosmetology clinics have to work in a totally different period that they have never experienced before. The pandemic has had serious impacts on both medical and socioeconomic issues which had to be managed carefully.

Tumor necrosis factor α-308 G/A and interleukin 1 ß-511 C/T gene polymorphisms in patients with scarring acne.

BACKGROUND: Acne is a chronic inflammatory skin disorder which may heal with scarring. Tumor necrosis factor alpha (TNF α) and interleukin 1 ß (IL-1ß) are considered as the main responsible proinflammatory mediators of acne pathogenesis. Oversecretion of these cytokines was found to be associated with TNF α-308 G>A and IL-1ß-511 C .05). CONCLUSION: TNF α-308 and IL-1ß polymorphic variants are not associated with acne and postacne scarring susceptibility and acne severity.

Plasma Thiol Levels are Associated with Disease Severity in Nonsegmental Vitiligo.

BACKGROUND: Vitiligo is a depigmenting cutaneous disorder with complex pathogenesis. Thiol compounds are well-known organic structures that play a major role in melanogenesis. AIM: The aim of this study was to determine the association between plasma thiol level and disease severity in patients with nonsegmental vitiligo. METHODS: A total of 73 patients with nonsegmental vitiligo (57 generalized and 16 localized type) and age- and sex-matched 69 healthy controls were enrolled in the study. Plasma levels of native thiols, disulfides, and total thiols were measured by a novel and automated assay. Disease severity of vitiligo was assessed with Vitiligo Area Scoring Index (VASI) score. The extent, stage, and spread of vitiligo of patients were evaluated according to the Vitiligo European Task Force (VETF) system. RESULTS: The native and total thiol levels of vitiligo patients were higher than those of healthy control group (P≤0.001 and 0.001, respectively). The median VASI score of patients was 0.7 (0.02-28.30). Univariate analyses showed that plasma native thiol levels, VETF spread score, disease duration, and vitiligo type significantly correlated with VASI scores (r=0.237, P=0.043; r=0.458, P<0.001; and P<0.001, respectively). Stepwise multivariate analysis revealed that disease duration (ß=0.017; P=0.005) and spread score (ß=1.301; P=0.001) were found statistically significant as independent factors on VASI score. CONCLUSION: Although plasma native thiol level significantly correlated with VASI scores of patients, it is not a predictive factor for vitiligo severity.

Outcomes of long term treatments of type I hereditary angioedema in a Turkish family.

BACKGROUND: Hereditary angioedema is a rare autosomal dominantly inherited immunodeficiency disorder characterized by potentially life-threatening angioedema attacks. OBJECTIVE: We aimed to investigate the clinical and genetic features of a family with angioedema attacks. METHODS: The medical history, clinical features and C1-INH gene mutation of a Turkish family were investigated and outcomes of long-term treatments were described. RESULTS: Five members had experienced recurrent swellings on the face and extremities triggered by trauma. They were all misdiagnosed as familial Mediterranean fever (FMF) depending on frequent abdominal pain and were on colchicine therapy for a long time. They had low C4 and C1-INH protein concentrations and functions. A mutation (c.1247T>A) in C1-INH gene was detected. They were diagnosed as having hereditary angioedema with C1-INH deficiency (C1-INH hereditary angioedema) for the first time. Three of them benefited from danazol treatment without any significant adverse events and one received weekly C1 esterase replacement treatment instead of danazol since she had a medical history of thromboembolic stroke. STUDY LIMITATIONS: Small sample size of participants. CONCLUSION: Patients with C1-INH hereditary angioedema may be misdiagnosed as having familial Mediterranean fever in regions where the disorder is endemic. Medical history, suspicion of hereditary angioedema and laboratory evaluations of patients and their family members lead the correct diagnoses of hereditary angioedema. Danazol and C1 replacement treatments provide significant reduction in hereditary angioedema attacks.

Outcomes of long term treatments of type I hereditary angioedema in a Turkish family

Abstract: Background: Hereditary angioedema is a rare autosomal dominantly inherited immunodeficiency disorder characterized by potentially life-threatening angioedema attacks. Objective: We aimed to investigate the clinical and genetic features of a family with angioedema attacks. Methods: The medical history, clinical features and C1-INH gene mutation of a Turkish family were investigated and outcomes of long-term treatments were described. Results: Five members had experienced recurrent swellings on the face and extremities triggered by trauma. They were all misdiagnosed as familial Mediterranean fever (FMF) depending on frequent abdominal pain and were on colchicine therapy for a long time. They had low C4 and C1-INH protein concentrations and functions. A mutation (c.1247T>A) in C1-INH gene was detected. They were diagnosed as having hereditary angioedema with C1-INH deficiency (C1-INH hereditary angioedema) for the first time. Three of them benefited from danazol treatment without any significant adverse events and one received weekly C1 esterase replacement treatment instead of danazol since she had a medical history of thromboembolic stroke. Study limitations: Small sample size of participants. Conclusion: Patients with C1-INH hereditary angioedema may be misdiagnosed as having familial Mediterranean fever in regions where the disorder is endemic. Medical history, suspicion of hereditary angioedema and laboratory evaluations of patients and their family members lead the correct diagnoses of hereditary angioedema. Danazol and C1 replacement treatments provide significant reduction in hereditary angioedema attacks.

Receptor for advanced glycation end products is overexpressed in psoriatic plaques independent of disease severity.

BACKGROUND: Enhanced expression and excitation of the receptor for advanced glycation end products is considered to play a role in the regulation of many pro-inflammatory genes involved in the pathogenesis of psoriasis. AIM: We investigated the expression of receptor for advanced glycation end product in various cell types, in lesional and peri-lesional skin of patients with psoriasis, and its correlation with disease severity. METHODS: Paraffin-embedded punch biopsy tissue taken from psoriatic plaques and peri-lesional normal appearing skin tissue of twenty patients with psoriasis, and normal skin samples of eleven healthy participants, were enrolled in the study. The sections were stained immunohistochemically with anti-receptor for advanced glycation end product antibody. The intensity of receptor for advanced glycation end product expression was assessed semi-quantitatively on epidermal cells, microvascular endothelium, dermal fibroblasts and inflammatory cells. They were graded as follows: 0 (no staining), 1 (weak), 2 (moderate) and 3 (strong) intensity. RESULTS: Receptor for advanced glycation end product expression on epidermis, microvascular endothelium, inflammatory cells and fibroblasts in the psoriatic plaques was more intense than perilesional and normal tissue (all P < 0.05). It did not correlate with disease severity. LIMITATIONS: The main limitation of our study is that this was a semi-quantitative assessment, detected immunohistochemically in skin biopsies. CONCLUSION: Receptor for advanced glycation end product expression may have an important role in psoriasis pathogenesis, independent of disease severity.