Real-Life Utility of Basophil Activation Test in the Diagnosis of Immediate Hypersensitivity Drug Reactions.

INTRODUCTION: The basophil activation test (BAT) is a flow cytometry laboratory technique that assesses the level of activation indicators expressed on the surface of basophils. We conducted a real-life study in a prospective cohort of patients with reported drug hypersensitivity reactions to determine the true relevance of BAT as a diagnostic tool for assessing immediate hypersensitivity reactions to medicines. METHODS: We prospectively assessed individuals with clinical suspicion of immediate hypersensitivity reactions to drugs over a 2-year period. The allergological evaluation was carried out in accordance with European Academy of Allergy and Clinical Immunology (EAACI) guidance. All patients underwent BAT using the activation marker CD63. RESULTS: In total 13 patients with 54 reported immediate drug hypersensitivity reactions to medications were included in this study. Twelve were female (92.3%) and one was male (7.70%). The mean ± SD age of the patients was 47.31 ± 19.94 years. Antibiotics were tested in 35.2% (19/54) of patients, corticosteroids in 24.1% (13/54), iodinated contrast medium in 14.8% (8/54), and NSAIDs in 5.6% (3/54). There was no correlation between the BAT results and the age of patients, gender, type of medication, or time interval between the allergic reaction and BAT procedure. The sensitivity of BAT 5% CD63+ basophils to drugs was 97.6%, specificity was 96% for drug allergies, positive predictive value (PPV) was 94.3%, and negative predictive value (NPV) was 95.2%. CONCLUSIONS: The sensitivity of BAT for drug allergies is limited, but it can nevertheless be very helpful before contemplating provocation testing in cases of life-threatening drug allergies where patients cannot be rechallenged or in cases of medications for which no other tests are available or their results are ambiguous.

Pruritogenic Mediators and New Antipruritic Drugs in Atopic Dermatitis.

Atopic dermatitis (AD) is a common highly pruritic chronic inflammatory skin disorder affecting 5-20% of children worldwide, while the prevalence in adults varies from 7 to 10%. Patients with AD experience intense pruritus that could lead to sleep disturbance and impaired quality of life. Here, we analyze the pathophysiology of itchiness in AD. We extensively review the histamine-dependent and histamine-independent pruritogens. Several receptors, substance P, secreted molecules, chemokines, and cytokines are involved as mediators in chronic itch. We also, summarize the new emerging antipruritic drugs in atopic dermatitis.

The IL-4/-13 Axis and Its Blocking in the Treatment of Atopic Dermatitis.

Atopic dermatitis (AD) is a common inflammatory skin disease with a complex pathophysiology, intertwining immune dysregulation, epidermal barrier dysfunction, IgE sensitization, environmental factors and genetic predisposition. It has been recently identified that interleukins -4 and -13 play crucial roles in the type-2-driven inflammation that characterizes AD, contributing to its symptomatology. Novel therapeutic approaches that target Th2 cytokines and their respective pathways have been developed, aiming to optimize the treatment of AD.

Awareness, knowledge, and attitudes towards sun protection among patients with melanoma and atypical mole syndrome.

BACKGROUND: Patients with atypical mole syndrome (AMS) have a 3- to 20-fold higher risk of developing malignant melanoma (MM) than individuals without. The most modifiable risk factor for developing MM is the ongoing ultraviolet exposure. AIM: To assess awareness, knowledge, and attitudes towards sun protection among patients with MM and AMS. METHODS: From January 2020 till December 2021, a written survey was administered to patients with MM and AMS and a control group who attended a specialist mole clinic at the Dermatology Department of the University Hospital of Heraklion in Heraklion, Crete, Greece. Demographic data and photoprotective practices, knowledge, and perceived barriers were collected. Relevant statistical analyses were performed using SPSS IBM 25. RESULTS: In total, 121 subjects consented and participated in the survey. Their mean age was 43.92 ± 12.55 years. There were 66 (54.4%) females and 55 (45.4%) males. Forty-seven (38.8%) patients had AMS, 26 (21.5%) had a past medical history of MM, and 48 (39.7%) attended the clinic for a full skin checkup for their naevi without having AMS or MM. Although 104 (86%) participants reported using sunscreen with the majority of them (59/121 = 48.8%) wearing sunscreen with a sun protection factor of > 50, only 22 (18.2%) patients did so every day and only 20 (16.5%) all year round. Approximately 74.4% of patients recalled having received advice on how to protect their skin from sunlight, and 73% were interested in receiving education about sun protection. The most mentioned barriers in photoprotection were concerns over adequate vitamin D and lack of time. CONCLUSION: Despite mentioning having received adequate education in photoprotection, adherence to photoprotection practices is suboptimal in patients with MM and AMS.

Clinical Characteristics of Patients with Erythema Nodosum and Risk of Relapse - a 17-Year Study.

Erythema nodosum (EN) is the most common type of septal panniculitis which causes inflammation of the subcutaneous fat, being the result of a hypersensitivity reaction to specific triggers. It usually presents with erythematous painful rounded lumps symmetrically on the anterior surface of the lower limbs. Rarely, it may occur in other areas such as thighs, neck and arms. This is a retrospective study describing a cohort of patients hospitalized in the University Hospital of Heraklion, Heraklion, Greece. The present research compares characteristics between patients with and without relapse and identifies independent factors associated with relapse. All patients with EN hospitalized during a 17-year period were included. Data regarding epidemiology, current or recent infections, symptoms, laboratory values and relapses were all recorded and evaluated. In total, 138 patients, of which 27 (19.6%) males, with a median age of 46.5 years, were evaluated. Clinical presentation involved multiple lesions in 115 (83.3%) patients, while 12 (8.7%) of them were febrile. Relapse was noted in 27 (19.6%) subjects. Multivariate logistic regression analysis showed that male gender was associated with a higher risk of relapse, while cases with multiple lesions were associated with a lower risk.

Merkel Cell Carcinoma-Update on Diagnosis, Management and Future Perspectives.

MCC is a rare but highly aggressive skin cancer. The identification of the driving role of Merkel cell polyomavirus (MCPyV) and ultraviolet-induced DNA damage in the oncogenesis of MCC allowed a better understanding of its biological behavior. The presence of MCPyV-specific T cells and lymphocytes exhibiting an 'exhausted' phenotype in the tumor microenvironment along with the high prevalence of immunosuppression among affected patients are strong indicators of the immunogenic properties of MCC. The use of immunotherapy has revolutionized the management of patients with advanced MCC with anti-PD-1/PD L1 blockade, providing objective responses in as much as 50-70% of cases when used in first-line treatment. However, acquired resistance or contraindication to immune checkpoint inhibitors can be an issue for a non-negligible number of patients and novel therapeutic strategies are warranted. This review will focus on current management guidelines for MCC and future therapeutic perspectives for advanced disease with an emphasis on molecular pathways, targeted therapies, and immune-based strategies. These new therapies alone or in combination with anti-PD-1/PD-L1 inhibitors could enhance immune responses against tumor cells and overcome acquired resistance to immunotherapy.

Apocrine and eccrine hidrocystomas: a clinicopathological study.

INTRODUCTION: Eccrine and apocrine hidrocystomas are uncommon, benign, cystic proliferations of the sweat glands usually located on the head and neck area. OBJECTIVES: To describe the key clinical and histopathological characteristics of a large series of hidrocystomas in Greece to improve diagnostic accuracy, and to perform a historical review of the medical term hidrocystoma. METHODS: A case series of 22 hidrocystomas from 20 consecutive patients treated with surgery at University Hospital of Heraklion in Crete, Greece, from January 1, 1998 to January 1, 2020 was performed along with a comprehensive historical literature review of the term hidrocystoma and its corresponding term hydatis from ancient Greek literature to the present. Data were obtained from medical records. All patients had a histopathologically confirmed diagnosis of hidrocystoma. Formalin-fixed paraffin-embedded (FFPE) sections of 22 tumors of the 20 consecutive patients were retrieved from the pathology laboratory archive and stained for SMA, p63, and GCDFP-15 with immunochemistry and periodic acid-Schiff (PAS) histochemical stain. RESULTS: Overall, 22 hidrocystomas (11 apocrine and 11 eccrine hidrocystomas) surgically excised from 20 patients were included in this study. Of the 20 patients, 10 (50%) were male and 10 (50%) were female, with a mean age of 56 ± 15 years. Hidrocystomas commonly occurred on the eyelids (73%), inner canthus (9%), eyebrow (4.5%), neck (4.5%), nose (4.5%), and ear (4.5%). All apocrine hidrocystomas stained positive for SMA, GCDFP-15, CAM 5.2, PAS, and PAS-D. No recurrence was observed. CONCLUSIONS: Here we have presented the clinicopathological characteristics of the largest case series of hidrocystomas in Europe and the Mediterranean region. Only apocrine hidrocystomas stained positive for SMA, GCDFP-15, CAM 5.2, PAS, and PAS-D.

Mucocutaneous manifestations in patients with inflammatory bowel disease: a decade study from a Greek cohort.

BACKGROUND: We sought to investigate the prevalence of mucocutaneous manifestations (MCM) and potential associations with clinical characteristics in Greek patients with IBD. METHODS: This was a retrospective observational single-center study. Patients with IBD diagnosis attending a tertiary referral hospital in Heraklion, Crete, from January 2010 to January 2020 were included. Data were extracted with relevant medical information from the IBD registry. Standard statistical tests, descriptive statistics tests, chi-square, Pearson correlation and multivariate analysis tests were performed, using IBM SPSS Statistics 25. RESULTS: A total of 806 IBD patients were included in the study: 463 (57.4%) males, 441 (54.7%) Crohn's Disease, 352 (43.7%) ulcerative colitis and 13 (1.6%) IBD unclassified (IBD-U). Mean age was 50.67 ± 17.67 years, mean age of IBD diagnosis 36.67 ± 16.53 years and mean disease duration 13.65 ± 9.89 years. The prevalence of MCM was 171/806 (21.2%), 9.65% in ulcerative colitis and 30.84% in CD. The presence of MCM was significantly correlated with younger age of IBD diagnosis, longer IBD duration, CD diagnosis, inflammatory behavior and ileal or ileocolonic location of CD, extensive colitis in ulcerative colitis, intestinal manifestations (EIMs) and treatment with immunosuppressant or anti-TNFa. The development of MCM was independently associated with the presence of other EIMs odds ratio (OR), 4.03 [95% confidence interval (CI), 2.60-6.24; P < 0.001] and treatment with immunosuppressant (OR, 1.87; 95% CI, 01.14-3.07; P = 0.013) or anti-TNFa (OR = 2.47; 95% CI, 1.59-3.84; P < 0.01). CONCLUSIONS: In our study, about one-fifth of IBD patients developed MCM that was more frequently present in CD than in ulcerative colitis.

Pyoderma gangrenosum associated with chronic refractory pouchitis: a case successfully treated with infliximab.

Pouchitis-associated pyoderma gangrenosum (PG) is rare, with only a few cases reported in the literature. Here we report a rare case of chronic refractory pouchitis-associated PG successfully treated with infliximab (IFX). A 43-year-old Caucasian male, with a past medical history of chronic refractory pouchitis after proctocolectomy and ileal pouch-anal anastomosis for severe ulcerative colitis, developed PG on his right lower leg. This subsided after treatment with intravenous IFX at a dose of 5 mg/kg at weeks 0, 2, 6 and then every 8 weeks. Pouchitis-associated PG is rare. Clinicians should be aware of the risk of PG in patients who suffer from pouchitis and develop rapidly extensive painful ulcers. Furthermore, the therapeutic choice should take into consideration the effectiveness of IFX on the inflammatory background, which sustains both intestinal and skin disease in these types of patients.

Pruritic annular and vesicular eruption on trunk and extremities.

We report a case of linear IgA bullous dermatosis, a rare autoimmune blistering disorder that usually presents with the abrupt onset of tense bullae. We also emphasize the importance of direct immunofluorescence for the definitive diagnosis.

Hydroxychloroquine-induced erythema multiforme.

Hydroxychloroquine is a commonly used medication and rarely may result in development of erythema multiforme. This potential cutaneous side effect should be highlighted in information given to patients prior to hydroxychloroquine commencement.