The association of bullous pemphigoid with dipeptidyl-peptidase 4 inhibitors: a ten-year prospective observational study.

BACKGROUND: Bullous pemphigoid is the most common bullous chronic autoimmune skin disease. Recent studies have suggested dipeptidyl-peptidase 4 inhibitors as possible predisposing agents of bullous pemphigoid. The objective of our study was to prospectively estimate the association between gliptins and the development of bullous pemphigoid. METHODS: We conducted a prospective study which included all patients diagnosed with biopsy-proven bullous pemphigoid in the Dermatology Department of our hospital between April 1, 2009 and December 31,2019. The diagnosis of bullous pemphigoid was based on specific clinical, histological and immunological features. RESULTS: Overall 113 consecutive patients (age 75 ± 13 years, 62 females) with the diagnosis of bullous pemphigoid were enrolled. Seventy-six patients (67.3%) suffered from type 2 Diabetes and 52 (46%) were treated with dipeptidyl-peptidase 4 inhibitors. The most frequent prescribed gliptin was vildagliptin, being administered to 45 cases (39.8% of total patients enrolled, 86.5% of the patients treated with gliptins). Gliptins were withdrawn immediately after the diagnosis of bullous pemphigoid, which together with steroid administration led to remission of the rash. CONCLUSIONS: This study revealed that treatment with dipeptidyl-peptidase 4 inhibitors, especially vildagliptin, is significantly associated with an increased risk of bullous pemphigoid development.

Use of Dermoscopy among Greek Dermatologists in Everyday Clinical Practice: A National Questionnaire-Based Study.

Background: Dermatoscopy has been established as an important diagnostic tool for a wide range of skin diseases. This study aims to evaluate the use of dermatoscopy in clinical practice among Greek dermatologists. Methods: A nationwide questionnaire-based survey was conducted collecting data on the frequency of dermatoscopic examinations, the types of lesions examined, training and educational resources, as well as factors influencing the choice to incorporate dermatoscopy into daily clinical routines. Results: A total of 366 Greek dermatologists participated in the survey. Most of the respondents reported the daily use of dermatoscopy in their practice. Pigmented and non-pigmented lesions, inflammatory diseases, cutaneous infectious, hair disorders, and nail lesions were the most common indications for dermatoscopy. Factors influencing the utilization of dermatoscopy included increased diagnostic accuracy, enhanced patient care, better patient communication and general compliance, and improved satisfaction among dermatologists. Conclusions: This national questionnaire-based study demonstrates that dermatoscopy has become an integral part of daily dermatological practice in Greece. The findings highlight the significance of structured training and education to promote dermoscopy's effective and routine use. Incorporating dermatoscopy into clinical practice not only improves diagnostic precision but also enhances patient care, contributing to the overall quality of dermatological services in Greece.

Diffuse panniculitis in a teenage male with ZZ α1-antitrypsin deficiency.

Diffuse panniculitis is a rare manifestation of α1-ATD, albeit perhaps the most fulminant and life-threatening complication, associated usually with ZZ phenotype. Intravenous α1-AT treatment is lifesaving. https://bit.ly/3EDmCzT.

Diagnosis and Differential Diagnosis of Poikiloderma of Civatte: A Dermoscopy Cohort Study.

INTRODUCTION: Poikiloderma of Civatte (PC) is a common, acquired, chronic, benign poikiloderma of the neck and face, most commonly affecting peri-menopausal females. At the time of writing, few studies have been published regarding the dermoscopy of PC. OBJECTIVE: To describe the dermoscopic picture of PC, so as to provide a clinico dermoscopic diagnosis and differential diagnosis for PC. METHODS: Twenty-eight patients with PC, aged 26-73 years, of whom 19 females (67.86%) were evaluated by detailed history, clinical examination, and dermoscopic examination with hand-held dermoscope. RESULTS: The reticular pattern was observed in 15 cases (53.6%); the white dot in 10 (35.7%); the non-specific in 9 (32.1%); and the combination of linear and dotted vessels in 8 (28.6%). Regarding local dermoscopic features, converging curved vessels were observed in 18 cases (64.3%); linear irregular vessels in 17 (60.7%); rhomboidal/polygonal vessels in 15 (53.6%); dotted/globular vessels in 10 (35.7%); white macules in 23 (82.1%); brown macules in 11 (39.3%); and whitish follicular plugs in 6 (21.4%). CONCLUSIONS: The dermoscopic picture of PC is highly characteristic and corresponds well to both clinical and histological findings. Dermoscopy may assist clinical diagnosis, as well as the differentiation from other dermatoses of the neck and face, especially poikilodermas with guarded prognosis.

Real-world data show high efficacy of IL23 inhibitors guselkumab and risankizumab in psoriatic arthritis and difficult-to-treat areas.

BACKGROUND: Psoriasis and psoriatic arthritis are chronic inflammatory skin and joint diseases requiring effective therapies. Although clinical studies have shown the efficacy of IL-23 inhibitors, real-world data are limited. METHODS: We conducted a single-center retrospective Greek study enrolling patients with psoriatic arthritis and moderate-to-severe plaque psoriasis being treated at our multidisciplinary psoriasis outpatient clinic. Our aim was to investigate the efficacy and safety of IL-23 inhibitors guselkumab and risankizumab. Additionally, we sought to determine the clinical characteristics affecting treatment response. Primary endpoints were the evaluation of absolute Psoriasis Area and Severity Index (aPASI) and Disease Activity Index for Psoriatic Arthritis (DAPSA) at week 24. RESULTS: Fifty-nine patients (55.9% male, 69.5% early onset) with a mean age of 51.7 years were included. Twenty-four patients (40.7%) had a concomitant psoriatic arthritis. Obesity was the main comorbidity (49.2%) with a mean body mass index (BMI) of 31.3 kg/m2 . Additional comorbidities were hypertension (44.1%), dyslipidemia (32.2%), and diabetes (18.6%). Only eight patients (13.6%) were naïve to previous systemic treatments, whereas 40 patients (67.8%) were bio-experienced. A statistically significant improvement of aPASI and DAPSA was demonstrated after 4, 16, and 24 weeks of treatment (P < 0.05). IL23 blockers were also efficacious in difficult-to-treat areas. Clinical outcome was affected from previous treatment with biologics. Treatment response was the same between guselkumab and risankizumab (P > 0.05). CONCLUSION: This real-world study confirms the efficacy and safety of guselkumab and risankizumab in psoriatic arthritis and psoriasis reported from clinical trials.

Actinic Keratoses (AK): An Exploratory Questionnaire-Based Study of Patients' Illness Perceptions.

BACKGROUND: Decreased illness perception among actinic keratoses (AK) patients is a major barrier to the effective management of AK. OBJECTIVE: We aimed to investigate patients' illness and treatment perceptions, their correlation to demographics and AK/skin cancer history, and secondarily the influence of these perspectives on treatment and sunscreen use. MATERIALS AND METHODS: Participants completed questionnaires based on the Brief Illness Perception Questionnaire and statistical analysis was performed. RESULTS: In total, 208 AK patients were enrolled. A large proportion were poorly aware of the disease (41.4%), with less than half (43%) being familiar with AK. Patients were aware of the chronic nature of the disease and its correlation to sunlight regardless of demographic characteristics. The level of education played a role in disease awareness (p = 0.006), and treatment plan perception (p = 0.002). The increase in sunscreen protection after AK diagnosis was higher in women (p = 0.009) and younger patients (p = 0.044). Patients' concerns regarding treatment were mainly related to the duration (30%) and effectivity (25%). Dermatologists' statements highlighting that AK are precancerous lesions (86.2%) influenced patients' willingness for treatment. CONCLUSION: Improved awareness of AK is necessary to increase treatment seeking and compliance, regarding both treatment and sunscreen use. Dermatologists' statements may have critical influence on patients' decisions to receive treatment for AK.

Real world evidence: Patients with refractory pemphigus treated with Rituximab.

BACKGROUND: Pemphigus is a group of autoimmune blistering diseases, potentially life-threatening. Rituximab received FDA approval in June 2018 for the treatment of moderate to severe pemphigus vulgaris. OBJECTIVES: To evaluate the efficacy and safety of rituximab in patients with pemphigus, resistant to previous therapies or unable to receive classic immunosuppressive treatment due to serious adverse events or comorbidities. MATERIALS AND METHODS: Twenty-five patients (9 men, 16 women), mean age 49.4 ± 15.9 years (range 21-74 years), mean disease duration 4 ± 2.7 years (range 0.25-10 years) were included in the study: 19 patients with pemphigus vulgaris and 6 with pemphigus foliaceous. The efficacy of rituximab was evaluated according to the control of disease, retention of remission, disease severity, previous treatments and adverse reactions. During COVID-19 pandemic patients are monitored closely through tele-dermatology. RESULTS: Twenty-three out of 25 patients had great improvement, 2 out of 25 ceased therapy due to adverse events (arthralgias and dyspnea). Sixteen out of 23 received additional course after 8 months (range 5-60 months). More aged patients presented more frequently adverse events and underwent additional courses (p = 0.002). Rituximab was found superior to classic immunosuppressive treatment in terms of efficacy and safety, with larger periods of remission and lower doses of corticosteroids and immunosuppressants. No major adverse events were noticed. CONCLUSIONS: Rituximab is a very effective treatment of pemphigus and, remarkably, superior to classic immunosuppressive treatment.

Female Patient with Two Simultaneous Giants Facial Basal Cell Carcinomas Demonstrates a Positive Response to Vismodegib as a Monotherapy.

Basal cell carcinoma (BCC) is the most frequent histological type of cancer in the world and accounts for approximately 80% of all skin cancers. In the majority of cases, they are slow-growing, low metastatic potential tumors, easy to cure by surgical or nonsurgical procedures. Giant BCC (GBCC) is a rare variant of BCC and according to the American Joint Committee on Cancer, this includes lesions with a diameter larger than 5 cm. GBCC's incidence has been reported to be less than 1%, and it displays a more aggressive behavior with both local invasion and higher metastatic potential. Archodaki et al. specifically reported that metastasis was present in 17.6% of GBCC patients during the primary examination. Patients with GBCC who are not suitable for either surgery or radiotherapy since 2012 seem to have another therapeutic option. Vismodegib is an oral small-molecule inhibitor of the Hedgehog pathway (HPI) that was approved for treating metastatic or locally advanced BCC in patients who are poor candidates for surgery or radiotherapy. In this case, we present a woman with two simultaneous facial GBCCs who was treated successfully using vismodegib as a monotherapy.

Dermoscopy as a Tool in Differentiating Cutaneous Squamous Cell Carcinoma From Its Variants.

BACKGROUND: Dermoscopic features of cutaneous squamous cell carcinoma (cSCC) have been widely studied, but their accuracy should be further investigated. OBJECTIVES: This study assessed the diagnostic accuracy of a set of predetermined dermoscopic structures for 3 variants of cSCC, namely Bowen disease, keratoacanthoma and invasive cSCC. METHODS: Dermoscopic images of 56 histopathologically confirmed cSCC lesions (9 Bowen disease lesions, 7 keratoacanthomas, and 40 invasive cSCCs) were examined, and the diagnostic accuracy of dermoscopic structures was assessed. Discriminative ability of statistically significant positive predictors was determined using receiver operating characteristic (ROC) curves, and defined as an area under the ROC curve >0.700. RESULTS: Dermoscopic structures with statistical significance and discriminative ability were: for Bowen disease, clustered glomerular vessels and erosions; for keratoacanthoma, a central keratin plug; and for invasive cSCC, a mixed color of the background. Clustered and glomerular vessels had, for Bowen disease, perfect diagnostic accuracy, with: sensitivity of 88.9% for both features; specificity of 97.9% and 93.6%, respectively; positive predictive value (PPV) of 88.9% and 72.7%, respectively; and negative predictive value (NPV) of 97.8% for both. Erosions had, for BD, high specificity (87.2%) and NPV (91.1%), but low sensitivity (55.6%) and PPV (45.5%). A central keratin plug had, for keratoacanthoma, high specificity (87.8%) and NPV (93.5%), but low sensitivity (57.1%) and PPV (40%). A mixed background color had, for invasive cSCC, high specificity (81.3%) and PPV (89.7%), but low sensitivity (65%) and NPV (48.2%). CONCLUSION: Dermoscopy accurately differentiates BD, through clustered glomerular vessels, from keratoacanthoma and invasive cSCC. Dermoscopic structures of keratoacanthoma and invasive cSCC overlap, and only histopathologic analysis differentiates them precisely.

Novel Insights for Patients with Multiple Basal Cell Carcinomas and Tumors at High-Risk for Recurrence: Risk Factors, Clinical Morphology, and Dermatoscopy.

INTRODUCTION: Basal cell carcinoma (BCC) quite frequently presents as multiple tumors in individual patients. Neoplasm's risk factors for local recurrence have a critical impact on therapeutic management. OBJECTIVE: To detect risk factors for multiple BCCs (mBCC) in individual patients and to describe clinical and dermatoscopic features of low- and high-risk tumors. MATERIALS & METHODS: Our study included 225 patients with 304 surgically excised primary BCCs. All patients' medical history and demographics were recorded. Clinical and dermatoscopic images of BCCs were evaluated for predefined criteria and statistical analyses were performed. RESULTS: Grade II-III sunburns before adulthood (OR 2.146, p = 0.031) and a personal history of BCC (OR 3.403, p < 0.001) were the major predisposing factors for mBCC. Clinically obvious white color (OR 3.168, p < 0.001) and dermatoscopic detection of white shiny lines (OR 2.085, p = 0.025) represented strongly prognostic variables of high-risk BCC. Similarly, extensive clinico-dermatoscopic ulceration (up to 9.2-fold) and nodular morphology (3.6-fold) raise the possibility for high-risk BCC. On the contrary, dermatoscopic evidence of blue-black coloration had a negative prognostic value for high-risk neoplasms (light OR 0.269, p < 0.001/partial OR 0.198, p = 0.001). CONCLUSIONS: Profiling of mBCC patients and a thorough knowledge of high-risk tumors' clinico-dermatoscopic morphology could provide physicians with important information towards prevention of this neoplasm.