Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis: 11-year Demographic Clinical and Prognostic Characteristics.

Background: Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are acute, life-threatening, severe drug reactions. Randomized studies on these diseases are difficult to perform. Aims and Objectives: The purpose of this study was to summarize the demographic and clinical characteristics of patients with SJS and TEN in a tertiary hospital in Turkey. Materials and Methods: We evaluated the records of 33 patients with SJS and TEN who were followed in our clinic or examined between January 2008 and June 2019, retrospectively. Age, sex, time of admission to hospital, causative drug, presence of concomitant disease, skin findings, mucosal involvement, the severity-of-illness score for TEN, the medication used, antibiotic use, transfer to intensive care, development of complications, and death or discharge status were noted. Results: Of the 33 patients, 11 (33.3%) had SJS, 3 (9.1%) had SJS/TEN overlap, and 19 (57.6%) had TEN. The majority (60.6%) of the patients were female. Nineteen (57.6%) patients had one, and 13 (39.4%) had more than one suspected drug exposure in their history. The most commonly suspected drugs were antibiotics. Twelve (36.4%) patients had intensive care unit hospitalization. Ten (30.3%) patients died. Conclusion: The demographic data of our study were consistent with the literature. Similar to the literature, antibiotics were the most common reaction-causing drugs. However, antiepileptic drugs, which were more frequently reported in other studies, were identified as suspicious in only one patient. We believe that our study will contribute to the determination of characteristics of this rare disease with real-life data.

Evaluation of association with subtypes and alleles of Blastocystis with chronic spontaneous urticaria.

Blastocystis is a single-celled parasite commonly found in humans and its pathogenic role is still controversial. In recent years, some studies have suggested that Blastocystis may be a possible agent of gastrointestinal and dermatological symptoms such as acute or chronic urticaria, angioedema, rash, itch, palmoplantar, and diffuse pruritus. We aimed to investigate whether there is a relationship between Blastocystis subtypes and alleles in patients with chronic spontaneous urticaria (CSU) as a case-control study. In this study, stool samples were collected from patients with CSU (n=135) and healthy individuals (n=54). The presence of Blastocystis was investigated using the direct saline smear, Lugol's iodine staining, trichrome staining, Jones' medium culture and PCR assays in stool samples and subtypes (STs) were determined by sequencing according to DNA barcoding. The presence of Blastocystis was identified in 30.4% (64/210) the stool samples, including 31.9% (43/135) of the patients with CSU and 14.8% (8/54) of the control group. Moreover, it was found statistically significant the presence of Blastocystis in terms of both groups (p<0.018). ST3 was detected in 45.9% and 62.5 % as the most prevalent subtype the patients with CSU and the control group, respectively. ST1 (18.9%), ST2 (27%) and ST7 (8.1%) was identified in the patients with CSU group. There was no statistically significant correlation between Blastocystis subtypes and both the groups (p<0.240, p<0.323). Allele 4 for ST1; alleles 9, 10, 11 and 12 for ST2; alleles 34, 36 and 38 for ST3; alleles 41 and 101 for ST7 were detected. Allele 34 (ST3) was found significant in the patients with CSU as compared with control group (p<0.020). Moreover, statistically significant association was found between total IgE value and the certain subtypes (ST2 and ST3) (p<0.0001). As a result of this study, the presence of Blastocystis ST3 allele 34 significantly associated with chronic spontaneous urticaria was revealed.

Evaluation of dry eye and Meibomian gland dysfunction in female androgenetic alopecia patients.

PURPOSE: To evaluate dry eye tests and Meibomian gland functions in female androgenetic alopecia patients. MATERIALS AND METHODS: Twenty left eyes of 20 female androgenetic alopecia patients (Group 1) and 20 left eyes of 20 healthy female volunteers (Group 2) were enrolled in the study. The presence of dry eye was evaluated with Schirmer 1 test, invasive tear film break-up time (T-BUT), Oxford scale scoring and Ocular Surface Disease Index (OSDI) score assessments. Evaluation of upper and lower eyelid Meibomian glands was performed using infrared filter of a slit-lamp biomicroscope, and the drop out ratio was scored for each eyelid. RESULTS: The mean ages of Group 1 and Group 2 were 48.3 ± 6.6 (range, 38-58) and 49.8 ± 5.4 (range, 38-59) years, respectively (P = 0.437). Mean T-BUT was statistically significantly lower in Group 1 (P = 0.013); mean OSDI score was significantly higher in Group 1 (P = 0.049). Mean Schirmer 1 score was lower and mean Oxford score was higher in Group 1, but the differences were not statistically significant (P = 0.291, P = 0.088, respectively). In addition, upper, lower and total meiboscores were significantly higher in Group 1 (P = 0.007, P = 0.003, P = 0.002, respectively). CONCLUSION: Alterations in sex hormones play a role in the pathogenesis of both female androgenetic alopecia and Meibomian gland dysfunction. Significant differences were detected in the results of dry eye tests and Meibomian gland dropout ratios between subjects with female androgenetic alopecia and healthy control subjects. For this reason, female androgenetic alopecia patients should be examined for dry eye and Meibomian gland dysfunction and should be treated to prevent serious consequences.

Dry eye and Meibomian gland dysfunction evaluation in sarcoidosis patients.

PURPOSE: To analyze the relation between Meibomian gland dysfunction, dry eye, and sarcoidosis. MATERIALS AND METHODS: Twenty eyes of 10 sarcoidosis patients (Group 1) and 20 left eyes of 20 age-sex matched healthy volunteers (Group 2) were included. Presence of dry eye was evaluated with Schirmer 1 test, tear film break-up time (T-BUT), Oxford scale scoring, Ocular Surface Disease Index (OSDI) score assessments. A slit-lamp biomicroscope infrared filter (Topcon, SL-D701, IJssel, The Netherlands) was used to evaluate Meibomian glands. The drop-out ratio according to meibography was scored for each eyelid from grade 0 (no loss) through grade 3 (lost area >2/3 of the total Meibomian gland area). RESULTS: Among dry eye tests mean Schirmer 1 and T-BUT values were lower and OSDI score was higher in Group 1 compared to Group 2 and the differences were statistically significant (p = 0.017, p = 0.039, p = 0.003, respectively). In addition, the upper, lower and total meiboscores were statistically significantly higher in Group 1 (p = 0.047, p = 0.003, p = 0.005, respectively). CONCLUSION: A significantly higher presence of dry eye and Meibomian gland drop out ratios was detected in sarcoidosis patients. It is important to monitor sarcoidosis patients for dry eye and Meibomian gland dysfunction and when detected, to treat adequately to prevent ocular surface damage.

Comparison of Skin Scraping and Standard Superficial Skin Biopsy in the Laboratory Diagnosis of Scabies

Objective: Scabies is diagnosed based on the presence of burrows on the skin, Sarcoptes scabiei adult, egg, or scybala in skin scrapings. The laboratory diagnosis of scabies poses various challenges. We aimed to compare the analytical performance of skin scraping and standard superficial skin biopsy (SSSB) and to investigate the correlation with false negative results in the laboratory diagnosis of scabies. Methods: Skin scraping and SSSB were applied from July 1 to December 31, 2018 on 42 patients whose burrows were marked using dermatoscopy, as obtained from the laboratory information system. Results: The number of patients who tested positive for scabies with skin scraping was 18 (42.9%) and 24 (57.1%) with SSSB, and the difference was significant (p=0.003). Sensitivity was 42.9% for skin scraping and 57.1% for SSSB. The number of positive cases with both techniques was 15 (35.7%). The number of patients positive with only skin scraping was 3 (7.1%) and only SSSB was 9 (21.4%). Conclusion: To date, it has seemed impossible to diagnose scabies using a single clinical or laboratory test. According to our results, SSSB is an inexpensive and easy-to-apply method with high sensitivity for obtaining skin samples for scabies laboratory diagnosis.

Experience with Omalizumab for the treatment of chronic spontaneous urticaria in a tertiary center: real life experience.

PURPOSE: Chronic spontaneous urticaria (CSU) is defined as urticaria and/or angioedema that appears spontaneously due to known or unknown causes and lasts for at least 6 weeks. Omalizumab, an anti-IgE antibody that binds circulating free IgE, has recently emerged as a promising treatment for CSU, a condition which impairs patients' quality of life. We aimed to contribute real life data by reporting our experience with omalizumab in the treatment of intractable CSU. METHODS: Of 140 patients treated with omalizumab in our clinic between September 2013 and January 2018, 86 CSU patients with available current data were retrospectively evaluated in terms of sex, age, urticaria duration, urticaria activity score over 7 days (UAS7) before and after omalizumab, relapses and time to relapse, length of remission after omalizumab cessation, adverse events, and comorbidities. RESULTS: The mean age of the patients was 45.5 ± 14.3 years and 73.3% were women. Mean duration of urticaria before initiation of omalizumab therapy was 54.5 ± 67 months. All patients had used antihistamines before starting omalizumab treatment. The mean number of omalizumab doses was 11.9 ± 9.3. The mean duration of omalizumab treatment was 13.3 ± 10.4 months. Mean UAS7 score was 38.9 ± 4.1 before the start of omalizumab treatment, and 7.9 ± 10.5 after treatment. Treatment was discontinued in 10 patients (11.6%) due to nonresponse or loss of effect. Four patients (4.65%) experienced adverse events. Treatment was discontinued in 1 patient (1.16%) due to side effects. Of the 55 patients whose treatment was discontinued after their symptoms resolved, 31 (56.3%) relapsed after omalizumab cessation. Twenty-four patients (43.6%) did not relapse after omalizumab cessation. CONCLUSIONS: Our results show that omalizumab was an effective treatment for intractable CSU and did not cause any serious adverse effects other than asthenia, vertigo, and injection site reaction in four patients. These findings are relevant because they reflect real-life data.

Dry Eye and Quantitative and Qualitative Changes of Meibomian Glands in Patients With Pemphigus.

PURPOSE: To evaluate dry eye and quantitative and qualitative changes of meibomian glands with meibography in patients with pemphigus. METHODS: Twenty left eyes of 20 patients with pemphigus (group 1) and 20 left eyes of 20 age- and sex-matched healthy volunteers (group 2) were enrolled. Dry eye was evaluated with Oxford scale scoring, tear film breakup time, Schirmer 1 tests, and Ocular Surface Disease Index score assessments. Morphological changes and dropout ratio of meibomian glands were evaluated by upper and lower eyelid meibography (SL-D701; Topcon, IJssel, The Netherlands). Meibomian gland dropout ratio was scored for each eyelid from grade 0 (no loss) through grade 3 (lost area >2/3 of the total meibomian gland area). RESULTS: The mean ages of group 1 and group 2 were 51.8 ± 11.1 (range: 32-73) and 50.4 ± 9.6 years (range: 32-70), respectively (P = 0.672). Schirmer 1 and tear film breakup time values were lower and Oxford and Ocular Surface Disease Index scores were higher in group 1 when compared with group 2, and the differences were statistically significant (P = 0.01, P < 0.001, P = 0.001, and P < 0.001, respectively). Upper eyelid, lower eyelid, and total eyelid meiboscore values were significantly higher in group 1 (P = 0.005, P = 0.018, and P = 0.002, respectively). Morphological changes in meibomian glands were detected in 16 eyes (80%) among group 1. CONCLUSIONS: Between patients with pemphigus and those who were healthy, there were significant differences in the results of dry eye tests and meibomian gland morphology. Patients with pemphigus should be monitored for dry eye and meibomian gland dysfunction and be promptly treated to prevent the serious consequences of dry eye.

A novel mutation of interleukin-1 receptor antagonist (IL1RN) in a DIRA patient from Turkey: Diagnosis and treatment.

Sözeri B, Gerçeker-Türk B, Yildiz-Atikan B, Mir S, Berdeli A. A novel mutation of interleukin-1 receptor antagonist (IL1RN) in a DIRA patient from Turkey: Diagnosis and treatment. Turk J Pediatr 2018; 60: 588-592. Autoinflammatory diseases can cause severe inflammation in bone and skin such as neonatal-onset multisystem inflammatory disease (NOMID), Majeed syndrome, interleukin-36 receptor antagonist deficiency (DITRA) and deficiency of interleukin-1 (IL-1) receptor antagonist (DIRA) syndrome. Here we report a five-year old boy who was admitted to the hospital with pustular skin lesions and fever in the first month of his life. Molecular analysis of IL1RN gene revealed a single homozygous C nucleotide deletion at nucleotide position 396 (p.Thr133Profs*118). The novel p.Thr133Profs*118 mutation found in our study caused frameshift mutation and as a result, the respective protein is most likely non-functional. The patient, who received a variety of treatments for various preliminary diagnoses until the final diagnosis (DIRA), was treated with recombinant IL-1Ra, anakinra, and experienced significant clinical improvement.

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.

Reflectance confocal microscopy of mammary Paget disease.

Mammary Paget disease is the intraepidermal adenocarcinoma of the nipple area which is characterized usually by a well-demarcated eczematous plaque. Reflectance confocal microscopy (RCM), is an in vivo noninvasive diagnostic tool with a high-resolution imaging of the skin, almost comparable to conventional histology. RCM findings of Paget disease are limited in the literature. Most of the reported cases are the extramammary type. In this report, we aimed to evaluate the RCM findings of a non-pigmented mammary Paget disease in a 65 year-old woman.

Pemphigoid Diseases as a Sign of Active Psoriasis: A Case Report and Brief Review.

BACKGROUND: Overlap of bullous pemphigoid (BP) with chronic psoriatic plaques (CPP) is a common condition. However, the association of BP with pustular psoriasis (PP) is uncommon. Moreover, perilesional erythema and pustular lesions on CPP are accepted as a sign of unstable psoriasis. Unstable psoriasis could be triggered by certain irritant topical treatments against psoriasis. These chemical agents could also induce a localized pattern of generalized PP. Here, we describe BP and PP collision in unstable CPP. OBJECTIVE: By this observation we suggest that BP could be a sign of active psoriasis. Presumably, psoriasis-induced BP is an inflammation activity-dependent condition. METHODS: This study is a case report and literature review. RESULTS: The dramatic response of bullo-pustular lesions to short-term methotrexate (MTX) treatment suggests the rule of 'no psoriasis, no BP'. Presumably, MTX supressed the active inflammation of CPP and BP disappeared following CPP control. CONCLUSION: BP can be a sign of active psoriasis in the present case.

Amiodarone-induced multiorgan toxicity with ocular findings on confocal microscopy.

Amiodarone is an antiarrhythmic medication that can adversely effect various organs including lungs, thyroid gland, liver, eyes, skin, and nerves. The risk of adverse effects increases with high doses and prolonged use. We report a 54-year-old female who presented with multiorgan toxicity after 8 months of low dose (200 mg/day) amiodarone treatment. The findings of confocal microscopy due to amiodarone-induced keratopathy are described. Amiodarone may cause multiorgan toxicity even at lower doses and for shorter treatment periods.

Clinical and mycological analysis of twenty-one cases of tinea incognita in the Aegean region of Turkey: a retrospective study.

Tinea incognita is a dermatophyte infection with atypical clinical features modified by the improper use of corticosteroids or calcineurin inhibitors. The aim of this study was to analyze clinical and microbiological features of patients with tinea incognita. A total of 6326 patients referred to mycology laboratory between January 2008 and January 2011 for mycological examination with a diagnosis of tinea incognita were reviewed retrospectively. Twenty-one patients, 13 (61.9%) women and 8 (38.1%) men, mean age 42.2±36.8, were included in the study. Of them, lesions were localized in 15 (71.4%) patients and widespread in six (28.6%) patients. The mean duration of the disease was 9.5 (range 1-120) months. All patients had a history of treatment with steroids. Before admission, most of them had been misdiagnosed as eczema or psoriasis. Microscopic examination revealed hyphae and spores in most of the cases (n=17, 80.95%). Mycological cultures were positive in 19 (90.5%) patients. The most frequently isolated dermatophyte was Trichophyton rubrum (n=14, 66.7%). This case series revealed Trichophyton rubrum as the most frequent agent of tinea incognita. To the best of our knowledge, this is the largest case series from Turkey describing clinical features and mycological agents of tinea incognita.

Follicular mucinosis and follicular mycosis fungoides: clinicopathological evaluation of seven cases.

OBJECTIVE: Follicular mucinosis is a disease characterized by follicular degeneration and mucin accumulation. It can be seen in mycosis fungoides, although idiopathic or forms associated with other diseases are also known. Follicular mycosis fungoides is a type of mycosis fungoides with different clinicopathological and prognostic features. MATERIAL AND METHOD: Seven cases with follicular centered lesions and multiple biopsies (2-6) were included. Cases were evaluated according to their clinical, histological and immunophenotypical features and follow-up data. RESULTS: All cases were male, and the mean age was 40.3 (range 18-61). Clinical complaints were follicular prominence, erythema and alopecia at head and neck, trunk, and lower limbs. Follicular mucinosis (6/7), and dermal lymphoid infiltration showing minimal-intensive folliculotropism accompanied by eosinophils was seen. Lymphoid infiltration was composed of small-medium sized cells, with scattered hyperchromatic nuclei in six cases. In one case there was only minimal cytological atypia. Intense folliculotropism of atypical lymphocytes and dense dermal infiltration without follicular mucinosis was seen in one case. Local and/or systemic treatments were applied and partial remission was achieved histologically. In three cases new and increasing lesions were seen. Density of infiltration and atypia were increased. CONCLUSION: The findings supported the opinion that follicular mucinosis is an important finding seen in mycosis fungoides. There can be important differences concerning the amount of infiltration and degree of atypia. In cases where the density of infiltration associated with follicular mucinosis is not diagnostic for MF, there can be progression over time. Long-term follow up is necessary in such cases where the differential diagnosis is difficult.