Monitoring recurrent angioedema: Findings from the Turkish angioedema control test validation study.

BACKGROUND: Determination of control level in recurrent angioedema (RAE) is necessary to guide management. Here, we validated a Turkish version of the angioedema control test (AECT) for 4-week (AECT-4wk) and for 3-month (AECT-3mth) and assessed their utility in monitoring RAE. METHOD: The recommended structured translation process for patient-reported outcome measures was completed. The final versions were administered to 51 patients with mast cell-mediated angioedema (MMAE) and 38 patients with hereditary angioedema, and the minimal clinically important difference (MCID) was determined. Additionally, anchor surveys comprising angioedema activity score for 28 days (AAS-28 day), visual analog score for angioedema control, Likert scale for the control level from the patient's perspective (LS-AEC), angioedema quality of life, short form-12 (SF-12) and patients' assessment of treatment sufficiency were applied. RESULTS: The Turkish AECT versions showed good convergent validity with a substantial correlation with anchor tools and known-group validity. Excellent internal consistency and reproducibility were observed. Equal or more than 10 of 16 points scored with the AECT-4wk and AECT-3mth identified patients with well-controlled disease. The disease activity, control and burden parameters were consistent with the disease control level defined depending on the cut-off point 10 of AECT. Three-point changes in AECT-4wk and -3 mt could detect MCID in disease control in all patients. CONCLUSIONS: Turkish AECT versions are valid and reliable tools for assessing and monitoring disease control in patients with RAE. The use of the Turkish versions of the AECT in routine patient care, clinical trials and angioedema research is recommended.

Systemic inflammation and its relationship with pruritus in early-stage mycosis fungoides.

The underlying mechanisms mycosis fungoides (MF)-related pruritus remain unclear, and the link between pruritus and systemic inflammation in MF is unexplored. We aimed to investigate systemic inflammation in MF patients and its potential connection to pruritus. In this retrospective study, demographic characteristics, MF stage, clinical and laboratory findings, and neutrophil-lymphocyte ratio (NLR), platelet-lymphocyte ratio (PLR), monocyte-lymphocyte ratio (MLR), systemic immune-inflammation index (SII), systemic inflammation response index (SIRI) and pan-immune inflammation value (PIV) were assessed for all participants. Additionally, mSWAT scores, Dermatology Life Quality Index (DLQI), and pruritus presence and intensity via Visual Analogue Scale (VAS) scoring were recorded for MF patients. A total of 81 patients with early-stage MF and 50 controls were enrolled. Itching was present in 41 patients (50.6%). NLR, PLR, SII, SIRI and CRP values in the MF group were significantly higher. CRP, NLR, mSWAT and DLQI score were significantly higher in MF patients with pruritus than those without. Pruritus was positively correlated with DLQI, mSWAT, CRP, NLR, MLR and SIRI. VAS score was positively correlated with eosinophil count and DLQI. In the multivariate logistic regression model, only NLR was an independent and significant associate of pruritus in patients with MF. This study provides evidence of enhanced systemic inflammation in early-stage MF patients. Additionally, the correlation between pruritus with mSWAT scores and systemic inflammation parameters suggests a potential link between pruritus and the inflammatory milieu in MF.

CRUSE® -An innovative mobile application for patient monitoring and management in chronic spontaneous urticaria.

BACKGROUND: Chronic spontaneous urticaria (CSU) is unpredictable and can severely impair patients' quality of life. Patients with CSU need a convenient, user-friendly platform to complete patient-reported outcome measures (PROMs) on their mobile devices. CRUSE® , the Chronic Urticaria Self Evaluation app, aims to address this unmet need. METHODS: CRUSE® was developed by an international steering committee of urticaria specialists. Priorities for the app based on recent findings in CSU were defined to allow patients to track and record their symptoms and medication use over time and send photographs. The CRUSE® app collects patient data such as age, sex, disease onset, triggers, medication, and CSU characteristics that can be sent securely to physicians, providing real-time insights. Additionally, CRUSE® contains PROMs to assess disease activity and control, which are individualised to patient profiles and clinical manifestations. RESULTS: CRUSE® was launched in Germany in March 2022 and is now available for free in 17 countries. It is adapted to the local language and displays a country-specific list of available urticaria medications. English and Ukrainian versions are available worldwide. From July 2022 to June 2023, 25,710 observations were documented by 2540 users; 72.7% were females, with a mean age of 39.6 years. At baseline, 93.7% and 51.3% of users had wheals and angioedema, respectively. Second-generation antihistamines were used in 74.0% of days. CONCLUSIONS: The initial data from CRUSE® show the wide use and utility of effectively tracking patients' disease activity and control, paving the way for personalised CSU management.

The effect of COVID-19 on development of hair and nail disorders: a Turkish multicenter, controlled study.

BACKGROUND: A broad spectrum of skin diseases, including hair and nails, can be directly or indirectly triggered by COVID-19. It is aimed to examine the type and frequency of hair and nail disorders after COVID-19 infection. METHODS: This is a multicenter study conducted on consecutive 2171 post-COVID-19 patients. Patients who developed hair and nail disorders and did not develop hair and nail disorders were recruited as subject and control groups. The type and frequency of hair and nail disorders were examined. RESULTS: The rate of the previous admission in hospital due to COVID-19 was statistically significantly more common in patients who developed hair loss after getting infected with COVID-19 (P < 0.001). Telogen effluvium (85%) was the most common hair loss type followed by worsening of androgenetic alopecia (7%) after COVID-19 infection. The mean stress scores during and after getting infected with COVID-19 were 6.88 ± 2.77 and 3.64 ± 3.04, respectively, in the hair loss group and were 5.77 ± 3.18 and 2.81 ± 2.84, respectively, in the control group (P < 0.001, P < 0.001). The frequency of recurrent COVID-19 was statistically significantly higher in men with severe androgenetic alopecia (Grades 4-7 HNS) (P = 0.012; Odds ratio: 2.931 [1.222-7.027]). The most common nail disorders were leukonychia, onycholysis, Beau's lines, onychomadesis, and onychoschisis, respectively. The symptoms of COVID-19 were statistically significantly more common in patients having nail disorders after getting infected with COVID-19 when compared to the control group (P < 0.05). CONCLUSION: The development of both nail and hair disorders after COVID-19 seems to be related to a history of severe COVID-19.

Omalizumab treatment for chronic spontaneous urticaria: data from Turkey.

Aim: The aim of the study was to investigate data from patients suffering from chronic spontaneous urticaria refractory to conventional therapy, and to document outcomes of omalizumab use. Material and methods: We conducted a single-centre retrospective study with 175 chronic spontaneous urticaria patients who were treated with 300 mg omalizumab subcutaneously every 4 weeks for at least 6 months. Efficacy, factors affecting outcome, and complications were examined. Results: The complete response rate was 70.9%. Minor complications were observed in 12% of our patients. Anaphylaxis occurred in 1 patient as a major complication. We did not notice any clinical or laboratory factors predicting response to omalizumab treatment. Conclusions: The findings show that omalizumab is effective and safe for the treatment of chronic spontaneous urticaria with a dosing of 300 mg/month subcutaneously. However, due to 1 case of anaphylaxis in this small group, we must still remind practitioners to be alert for this possible complication.

New markers to predict the response to omalizumab in chronic spontaneous urticaria.

Omalizumab has high treatment efficacy in patients with chronic spontaneous urticaria (CSU) who do not respond to high doses of antihistamines. Systemic immune-inflammation index (SII) and systemic inflammation response index (SIRI) were described as novel inflammatory and prognostic biomarkers. The present study aimed to evaluate the effectiveness of SII and SIRI in patients with CSU who receive omalizumab therapy. A total of 124 patients with severe urticaria who had an urticaria activity score over 7 days (UAS-7) ≥28 were included in the study. UAS-7, C-reactive protein (CRP), SII, and SIRI values ​​were recorded before and after omalizumab treatment. Patients with UAS-7 ≤6 at week 12 and/or week 24 of omalizumab treatment were considered responders. Three months after omalizumab treatment, significant decreases were observed in SII, SIRI, CRP, and UAS-7 compared to pre-treatment values ​​(p = 0.003, p < 0.001, p = 0.006, and p < 0.001, respectively). At the third and sixth months of treatment, baseline SII and SIRI levels of the omalizumab responder group were significantly higher than the non-responder group (p < 0.001). However, there was no difference in baseline CRP and UAS-7 levels between responders and non-responders (p Ëƒ 0.05). After adjusting for confounding factors, only pre-treatment SII (OR: 1.002, 95% CI: 1.000-1.004, p = 0.036) and SIRI (OR: 4.334, 95% CI: 1.751-10.726, p = 0.002) values were independently associated with response to omalizumab at 3 months in multivariate regression analysis. SII and SIRI could be effectively used to predict the response to omalizumab therapy. More comprehensive studies are needed to validate and elaborate on this relationship.

Evaluation of the Effect of Diclofenac Sodium and 5-Fluourasil in a 3D Cholesteatoma Cell Culture Model.

INTRODUCTION: Middle ear cholesteatoma is a benign disease with invasive and destructive clinical behaviors. It increases the rate of both chronic otitis media complications and revision surgeries. The most effective treatment of middle ear cholesteatoma is surgical excision, and there is no medical treatment for this disease. Exploring new medical treatment options may help to create treatment alternatives instead of surgery. MATERIALS AND METHODS: Required cholesteatoma tissues for cell culture were excised from 4 different participants who underwent surgery in our clinic and agreed to give tissue for the study. Cholesteatoma-derived keratinocytes and fibroblasts were cocultured in temperature-sensitive culture dishes to make a three-dimensional (3D) cholesteatoma model. Then, the effects of 1% and 2% diclofenac sodium on viability and cell proliferation rates were examined using WST-1 and annexin-V tests. RESULTS: Cell viability and proliferation rates were found to be lower and apoptosis rates were higher in the diclofenac sodium group versus the negative and positive control groups. CONCLUSION: In this present study, we described a new 3D cholesteatoma cell culture model developed using cell sheet technology and demonstrated the efficacy of diclofenac sodium on cholesteatoma for the first time in the literature. It may be used in patients with chronic otitis media with cholesteatoma, but further studies investigating ototoxic and neurotoxic effects of this molecule are needed.

The microvascular and morphostructural changes of nails in psoriatic patients with nail disease; a link between ultrasound and videocapillaroscopy findings in the nailfold.

OBJECTIVE: The objective of this study is to evaluate the link between nail fold vessel resistive index (NVRI) measured by ultrasound (US) and capillary loops diameters measured using nailfold videocapillarascopy (NVC), and to assess the morphological appearance of the nail bed in patients with psoriatic nail disease (PND) as compared with healthy controls (HCs). MATERIAL AND METHODS: This study was conducted in patients with PND and HCs. General demographic data were collected and clinical assessments were performed for all subjects. The nail plate thickness (NPT) was measured on gray scale using US. The NVRI was measured using color Doppler (CD) US. The measurements of the apical, arterial, venous limb diameters and morpho-structural changes (tortuous, cross-linked capillaries) were assessed using NVC. RESULTS: Thirty-four patients with PND and 15 HCs were enrolled in this study. The two groups were matched for age and body mass index (BMI). Patients with PND had higher NPT and NVRI in comparison with HCs [(20 (17-23) vs 14 (14-15), p<0.001), (0.55 (0.51-0.61) vs 0.43 (0.38-0.49), p<0.001), respectively]. A higher proportion of patients with PND had tortuous capillaries than HCs (62% and 20% respectively, p=0.005). The mean NVRI was higher in patients with PND who had tortuous capillaries than patients who did not have tortuous capillaries (0.58 (0.7) and 0.52 (0.09), respectively p=0.033). CONCLUSION: Microvascular changes can be detected easily using non-invasive methods such as US and NVC. These methods can provide an objective data to better assess PND.

Comparison of Famciclovir, Valaciclovir, and Brivudine Treatments in Adult Immunocompetent Patients With Herpes Zoster.

Herpes zoster (HZ) is a common disease characterized by the recurrence of varicella zoster, that stays dormant in sensory ganglia. The primary goal of this study was to compare efficiencies of famciclovir, valaciclovir, and brivudine in terms of pain relief in HZ patients. Records of patients who were admitted to the Dermatology Clinic of our hospital due to acute HZ between the years 2012 and 2014 were retrospectively analyzed. Treatment decisions were at the discretion of caring physicians as valaciclovir (VACV), famciclovir (FCV), and brivudine (BRV) based on the clinical observations. BRV, FCV, and VACV were effective in treating pain in acute HZ. There was no significant difference between mild and moderate HZ patients. In severe cases, a significant reduction in intensity of pain was observed on day 3 in the BRV group, on day 7 in the FCV group, and at 2-3 weeks in the VACV group. There were no significant side effects observed in any of the groups. Results of this study indicate that brivudine may be the first choice in severe HZ cases as it controls pain earlier and is easier to use because of its once daily administration.

Milia-like calcinosis cutis in a girl with Down syndrome.

Milia-like idiopathic calcinosis cutis (MICC) is a very rare dermatological disorder characterized by multiple whitish to skin colored, milia-like papules, mostly found on the hands. MICC can disappear spontaneously by adulthood; therefore, its early recognition is crucial to avoiding unnecessary interventions. Herein, we present a case of MICC in a 6-year-old girl with Down syndrome.

Milia-like calcinosis cutis in a girl with Down syndrome

Abstract: Milia-like idiopathic calcinosis cutis (MICC) is a very rare dermatological disorder characterized by multiple whitish to skin colored, milia-like papules, mostly found on the hands. MICC can disappear spontaneously by adulthood; therefore, its early recognition is crucial to avoiding unnecessary interventions. Herein, we present a case of MICC in a 6-year-old girl with Down syndrome.

DRESS syndrome potentially induced by allopurinol and triggered by influenza vaccine.

Drug Reaction with Eosinophilia and Systemic Symptoms (DRESS) syndrome, also known as drug-induced hypersensitivity syndrome (DHIS), is an acute, potentially life-threatening disease that includes skin rash, fever, haematological abnormalities and multiorgan involvement. Although its aetiopathogenesis is not exactly known, it is thought that inefficient drug detoxification leading to the accumulation of drug reactive metabolites causes autoimmune responses in skin and some internal organs, alters immune responses and induces reactivation of viral infections in people who have genetic predisposition. To the best of our knowledge, only one case of DRESS syndrome has been reported after delivery of the influenza vaccine, but the drug that induced the reaction in that case was sulfasalazine. We report a case of a 64-year-old woman, receiving allopurinol, who developed DRESS syndrome after taking the influenza vaccine.

Palmoplantar lichen planus successfully treated with acitretin.

Palmoplantar lichen planus (PPL) is an uncommon type of lichen planus (LP) that exclusively affects the palms and soles. We report a case of a 50-year-old man who had palmoplantar hyperkeratotic papules and plaques. The patient was diagnosed as a case of PPL by skin biopsy, and treated with acitretin. He showed a good response to acitretin within 2 months. Clinical appearance and some features of PPL may differ from classic LP. Acitretin may be a favourable treatment option for PPL.

First and only symptom of undiagnosed diabetes mellitus: eruptive xanthoma.

Eruptive xanthoma (EX) is a very rare dermatosis mostly occurring due to high levels of serum triglycerides or uncontrolled diabetes mellitus. When EX is encountered, it is important to keep in mind that it could be a sign of severe underlying metabolic derangements. Early recognition can help avoid serious complications such as pancreatitis. After treatment of the underlying metabolic disorders, lesions mostly disappear without leaving scars. We present a case of a 55-year-old woman who presented with solely EX lesions and who was eventually diagnosed with diabetes mellitus and severe hypertriglyceridaemia.

A case of prolidase deficiency accompanying leg ulcers.

Prolidase deficiency (PD) is a rare autosomal recessive disorder that has symptoms such as skin ulcers, characteristic facies, mental retardation, skeletal deformities, hematological anomalies, splenomegaly, and chronic infections. Deficiency of prolidase leads to the increased excretion of proline in urine, which causes impaired collagen synthesis and delay in wound healing. This case reports a 40-year-old female who has had cutaneous ulcers since the age of 7 years. We also recognized borderline intellectual functioning as well as hematologic abnormalities and splenomegaly. We present this rare case to draw attention to consider prolidase deficiency in the differential diagnosis of leg ulcers.

Diabetic fibrous mastopathy.

Diabetic fibrous mastopathy is an uncommon self-limiting fibroinflammatory diseae of the breast that is seen predominantly in premenopausal women with long standing type I (insulin dependent) diabetes mellitus. In this report, we present a 29 years old female with uncontrolled diabetes mellitus presenting with bilateral breast masses which were irregular and hypoechoic on ultrasound, gradual enhancement on MRI and diagnosed as diabetic fibrous mastopathy on histopathology. It is quite difficult to distinguish it from malignancy on mammographic and ultrasonographic features or clinical findings. Correlation of the pathological features may help to make the correct diagnosis for this disease.

Mesenteric panniculitis: a case report and review of the literature.

Mesenteric panniculitis is a benign disease characterized chronic nonspecific inflammation of the mesentery adipose tissue of the small intestine and colon. The specific etiology of the disease is unknown. The diagnosis is suggested by computed tomography (CT) and is usually confirmed by surgical biopsies. In recent years, abdominal CT is used as an effective method for diagnostic evaluation.We reported a case of the mesenteric panniculitis diagnosed with CT and operated for acute pancreatitis and cholecystitis five years ago.