Unveiling Treatment Response Predictors in Predominant Subtypes of Chronic Inducible Urticaria.

INTRODUCTION: Chronic inducible urticaria (CIndU) is a subtype of chronic urticaria (CU), which requires specific triggers to occur. Despite their common occurrence, treatment response rates and predictors of treatment responses are largely lacking in the literature. This study evaluates antihistamine (AH) and omalizumab response rates in the most common CIndU subtypes and examines whether certain features can predict treatment responses. METHODS: We retrospectively analyzed CU patients with at least one CIndU subtype and performed comparisons between subgroups, in a total of 423 patients (70% CIndU, 30% chronic spontaneous urticaria [CSU] plus CIndU). RESULTS: The treatment response rates in CIndU were 51.6%, 51.5%, and 86.5% with standard-dose second-generation H1-antihistamines (sgAHs), updosed/combined sgAH, and omalizumab, respectively. Overall AH response was higher in CIndU than CSU plus CIndU (78.3% vs. 62%, p = 0.002) and in symptomatic dermographism (SD) and cold urticaria (ColdU) than cholinergic urticaria (ChoU) (83.2% vs. 78.3 vs. 60.9%, p = 0.04). AH-refractory patients had a longer disease duration (45.2 ± 56.7 months vs. 37 ± 51.9 months, p = 0.04), more angioedema, accompanying CSU, mixed CIndU subtypes (37.5% vs. 21.1%, p = 0.003; 45.1% vs. 27.1%, p = 0.002; 8.8% vs. 2.4%, p = 0.014), and lower baseline urticaria control test scores (5.86 ± 3.3 vs. 8.6 ± 3.6, p < 0.001) than AH-responsive patients. CONCLUSION: CIndU exhibits a good response to both AHs and omalizumab. Notably, the response to AHs is more pronounced in SD and ColdU compared to ChoU. Disease duration, angioedema, accompanying CSU, mixed CIndU, and lower baseline UCT scores may be used to predict AH treatment outcome in CIndU.

Adenoid cystic carcinoma located on the lower lip.

Adenoid cystic carcinoma is a relatively rare epithelial tumor of the major and minor salivary glands that makes up less than 1% of head and neck neoplasms. The typical clinical and pathological findings of this neoplasm include slow growth, perineural invasion, multiple local recurrences, and distant metastasis. Herein, we report a patient with adenoid cystic carcinoma located to the lower lip which is quite uncommon.

Fric Test Revisited: A Suggestion for a New Scoring System and Its Correlation with Urticaria Control Test and Dermatology Life Quality Index.

BACKGROUND: Fric test is a useful tool for the diagnosis and threshold testing for symptomatic dermographism. When threshold testing is not available, Urticaria Control Test (UCT) and Dermatology Life Quality Index (DLQI) might be used to assess disease control and quality of life (QoL) impairment, respectively. OBJECTIVES: In this study, we aimed to describe a new scoring system for the Fric test and evaluate the correlations of Fric scores with UCT, DLQI, and other disease activity assessment scores. METHOD: Provocation test with Fric Test 4.0 was performed in all patients at referral and at the 4th week. We considered a 4-grade rating score for Fric Test (0-4) [Total Fric Score (TFS)]. A positive response with all of the four pins suggested severe dermographism (TFS = 4), while a wheal with only the largest pin (4.5 mm) was considered as milder disease (TFS = 1). Treatment responses were evaluated with Fric Test 4.0, UCT, patient's global assessment of disease severity (PatGA-VAS), the physician's global assessment of disease control (PhyGA-VAS), and DLQI at baseline and at the 4th week of treatment. The correlations of TFS with UCT, DLQI, PatGA-VAS, PhyGA-VAS at baseline as well as the changes in the mean scores after treatment (week 4) were performed. RESULTS: The mean UCT and DLQI scores were 8.69 ± 3.40 and 7.88 ± 6.02 at the first visit. At the second visit, TFS decreased from a mean of 2.79 ± 1.68 to 1.91 ± 1.85 (p < 0.001), and UCT scores and PhyGA-VAS were increased (p < 0.001), while DLQI scores, PatGA-VAS, and pruritus scores decreased significantly (p = 0.002; p = 0.001; p = 0.012). There was a positive correlation between TFS and pruritus scores (r = 0.378) and DLQI scores (r = 0.392). TFS was found to have a negative correlation with UCT score (r = -0.283) and PhyGA-VAS (r = -0.347). CONCLUSIONS: This new Fric scoring system allows comparison with other tools and shows moderate correlations with most of the tools. Using disease-specific tools is recommended since they provide a subjective evaluation of disease severity, QoL impairment, and disease control.

Assessment of quality of life in Turkish children with psoriasis and their caregivers.

BACKGROUND/OBJECTIVES: The effect of pediatric psoriasis on quality of life has been demonstrated, but data regarding its influence on caregiver quality of life are scarce. The objective was to investigate how psoriasis affects quality of life of children and their caregivers. METHODS: This multicenter study included 129 children with psoriasis and their caregivers, who were family members accompanying patients to the clinic. Patient quality of life was measured using the Child Dermatology Life Quality Index. Caregiver quality of life was assessed using Dermatological Family Impact Scale, a 15-item questionnaire validated for use in the Turkish language. RESULTS: Mean Child Dermatology Life Quality Index score was 7.6, indicating a moderate effect on patient quality of life. Symptoms and feelings were the most severely impaired domains of patient quality of life, and emotions was the most severely impaired domain of caregiver quality of life. Dermatological Family Impact Scale score was significantly correlated with Child Dermatology Life Quality Index (correlation coefficient [r] = .554, P < .001) and Psoriasis Area and Severity Index (r = .350, P < .001). Caregivers of patients receiving systemic agents or phototherapy had relative impairment of multiple domains of quality of life compared to caregivers of patients receiving topical treatment only. CONCLUSION: Psychosocial effect of pediatric psoriasis was shown to extend beyond the individual, highlighting the importance of addressing patient and caregiver quality of life concerns in an integrated approach.

Pruritus in Systemic Diseases: A Review of Etiological Factors and New Treatment Modalities.

Pruritus is the most frequently described symptom in dermatology and can significantly impair the patient's quality of life. In 10-50% of adults with persistent pruritus, it can be an important dermatologic clue for the presence of a significant underlying systemic disease such as renal insufficiency, cholestasis, hematologic disorder, or malignancy (Etter and Myers, 2002; Zirwas and Seraly, 2001). This review describes the presence of pruritus in different systemic diseases. It is quite important to discover the cause of pruritus for providing relief for the patients experiencing substantial morbidity caused by this condition.

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.

Perianal rhabdomyosarcoma: report of a case in an infant and review of the literature.

Rhabdomyosarcoma of the perianal and perineal region is a rare sarcoma of childhood with a poor prognosis. The clinical findings are similar to those of condyloma accuminata, which makes it difficult to diagnose. There are a few reports of this disease in the dermatologic literature. We present a 15-month-old girl with a polypoid anal mass diagnosed as embryonal rhabdomyosarcoma and a review of the current literature.

Etiological factors and histopathological features in erythema nodosum: a 6-year retrospective cross-sectional study.

INTRODUCTION: Erythema nodosum (EN) is the most common type of panniculitis. The most frequent etiological factors are streptococcal pharyngitis, sarcoidosis, Behçet's disease, and tuberculosis. Our objective was to identify the etiological factors and to evaluate the patients' clinical, laboratory, and histopathological findings. METHODS: Eighty-eight patients diagnosed with EN at our clinic between 2013 and 2019 were evaluated retrospectively. Sixty-five patients were evaluated histopathologically. RESULTS: The patients' ages ranged between 17 and 76 (mean age: 41.91 ± 13.07 years). EN was 7.8 times more frequent in women. Patients presenting with idiopathic EN were significantly older than secondary cases (p < 0.05). Sixty-one patients (69.3%) had an underlying disease (secondary EN). The most common etiological factors were upper respiratory tract infections (n = 26), followed by Behçet's disease (n = 20). Septal panniculitis was present in 89.2% of cases evaluated histopathologically. Mixed or lobular panniculitis was present in 35.7% of Behçet's disease patients with EN-like lesions. Vasculitis was also noted in 35.7% of Behçet's disease patients. CONCLUSIONS: Our data confirm the predominance of upper respiratory tract infections and Behçet's disease among patients with EN in Turkey. Behçet's disease patients presenting with EN-like lesions may show mixed panniculitis and vasculitis, whereas classic EN patients predominantly show septal panniculitis.

Omalizumab as a Succesfull Therapy in Normocomplementemic Urticarial Vasculitis: A Series of Four Patients and Review of the Literature.

Urticarial vasculitis is an eruption characterized by inflamed itchy or painful red papules or plaques that resemble urticaria but last longer than 24 hours and heal with residual pigmentation or purpura. Histopathologically, urticarial vasculitis presents as leukocytoclastic vasculitis with perivascular infiltrate and fibrin deposits. The treatment options are oral antihistamines, oral corticosteroids, dapsone, colchicine and hydroxychloroquine. We report four cases with normocomplementemic urticarial vasculitis who were treated with omalizumab and a brief review of the literature on the use of omalizumab in normocomplementemic urticarial vasculitis.

Management of chronic inducible urticaria according to the guidelines: A prospective controlled study.

BACKGROUND: The recommended treatment approach in chronic inducible urticarias (CIndU) is the same as that for chronic spontaneous urticaria (CSU). But there is a lack of controlled trials assessing efficacy of available treatment options. OBJECTIVE: We aimed to evaluate the efficacy of treatment algorithm recommended by the guidelines and comparison of treatment responses in CIndU vs CSU. METHODS: This prospective parallel group controlled study included 70 CIndU and 66 CSU patients. The same treatment algorithm recommended by the European Academy of Allergology and Clinical Immunology/Global Allergy and Asthma European Network/European Dermatology Forum/World Allergy Organization (EAACI/GA2LEN/EDF/WAO) was implemented to both CSU and CIndU patients. Treatment responses of the groups were evaluated with urticaria control test (UCT) and dermatology life quality questionnaire (DLQI) scores at the 0, 4, 8, 12 and 24th weeks for CIndU and 0, 4, 12 and 24 weeks for CSU. RESULTS: Fourteen patients (20,9%) with CIndU and 25 (37,9%) with CSU responded to standard doses of H1-AHs which was significantly higher in the CSU group (p=0,031, p<0,05). Patients with CIndU and CSU showed statistically similar responses to 2nd line treatments (combining or updosing AHs) (p=0,979; p>0,05). Twenty-seven (40,3%) of CIndU patients and 21 (31,8%) of CSU patients were diagnosed as AH-resistant urticaria. Omalizumab was administered to 15 CSU patients and 17 CIndU patients. Response rates to omalizumab were similar in both groups. Total response rates increased from 37,9% (n:25) to 68,2% (n:45) with the 2nd line treatments in CSU group while it increased from 20,9% (n:14) to 59,7% (n:40) in CIndU group. When omalizumab was introduced to AH-refractory cases as a 3rd line treatment, total response rates evaluated at the 12th week were 76,1% (n:51) in patients with CIndU and 83,3% (n:55) in CSU. Continuing omalizumab treatment for 24 weeks increased response rates in patients who were unresponsive at week 12. CONCLUSION: CIndU seem to be more resistant to standard doses of AHs and higher doses of AHs are required for the control of symptoms. The same guidelines for CSU may be implemented to patients with CIndU.

Ichthyosis linearis circumflexa in a child. Response to narrowband UVB therapy.

BACKGROUND: Ichthyosis linearis circumflexa is a rare form of ichthyosis characterized by polycyclic and annular lesions which are bordered by a double-edged scale. Netherton syndrome is a genodermatosis in which ichthyosis linearis circumflexa is accompanied by characteristic hair shaft abnormalities and atopic diathesis. Different treatment modalities such as emmolients, keratolytics, calcipotriene, topical corticosteroids, topical calcineurin inhibitors, systemic retinoids, phototherapy and photochemotherapy have been used with variable results. Topical therapies may result in considerable absorption because of the defective barrier function in ichthyosis linearis circumflexa/Netherton syndrome. MAIN OBSERVATION: A 12-year-old patient diagnosed as ichthyosis linearis circumflexa demonstrated considerable improvement with narrowband UVB phototherapy at the end of 30 sessions. CONCLUSION: Short-term narrowband-UVB may an effective treatment option in patients with ichthyosis linearis circumflexa/Netherton syndrome.