Evaluation of the Factors Influencing Mortality in Patients with Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis: A Multicenter Study of 166 Patients.

INTRODUCTION: Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are life-threatening acute mucocutaneous disorders usually triggered by drugs. In this study, we aimed to evaluate the factors affecting mortality in patients with SJS-TEN. METHODS: Our study is a retrospective cohort study, analyzing data collected from a total of 12 tertiary care centers between April 2012 and April 2022. RESULTS: The study included 59 males and 107 females, a total of 166 patients, with an average age of 50.91 ± 21.25 years. Disease classification was TEN in 50% of cases, SJS in 33.1%, and SJS-TEN overlap in 16.9%. The average SCORTEN within the first 24 h was 2.44 ± 1.42. Supportive care was provided to 99.4% of patients. The most commonly used systemic immunomodulatory treatments were systemic steroids (84.3%), IVIG (intravenous immunoglobulin) (49.3%), and cyclosporine (38.6%). Plasmapheresis was administered to five patients. While 66.3% of patients were discharged, 24.1% resulted in exitus. Our comparative analysis of survivors and deceased patients found no effect of systemic steroids, IVIG, and cyclosporine treatments on mortality. Univariate analysis revealed that the SCORTEN scores on days 1 and 3 as well as the rates of detachment at the onset and during follow-up were significantly higher in deceased patients compared to survivors. The rates of fever, positive blood cultures, and systemic antibiotic use were higher in deceased patients compared to survivors. The presence of comorbidities, diabetes, and malignancy were significantly more common in deceased patients. Multivariate regression analysis indicated that over SCORTEN 2, the mortality risk exponentially rose with each SCORTEN increment, culminating in an 84-fold increase in mortality at SCORTEN 5-6 (odds ratio [95% confidence interval]: 13.902-507.537, p < 0.001) compared to SCORTEN 0-1. Additionally, the utilization of plasmapheresis was associated with a 22-fold increase in mortality (odds ratio [95% confidence interval]: 1.96-247.2, p = 0.012). CONCLUSION: Our study found that a high SCORTEN score within the first 24 h and the use of plasmapheresis were related to increased mortality, while systemic steroids, IVIG, and cyclosporine treatments had no impact on mortality. We believe that data gathered from one of the most comprehensive studies which we conducted on SJS-TEN will enrich the literature, although additional research is warranted.

Omalizumab versus cyclosporin-A for the treatment of chronic spontaneous urticaria: can we define better-responding endotypes?

BACKGROUND: Chronic Spontaneous Urticaria (CSU) is characterized by recurrent wheals and/or angioedema for longer than 6-weeks. Guidelines recommend Omalizumab (Oma) as first-line and Cyclosporine-A (Cs-A) as second-line treatment in antihistamine resistant CSU. This step-wise algorithm might be time-consuming and costly. OBJECTIVE: To determine indicators of response to Oma or Cs-A in CSU patients. METHODS: We retrospectively analyzed data from seven centers in Turkey; the inclusion criteria for patients were to receive both Oma and Cs-A treatment (not concurrently) at some point in time during their follow-up. Clinical and laboratory features were compared between groups. RESULTS: Among 110 CSU patients; 47 (42.7%) were Oma-responders, 15 (13.6%) were Cs-A-responders, and 24 (21.8%) were both Oma and Cs-A responders and 24 (21.8%) were non-responders to either drug. High CRP levels were more frequent in Cs-A-responders (72.7% vs. 40.3%; p = 0.055). Oma-responders had higher baseline UCT (Urticaria Control Test) scores (6 vs. 4.5; p = 0.045). Responders to both drugs had less angioedema and higher baseline UCT scores compared to other groups (33.3% vs. 62.8%; p = 0.01 and 8 vs. 5; p = 0.017). Non-responders to both drugs had an increased frequency in the female gender and lower baseline UCT scores compared to other groups (87.5% vs. 61.6%; p = 0.017 and 5 vs. 7; p = 0.06). STUDY LIMITATIONS: Retrospective nature, limited number of patients, no control group, the lack of the basophil activation (BAT) or BHRA (basophil histamine release assay) tests. CONCLUSIONS: Baseline disease activity assessment, which considers the presence of angioedema and disease activity scores, gender, and CRP levels might be helpful to predict treatment outcomes in CSU patients and to choose the right treatment for each patient. Categorizing patients into particular endotypes could provide treatment optimization and increase treatment success.

Birt-Hogg-Dubé Syndrome: Diagnostic Journey of Three Cases from Skin to Gene.

Birt-Hogg-Dube syndrome (BHDS) is a rare disorder characterized by the triad of cutaneous lesions, renal tumors, lung cysts and inactivation of the gene folliculin (FLCN). Here, we present three female patients diagnosed with BHDS. First case a 55-year-old female had flesh moles histopathology compatible with angiofibroma, multiple cysts in the lung and kidneys, FLCN gene mutations ('c.1285dupC [p.His429Profs*]' 11th exon and 'c.653G>A [p.Arg258His]' 7th exon). The second case a 76-year-old female had trichodiscoma on her skin, multiple cysts in the lung, spontaneous pneumothorax, FLCN gene mutation 'c.1285dupC (p.His429Profs*27) 11th exon' and, her son had renal carcinoma history under 50 years of age. Our third case, also the daughter of case 2, had dermal papules histopathology compatible with trichodiscoma, spontaneous pneumothorax, FLCN gene mutation 'c.1285dupC (p.His429Profs*27) 11th exon' and, parotid oncocytoma. Through our cases, we document the first case of two mutations ('c.1285dupC [p.His429Profs*]' 11th exon and 'c.653G>A [p.Arg258His]' 7th exon) in the same FLCN gene and the 11th known case of parotid oncocytoma associated with BHDS in the light of the literature.

Patch Test Results to European Baseline Series in Turkey: A Prospective and Multicenter Study.

BACKGROUND: Patch testing with the European Baseline Series (EBS) is an essential diagnostic tool for the assessment of allergic contact dermatitis. OBJECTIVE: The aims of the study were to describe the most common contact allergens identified with patch testing in Turkey and to establish the distribution of contact allergens among different regions in Turkey. PATIENTS AND METHODS: Twelve centers consisting of tertiary health care institutions were included in the study. A total of 1169 patients were patch tested with the EBS from 2015 to 2017. RESULTS: A total of 596 patients (51.0%) had 1 or more positive patch test reactions to the EBS. A total of 30.2% (n = 353) of those tested had clinical relevance. Contact sensitivity was more common in young patients. The most common 10 allergens were nickel sulfate (20.4%), followed by textile dye mix (8.6%), cobalt chloride (8.3%), potassium dichromate (8.0%), p-phenylenediamine base (4.5%), balsam of Peru (4.3%), methylchloroisothiazolinone/methylisothiazolinone (3.8%), thiuram mix (3.6%), fragrance mix I (3.0%), and methylisothiazolinone (2.9%). CONCLUSIONS: This study provides a comprehensive profile of patch test results with the EBS in Turkey. An ongoing decrease in contact allergy to methylchloroisothiazolinone/methylisothiazolinone and methylisothiazolinone was observed. Textile dye mix and p-phenylenediamine are beginning to pose a new risk in Turkey.

Epidemiology of Pemphigus in Turkey: One-year Prospective Study of 220 Cases.

Pemphigus is a group of rare and life-threatening autoimmune blistering diseases of the skin and mucous membranes. Although they occur worldwide, their incidence shows wide geographical variation, and prospective data on the epidemiology of pemphigus are very limited. Objective of this work is to evaluate the incidence and epidemiological and clinical features of patients with pemphigus in Turkey. All patients newly diagnosed with pemphigus between June 2013 and June 2014 were prospectively enrolled in 33 dermatology departments in 20 different provinces from all seven regions of Turkey. Disease parameters including demography and clinical findings were recorded. A total of 220 patients were diagnosed with pemphigus during the 1-year period, with an annual incidence of 4.7 per million people in Turkey. Patients were predominantly women, with a male to female ratio of 1:1.41. The mean age at onset was 48.9 years. Pemphigus vulgaris (PV) was the commonest clinical subtype (n=192; 87.3%), followed by pemphigus foliaceus (n=21; 9.6%). The most common clinical subtype of PV was the mucocutaneous type (n=83; 43.2%). The mean Pemphigus Disease Area Index was 28.14±22.21 (mean ± Standard Deviation). The incidence rate of pemphigus in Turkey is similar to the countries of South-East Europe, higher than those reported for the Central and Northern European countries and lower than the countries around the Mediterranean Sea and Iran. Pemphigus is more frequent in middle-aged people and is more common in women. The most frequent subtype was PV, with a 9-fold higher incidence than pemphigus foliaceus.

The efficacy of narrowband UVB treatment in pediatric vitiligo: a retrospective analysis of 26 cases.

BACKGROUND/AIM: Narrowband UVB (Nb UVB) treatment is commonly used for the management of psoriasis and atopic dermatitis, and is less often used for vitiligo in children. The aim of this study was to evaluate the efficacy and short-term safety of Nb UVB phototherapy in children diagnosed with vitiligo retrospectively. MATERIALS AND METHODS: A total of 26 patients younger than 18 years with the diagnosis of vitiligo and managed with Nb UVB phototherapy as documented in archive records were evaluated. Clinical response was assessed according to repigmentation of the lesions: good response when there was more than 75% repigmentation, moderate response when there was 25%-74% repigmentation, poor response when repigmentation was less than 24%, and unresponsive when there was no pigmentation and new lesions occurred. RESULTS: A total of 26 patients received Nb UVB treatment; 14 were girls and 12 were boys. The age at onset of the disease varied between 2 and 18 years, with a mean age of onset of 10.07 ± 4.53 years. Repigmentation rate of >75% was detected in 45.4% of cases. CONCLUSION: Nb UVB phototherapy seems to be a well-tolerated effective and safe treatment option in children, especially those unresponsive to topical treatment and those with widespread lesions. However, long-term risks such as photocarcinogenesis and photoaging should kept in mind.

A case of mycosis fungoides-like lesions developing after levetiracetam therapy.

Levetiracetam is a relatively new antiepileptic drug that has previously not been associated with severe dermatological side effects. We report the case of a 31-year-old male treated with levetiracetam for seizures who subsequently developed a mycosis fungoides-like drug reaction that resolved upon dosage reduction.

Multiple warty dyskeratomas: case report.

Warty dyskeratoma is a rare tumor that presents mainly as an isolated papule or nodule on the scalp, face or neck in the adults. Warty dyskeratoma frequently arises as a single lesion with a central keratotic plug on the skin of middle-aged or elderly people. Multiple warty dyskeratomas are very rare cutaneous lesions which are mostly seen on the scalp. We herein report a multiple warty dyskeratoma in a female patient.

Cutaneous heterotopic bronchogenic tissue in the scapular area.

Cutaneous bronchogenic anomalies are very rare lesions, and most of them present as cysts and/or sinus tracts in the subcutaneous layer. We report a 15-year-old boy who presented with a crusted and papillomatous plaque over the right scapular region. Histopathological examination of this plaque revealed pseudostratified ciliated columnar epithelium with papillary invaginations that included mucinous material. The lesion had no cyst or sinus formation and was bordered with smooth muscle bundles in stroma.

Generalized Sweet syndrome lesions associated with Behçet disease: a true association or simply co-morbidity?

Sweet syndrome or acute febrile neutrophilic dermatosis is a condition characterized by painful erythematous papules, nodules, and plaques, which demonstrate mature neutrophilic infiltration of the upper dermis histopathologically, and is usually associated with systemic symptoms such as fever and neutrophilia. Generalized Sweet syndrome lesions are usually noted in association with malignancies. Sweet syndrome lesions are rarely seen in patients with Behçet disease and, if present, are usually fewer in number. In this report, we present two female patients who developed generalized Sweet syndrome lesions during follow-up after the diagnosis of Behçet disease. The clinical and histopathologic findings in our patients together with the data in the literature suggest that generalized Sweet syndrome lesions in a patient with clinically inactive Behçet disease should remind the clinician that a strict differential diagnosis needs to be made between Sweet syndrome associated with Behçet disease and the rare cutaneous manifestation of Behçet disease that resembles the lesions of Sweet syndrome.

Miliary osteoma cutis of the face: treatment with the needle microincision-extirpation method.

Multiple miliary osteoma cutis of the face represents primary extra-skeletal bone formation that arises within the skin of the face. Multiple miliary osteoma cutis of the face is a rare complication of chronic inflammatory acne vulgaris and has invasive and non-invasive treatment alternatives different from acne vulgaris. Invasive techniques should be simple, easy, and inexpensive, with minimal risk of scarring and pigmentation. We used a needle microincision-extirpation technique in a patient with multiple miliary osteoma cutis unresponsive to non-invasive treatment modalities. Skin overlying the papules was incised with a needle and then the calcificated papules were extirpated by using a small curettage device. Lesions were left to secondary healing. Results were quite good and cosmetically acceptable.

Parry-Romberg syndrome associated with borreliosis: could photochemotherapy halt the progression of the disease?

Parry-Romberg syndrome (PRS) or progressive hemifacial atrophy is a rare entity characterized by unilateral atrophy of the skin, subcutaneous tissue and sometimes bone and cartilage. Although this syndrome has overlapping features of scleroderma 'en coup de sabre', it shows little or no sclerosis and may affect the entire distribution of the trigeminal nerve including the eye and tongue. As the pathogenesis is unknown, no effective therapy exists. We present here the third case of PRS associated with borreliosis and more interestingly two cases whose progressive course have been stabilized with gel PUVA therapy.

A case of epidermodysplasia verruciformis associated with squamous cell carcinoma and Bowen's disease: a therapeutic challenge.

Epidermodysplasia verruciformis (EV) is a very rare and chronic disease characterized by a susceptibility to cutaneous infections by a group of phylogenetically related human papillomavirus (HPV) types. We present here a 52-year-old man previously diagnosed and treated as squamous cell carcinoma but also found to have epidermodysplasia verruciformis lesions in association with Bowen's disease. The patient was effectively treated with a combination of interferon therapy (6 million units, twice a week) and topical imiquimod for Bowen's disease (five times a week). We conclude that topical imiquimod is very effective in the treatment of Bowen's disease and may be combined with systemic interferon therapy in rare cases with multiple vivid presentations of HPV infections.

Localized unilateral hyperhidrosis and neurofibromatosis type 1: case report of a new association.

Localized unilateral hyperhidrosis (LUH) is an uncommon but well-defined special form of localized hyperhidrosis with diverse causes. It often occurs within a sharply demarcated area on the face or upper extremities. Here, we report a case of LUH in a 16-year-old male with neurofibromatosis type 1 (NF1) and review related data in the literature. The association of this rare entity and NF1 has not yet been described. Subcutaneous botulinum toxin A injection provided a good clinical response with symptomatic relief.

Lack of correlation between Helicobacter pylori infection and autologous serum skin test in chronic idiopathic urticaria.

BACKGROUND: There are controversial reports about the direct role of Helicobacterpylori infection in chronic idiopathic urticaria. The indirect role of H. pylori infection in the induction of pathogenetic antibodies is not fully elucidated either. This study aims to reveal the association of H. pylori infection with autologous serum skin test positivity in chronic idiopathic urticaria (CIU) patients. METHODS: A total of 47 patients (35 women, 12 men, age range 17-65 years) diagnosed as CIU were included in the study. Autologous serum skin test was performed on all patients. The patients were examined with a commercially available ELISA test for H. pylori-specific antibodies. Gastroscopy with mucosal biopsy and rapid urease tests were proposed to verify the presence of H. pylori infection. RESULTS: Helicobacter pylori infection was detected in 33 of the 47 patients (70%). No significant relation was found between the autologous serum skin test positivity and the serological and histopathological presence of H. pylori infection. CONCLUSION: The results of our study suggest that chronic H. pylori infection does not appear to have a role in the induction of autoantibodies in CIU.