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Introduction Chronic spontaneous urticaria (CSU) is defined as recurrent attacks of urticaria present for more than six weeks. The monoclonal anti-immunoglobulin E antibody, omalizumab, was approved for the treatment of CSU in patients who remain refractory to H1-antihistamines. Biologic agents are shown not to increase the risk of COVID-19 infection in different studies. Objective In the present study, we aimed to determine the prevalance of COVID-19 infection in relation to the age, gender, presence of other comorbidities, and treatment given for CSU. Methods We conducted a descriptive cross-sectional study of 233 patients diagnosed with CSU in a tertiary referral hospital. Demographical data, treatment given for CSU, the presence of COVID-19-related symptoms, history of close contact to a person with COVID-19 and COVID-19 real-time polymerase chain reaction (RT-PCR) results were determined via a telephone survey and checked from medical data records. Results One hundred sixty patients were female; whereas 73 were male. The mean age was 44.76. Out of 233 patients with chronic urticaria, 125 had symptoms related to COVID-19 infection. RT-PCR testing for COVID-19 was performed in 156 patients. Of 156 patients with COVID-19 RT-PCR test, RT-PCR result was positive in 15 cases. Conclusions No statistically significant relationship was found between COVID-19 RT-PCR positivity and the type of treatment administered for chronic urticaria when the patients are divided into omalizumab ± oral antihistamines and only oral antihistamines treatment groups (p = 0.150). Omalizumab seems to be safe in the era of COVID-19.

Objective: Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are dermatologic emergencies. There is a lack of consensus regarding appropriate management of SJS/TEN. The aim of this study was to evaluate demographic and clinical features, management and outcomes of SJS/TEN patients. Materials and Methods: The data of patients who were ≥18 years old and hospitalized with the diagnosis of SJS, SJS-TEN overlap and TEN at Hacettepe University, between 1992 and 2018 were analyzed retrospectively. Patient demographics, medications, time between the first causative drug intake and the onset of symptoms, mucous membrane involvement, treatment modalities including supportive measures, intravenous immunoglobulin, cyclosporine and systemic corticosteroids, duration of hospitalization, and mortality outcomes were recorded from patient charts. Results: A total of 23 patients (11 men; 12 women) with a mean age of 46.4 ± 19.5 years were included in the study. Twelve patients (52.2%) had SJS, 8 patients (34.8%) had SJS/TEN overlap and 3 patients (13%) had TEN. Mean duration of hospitalization was 12.6 ± 6.5 days. Twenty-two patients (95.7%) were attributed to a specific medication, whereas triggering factor was not detected in 1 patient (4.3%). More than one drug was responsible for 9 patients, including antimicrobials (n=11, 47.8%), anticonvulsants (n=8, 34.8%) and/or non-steroid anti-inflammatory drugs (n=5, 21.7%). The median time between the intake of medication and the onset of symptoms was 20 days (IQR 5.5-30). Sixteen patients used systemic corticosteroids (69.6%) and 4 patients used intravenous immunoglobulin (17.4%) whereas 3 patients used systemic corticosteroids in combination with intravenous immunoglobulin (n=2, 8.7%) or cyclosporin (n=1, 4.3%). The median time between the onset of symptoms and the onset of the rash to the treatment was 5 days (IQR 3-7). Mortality was not observed in our cohort. Conclusion: SJS/TEN was most commonly developed because of drugs, mainly antimicrobials, anticonvulsants and/or non-steroid anti-inflammatory drugs. The absence of mortality in our cohort was considered to be associated with younger age, low rate of TEN, prompt initiation of treatment (mainly corticosteroids) following the rapid discontinuation of the suspected drug.

The features of chronic rejection (CR) in full-face vascularized composite allotransplantation (VCA) are not well-known. Herein, we report a full-face transplant patient that experienced two episodes of acute rejection (AR) and three episodes of AR/CR over the course of 6-years. The patient noticed a small, round patch of hair loss in his beard 9 months after the second AR episode, which occurred 21 months post-transplantation. Biopsy of the alopecic patch showed lichen-planopilaris-like features, which were suggestive of early CR. Despite an increase in immunosuppressive dosages, the alopecia progressed. Following the second and third AR/CR episodes, the alopecia became more pronounced, with the addition of hyperpigmentation as well as sclerosis and telangiectasia. The findings of multiple biopsies showed CR. Based on these findings we think that alopecia with lichen-planopilaris-like histopathological features similar to grade III AR features, particularly in hair follicles appears to be an early finding of CR in the presented patient. The findings further indicate that follicular involvement may be a significant feature of CR in VCA patients and that it can present prior to sclerosis, vasculopathy, or loss of adnexa. The present case is uniquely important because of the distinctive presentation of CR, with hair follicles clinically and histopathologically affected, leading to progressive and irreversible alopecia with lichen-planopilaris-like histopathology.

Objective: Mycosis fungoides is the most common type of primary cutaneous T-cell lymphomas. In this study, we aimed to investigate the biochemical parameters of beta-2 microglobulin, lactate dehydrogenase, CD4/CD8 lymphocyte ratio determined by flow cytometry, and T cell clonality in patients with MF and to correlate these parameters in stage 1A and other stages. Materials and Methods: A hundred sixty-one (87 Male, 74 Female) patients followed-up between January 1995 and January 2019 were retrospectively evaluated. Patients’ demographics, stage of the disease, peripheral lymph node and organ involvement, peripheral blood beta-2-microglobulin, lactate dehydrogenase levels, CD4/CD8 + lymphocyte ratio and presence of T cell clonality in biopsy samples were evaluated. Results: Median beta-2 microglobulin levels were 1608.5 ng/ml for stage 1A and 1970.5 ng/ml for other stages. Peripheral blood median CD4/CD8 lymphocte ratio was 1.60 for stage 1A and 1.85 for other stages. We found statistically significant difference between two groups in terms of beta-2 microglobulin levels and peripheral blood median CD4/CD8 lymphocte ratio (p=0.001, p=0.04). No statistically significant difference was found between two groups in terms of lactate dehydrogenase levels and T-cell clonality (p=0.234, p=0.525). Conclusion: Our study supports that high peripheral blood beta-2 microglobulin level and CD4/CD8 lymphocyte ratio at the time of diagnosis may imply advanced stage and poor prognosis in Mycosis Fungoides.

Poikiloderma with neutropenia (PN), Clericuzio-type is a rare autosomal recessively transmitted genodermatosis caused by biallelic mutations in the USB1 gene and is characterized by early-onset poikiloderma and chronic neutropenia. Nail dystrophy, palmoplantar hyperkeratosis, hypogonadotropic hypogonadism, and recurrent infections can be associated with the disease. Herein, we present a 27-year-old Turkish male patient newly diagnosed as PN, Clericuzio-type after confirmation of a c.531delA (p.His179MetfsX86) homozygous deleterious mutation in exon 5 of the USB1 gene. The presented case highlights the importance of genetic testing for avoiding misdiagnosis based solely on clinical findings, as well as the benefit of a multi-disciplinary diagnostic approach, as he was initially misdiagnosed as Rothmund-Thompson syndrome and subsequently diagnosed as PN, Clericuzio-type at age 27 years.

Acquired reactive perforating collagenosis is a rare skin disorder characterized by the presence of umbilicated pruritic papules and nodules. Transepidermal elimination of altered and perforating bundles of basophilic collagen from the epidermis is a characteristic histologic feature of acquired reactive perforating collagenosis. Along with its well-known association with systemic diseases such as diabetes mellitus, chronic renal failure, and dermatomyositis, there are reports of acquired reactive perforating collagenosis being associated with malignancies. Herein, we present a case of acquired reactive perforating collagenosis associated with chronic lymphocytic leukemia, prostate adenocarcinoma, and Graves's disease. Clinicians are required to be more vigilant in evaluating patients with acquired reactive perforating collagenosis due to its unique association with malignancies and other systemic diseases.

Epidermolysis bullosa simplex migratory circinate erythema (EBS-Migr) is an uncommon subtype of EBS. We report a case of EBS-MIGR with a novel heterozygous pathogenic mutation in exon 9 (frameshift deletion c.1650delC) and likely benign heterozygous mutation in exon 2 (missense c.591C > A) of keratin 5. This novel pathogenic mutation in KRT5 expands the molecular spectrum of this rare subtype of EBS.

Aim This study aimed to determine the characteristics of dermatology consultation requests from the adult and paediatric emergency departments (EDs) of a university hospital during 8 months of the COVID‐19 pandemic in 2020 and to compare them with the same 8 months of 2019. Materials and Methods Electronic medical records of dermatology consultation requests from adult and paediatric EDs between 15 March 2019 and 15 November 2019, and between 15 March 2020 and 15 November 2020 were retrospectively reviewed. Results The study included 495 consecutive dermatology consultation requests. In total, 283 (57%) consultation requests occurred in 2019, vs 212 (43%) between in 2020 during the COVID‐19 pandemic. The number of consultation requests per day was significantly lower in 2020 (0.9 ± 0.1 per day) than in 2019 (1.15 ± 0.1 per day; P = .002), and was significantly lower in March, April and May 2020, as compared with March, April, and May 2019 (P = .004, P = .001, and P = .001, respectively). The median time from onset of dermatological symptoms to ED presentation was significantly longer in 2020 than in 2019 (4 days in 2019 vs 7 days in 2020; P

Background Rosacea is a chronic disease that is characterized by facial skin inflammation and vascular abnormality. Vascular endothelial growth factor (VEGF) is a potent mediator of vascular permeability and inflammation that might play a role in the pathogenesis of rosacea. Objective This study aimed to determine the association between VEGF gene polymorphisms and rosacea. Methods A case-control study design was used to compare 100 patients with rosacea and 100 age- and gender-matched control subjects in terms of VEGF polymorphisms based on polymerase chain reaction and the serum level of VEGF and VEGF receptors based on enzyme-linked immunosorbent assay. Results Heterozygous and homozygous +405C/G polymorphism of the VEGF gene was observed to increase the risk of rosacea 1.7-fold (95% confidence interval 1.2-4.2) and 2.3-fold (95% confidence interval 1.2-4.2), respectively. There was a significant positive correlation between the severity of rosacea and +405C/G polymorphism of the VEGF gene in patients with erythematotelangiectatic rosacea. Limitations Serum VEGF and VEGF receptor levels were measured in the limited number of patients. Conclusion The present findings indicate that +405C/G polymorphism of the VEGF gene increases the risk of rosacea.

BACKGROUND Lichen sclerosus et atrophicus (LSA) is a chronic inflammatory cutaneous disease. AIM To evaluate the characteristics of paediatric LSA patients in Turkey. MATERIAL AND METHODS Data for patients aged

The vast majority of primary immunodeficiencies (PIDs) occur due to the defects in cells originating from hematopoietic stem cells, while in some PIDs, there are defects in various genes responsible for non-leucocyte immune response such as seen in epidermodysplasia verruciformis (EV). EV caused by the mutations in TMC6, TMC8, and CIB1 genes is called "typical." "Atypical" EV may develop in patients with primary immunodeficiencies originating from hematopoietic stem cells, which include severe T-cell failure, caused by inactivating biallelic mutations of STK4, RHOH, CORO1A, ITK, TPP2, DCLRE1C, LCK, RASGRP1, or DOCK8 genes. Here, we present a family with TMC8 gene mutation leading to disseminated epidermodysplasia verruciformis including laryngeal papilloma and recurrent cutaneous squamous cell carcinomas. Typical EV with impaired local, keratinocyte-intrinsic immune response should be considered when routine immunological examinations are normal in patients presenting with clinical signs of EV. Although it is not possible to prevent EV lesions, early and appropriate surveillance for malignancy is mandatory.

The risk of active tuberculosis is still a concern in patients receiving biologics. To determine the risk of latent tuberculosis infection (LTBI) reactivation by Quantiferon-TB Gold (QFT) assay in psoriatic patients treated with biologics in 11 years' follow-up, along with chest radiography alterations. This retrospective study included 279 patients with plaque-type and/or pustular, or nail psoriasis who were treated with biologics, and had results for ≥2 LTBI tests. The QFT outcomes were defined according to the baseline and the follow-up QFT results; seroconversion as from negative to positive, seroreversion as from positive to negative, persistently seronegative as invariantly negative, persistently seropositive as invariantly positive, and other any result was accepted as indeterminate. Demographic features, the presence and the type of any chest X-ray abnormality was noted during the follow-up. Of 279 baseline QFT tests, the vast majority were negative (n = 193; 69%), with a less of positive (n = 86; 31%). Ten (5.2%) out of 193 patients converted from negative to positive QFT status after starting biologic therapy (P < 0.001) during 11 years' follow-up. Although these 10 patients exhibited seroconversion of QFT from negative to positive, only one patient was diagnosed with active TB. There was no statistically significant difference among biologics as regards with QFT seroconversion risk (P = 0.09). This study showed that 5.2% of patients showed seroconversion. Annual QFT testing remains a necessary and mandatory tool to prevent further TB reactivation in psoriasis patients taking biologic therapy although only one patient was diagnosed with active TB in this cohort. This article is protected by copyright. All rights reserved.

Recent reports indicate that hydroxychloroquine is a potential new treatment option for alopecia universalis; thus, we aimed to report on the safety and efficacy of hydroxychloroquine in 6 patients with refractory alopecia universalis that were treated with 400 mg/d continuously for ≥6 months. The treatment outcome was retrospectively evaluated using the Severity of Alopecia Tool (SALT), and at the end of 6 months, patients with a ≥50% decrease in the SALT score were considered as strong responders, a 5%-50% decrease as intermediate responders and a

Introduction: Cicatricial alopecia (CA) is a group of disorders that lead to permanent hair loss due to irreversible damage to hair follicles. The aim of this study is to examine clinical and demographic characteristics of patients diagnosed with primary lymphocytic cicatricial alopecia (PLCA).Methods: Patients admitted to our outpatient clinic between 2000 and 2010 and who were diagnosed with PLCA were retrospectively evaluated for demographic characteristics, dermatologic findings, personal and family history, histopathologic results, therapeutic options given to the patients, and patient responses to the treatments.Results: Thirty-six patients were included in this study. Histopathological examination of scalp hair samples revealed that findings were concordant with lichen planopilaris (LPP) in 26 patients, discoid lupus erythematosus (DLE) in 7 patients, pseudopelade de Brocq (PB) in 3 patients. The main first-line treatment most commonly reported was intralesional corticosteroid injection (ILCS, n=16, 44.4%), which was followed by hydroxychloroquine (n=10, 28%). Nineteen of the 36 patients responded to the first-line treatments, whereas 17 patients needed another treatment. Treatments that patients were most likely to respond to were hydroxychloroquine and ILCS for LPP patients; hydroxychloroquine for DLE patients; hydroxychloroquine, systemic corticosteroid, and topical corticosteroid treatments for PB patients.Conclusion: PLCA is an entity widely observed in middle-aged women and is mostly encountered in the form of LPP. In the current study, patients most likely benefited from ILCS and hydroxychloroquine treatments.

Neurofibromatosis type 1 (NF1) is the most common neurogenetic disorder worldwide, and its clinical presentations are highly variable. NF1 is caused by mutations in the NF1 gene, and 50% of NF1 cases are sporadic, which occur in the absence of a family history of the disease and usually result from a new mutation in the germline of a parent. Advanced paternal age may increase the risk for germinal NF1 mutations; however, some dominant conditions, including neurofibromatosis, have shown a lesser association with paternal age, although there are conflicting reports in the literature. We investigated the effects of paternal and maternal age in 241 NF1 patients (121 sporadic and 120 familial cases) who were seen in Hacettepe hospital, a reference center for genetic diseases in Turkey. For statistical analysis, Spearman’s and Chi-square tests were used. In this study, we evaluated paternal and maternal age at birth in sporadic and familial cases of NF1. We also compared the effect of parental age on the appearance and coexistence of various NF1 symptoms. There were no significant statistical differences between paternal age and coexistence of the NF1 symptoms. However, a slightly negative correlation was observed between paternal age and the coexistence of NF1 symptoms in familial cases (p

Alopecia areata (AA) is a common disease that results in nonscarring hair loss. Recently, tofacitinib (TOFA) has been a novel promising therapy for AA. The aim of this study is to determine the efficacy of oral TOFA treatment for alopecia areata (AA), and alopecia universalis (AU). Data of nine patients treated with oral TOFA with either AA or AU were retrospectively evaluated. Treatment outcome, disease severity, and therapeutic response were evaluated by Severity of Alopecia Tool (SALT) scores along with regular photographic surveillance done at third and sixth months. Treatment response was established on four categories: complete response (90% change in latest SALT score), intermediate response (51-90% change), moderate response (6-50% change), and nonresponse (

Background: Evidence regarding the vascular endothelial growth factor A (VEGFA) as a potent mediator of angiogenesis and inflammation in psoriasis has revealed variations in this gene as surrogate markers of psoriasis. Objective: VEGFA gene polymorphisms (−1540 C/A, −1512 Ins18, −460 T/C, and +405 C/G) in psoriasis susceptibility in Turkish population were investigated. Methods: A total of 200 age, sex and ethnicity-matched psoriatic and healthy individuals were examined for clinical type, response to therapy, serum VEGFA and its receptor levels, genotypes and haplotypes. Results: The +405 GG, +405 CG, −1540 CA, and −1512 +Ins18 genotypes conferred a significant risk for developing psoriasis. The C-InsTC haplotype in the controls and C+InsTG, A+InsTC, and A-InsTG haplotypes in psoriatic patients were observed to be significantly high. Increased serum levels of VEGFA were detected in psoriatic patients with the C-InsTC haplotype than that in the controls. The +405 GG genotype was significantly more frequent in psoriatic patients with a positive family history, and the moderate form of psoriasis was more frequent among C+InsTG haplotype carriers than that among the other patients. The +405 GG genotype was found to be more frequent in patients responding to oral retinoids. Serum VEGFR1/FLT1 and VEGFR2/KDR levels were not significantly different when psoriatic patients and controls were stratified based on the risk polymorphic variants. Conclusion: VEGFA gene +405 GG and CG, −1512+Ins18, and −1540 CA genotypes are associated with an increased risk of psoriasis in Turkish population. The G allele at +405 and an 18-bp insertion at −1512 are primarily the risk factors for psoriasis, and this risk is potentiated by the presence of the A allele at the −1540 locus. (Ann Dermatol 28(1) 30∼39, 2016)

Neurofibromatosis type 1 (NF1) is an autosomal dominant disease that is caused by mutations of the NF1 gene. NF1 is clinically characterized by neurofibromas, pigmentation anomalies, and an increased risk of malignant tumors. The mutation rate of NF1 is one of the highest known for human disorders: approximately 50% of all affected individuals are sporadic cases and carry de novo mutations Therefore mutation analysis of NF1 may be an important tool in early diagnosis and genetic counseling. This is the first large NF1 study performed in Turkey. The data collected in this study enabled us to overview the genetic and clinical aspects of NF1 molecular diagnostics. The patients, who were clinically diagnosed for NF1, were included in this study. These patients were clinically evaluated, and subgroup of them genotyped or DNA sequenced for mutations in NF1, either to confirm the clinical diagnosis or to identify pathogenic mutations. The mutation detection rate was 52%, based on analysis of only genomic DNA. We observed that frameshift mutations were the largest proportion of the identified mutations (38.5%). The frequency of microdeletions was 26.9% and the splice site and nonsense mutations were 11.5% in this cohort. Turkish NF1 patients have similar NF1 germline mutations compared to other populations. Considering that some of these detected mutations belonged to the patients who did not fulfill the NIH criteria for NF1 diagnosis, mutation analysis of NF1 is an important tool in early diagnosis and genetic counseling., Hacettepe University Scientific Research and Development Office [H.U.BAB. 010 T02 102]

BACKGROUND The negative effect of genital and extragenital warts on adult patient quality of life (QoL) is well known; however, the literature lacks data on the effect of extragenital warts on Turkish pediatric patient QoL. The aim of this study was to determine the effect of extragenital warts that persist for ≥6 months on Turkish pediatric patient QoL, as well as to determine the relationship between patient demographic and clinical characteristics, and QoL. METHODS The Pediatric Quality of Life Inventory Version 4.0 (PedsQLTM 4.0) was administered to 85 children and their parents (patient group), and 85 age- and gender-matched children without any skin disease and their parents (control group). Children`s Dermatology Life Quality Index (CDLQI) was administered to the patients. Higher CDLQI and PedsQLTM are indicative of lower QoL. RESULTS Median wart duration and median age at the time of wart onset was 12 months (range 6-84) and 10 years (range 1-16), respectively. In the patient group mean (±Standard deviation [SD]) CDLQI score was 5.20 ± 5.97, and warts had the greatest negative effect on CDLQI symptoms and feelings scores. Mean (±SD) PedsQLTM total score was higher in the affected patients than that for the controls (23.42 ± 12.33 versus 15.81 ± 7.37, P < 0.001), and school, social and emotional functionality subscales exhibited the greatest differences between these groups. Mean (±SD) PedsQLTM total score for the patients` parents was higher than that for the controls` parents (25.94 ± 12.49 versus 17.81 ± 6.87, P < 0.001), and social and emotional functionality subscales exhibited the greatest difference between these groups. CONCLUSIONS The findings show that Turkish children with warts that persist for ≥6 months had lower QoL than the controls.

Background and Design: Alopecia areata (AA) is a frequent type of acquired hair loss with a sudden onset. The aim of this study was to investigate the epidemiological and clinical features of AA and diseases associated with AA in the Turkish pediatric population. Materials and Methods: We retrospectively evaluated the medical data of patients under 16 years of age who were admitted to the pediatric dermatology outpatient clinic at Hacettepe University Hospital between 2006 and 2011 with a diagnosis of AA, alopecia totalis (AT) and alopecia universalis (AU). Results: A total of 89 patients (34 females and 55 males), were included in this study. The mean age of the subjects was 9.8 +/- 3.4 years (range:18 months-16 years). 9% (n=8) patients had other autoimmune diseases: Hashimoto's thyroiditis in 5, vitiligo in 1, juvenile rheumatoid arthiritis (JRA) in 1 and morphea in 1 patient. AA was located on the scalp in 86.5% (n=77) of the patients, 7.9% (n=7) of the patients had AA on the scalp together with eyelashes, eyebrows and the body. AT was noted in 1, AU was noted in 4 patients. Severity of hair loss was

Background Recurrent aphthous stomatitis (RAS) is a common disease of the oral mucosa with an unknown etiology. This study aimed to determine if food additives play a role in the etiology of RAS as well as to determine if patch testing can be used to detect which allergens cause RAS. Methods This prospective study included 24 patients with RAS and 22 healthy controls. All the participants underwent patch testing for 23 food additives. Results In total, 21 (87.5%) RAS patients and 3 (13.6%) controls had positive patch test reactions to ≥1 allergens; the difference in the patch test positivity rate between groups was significant (P < 0.05). The most common allergen that elicited positive patch test results in the patient group was cochineal red (n = 15 [62.5%]), followed by azorubine (n = 11 [45.8%]) and amaranth (n = 6 [25%]). Conclusions The present findings show that food additives might play a role in the etiology of RAS and that patch testing could be a method for determining the etiology of RAS.

Background/Objective Diaper dermatitis (DD) is a common infantile dermatosis with a highly variable prevalence and incidence. This study aimed to present the demographic and clinical features of babies with DD. Methods Data, including demographic and clinical features, obtained from DD patient forms were retrospectively analyzed. Results The study included 63 babies with DD (female: n = 35 [55.6%]; male: n = 28 [44.4%]; mean age 11.6 mos). Most of the patients (43.5%) were 0 to 6 months of age. In all, 76.3% were breastfed, of whom 31.7% were exclusively breastfed. The number of previous episodes of DD was significantly lower in breastfed babies. The most common diaper area cleansing method was wet wipes (54.1%). Diaper creams were used in 86.2% of patients. DD was severe in 22.2% of patients, moderate in 57.4%, and mild in 20.4%. Candida infection was noted in 77.4% of patients. The median number of previous episodes of DD was significantly higher in patients with Candida infection than in those without (p = 0.02). Treatment yielded greater than 50% improvement (complete and moderate response) in 59.4% of patients at the first follow-up visit (3–5 days) and 82.6% at the second follow-up visit (10 days). Conclusion Most babies with DD were 0 to 6 months of age. Breastfed babies had fewer previous episodes of DD, so mothers should be encouraged to breastfeed their babies to reduce the incidence of DD. Candida infection was common; clinicians should be aware of its association with DD.

Narrowband ultraviolet B (UVB) provides complete response (CR) in 54-91% of early mycosis fungoides (MF) patients. Data concerning relapse rate and relapse-free interval after discontinuation of therapy need clarification. The purpose of this study was to evaluate the relapse rate and the relapse-free intervals of early MF patients after achieving complete response with narrowband UVB phototherapy.Retrospective evaluation of data, which belong to early MF patients treated with narrowband UVB phototherapy between May 2000 and July 2010, and followed-up until May 2012 in a single institution, for the occurrence of relapse and the time to relapse.Of 31 patients, who were followed-up for a mean of 56.5 ± 30.2 months (median 55 months, range 20-120 months), relapse was observed in 11 (35.5%) patients, within a mean of 28.8 ± 18.2 months (median 33 months, range 4-59 months), whereas 20 (64.5%) patients stayed relapse-free for a mean of 54.2 ± 28.8 months (median 55.5 months, range 20-119 months). Patients received maintenance phototherapy with a median duration of 12 months (range 1-30 months) after achieving complete response.Results indicate that narrowband UVB phototherapy may induce low relapse rates and long relapse-free intervals for early MF.

Pityriasis lichenoides (PL) is not uncommon in childhood, but current knowledge about the efficacy of oral erythromycin therapy for its treatment in children is limited. To investigate the role of oral erythromycin therapy in the treatment of PL in children, the records of 24 children with PL who had been started on oral erythromycin treatment at our institution between 2005 and 2008 were retrospectively reviewed. The study included 24 patients (14 male, 10 female) with a median age of 7 years (range 2-14) of whom 15 (62.5%) had PL chronica (PLC), six (25%) PL et varioliformis acuta (PLEVA), and three (2.5%) PLEVA-PLC overlap. History of upper respiratory tract infection was reported in 33% (n = 8) of the patients. History of drug intake and vaccination was noted in 20% (n = 5) and 20% (n = 5), respectively. The disease began during spring (30%, n = 7) or fall (30%, n = 7) in the majority of patients. The median duration of the disease was 11 months (range 1-48 months). Fifteen (68.2%) patients had more than 100 lesions. Distribution was diffuse in 82% (n = 18) of the cases and peripheral in the remainder (n = 6). Oral erythromycin was started at a dosage of 30 to 50 mg/kg per day in three to four divided dosages for 1 to 4 months. Good response was recorded in 64% and 73% of patients in the first and second months of therapy, respectively. Response rate rose to 83% in the third month. In those for whom follow-up data were available (n = 16), relapse was recorded in 12.5% (n = 3). Oral erythromycin may be an effective and well-tolerated treatment option for PL in children and should be continued for at least 3 months.

We describe seven Turkish children with DOCK8 deficiency who have not been previously reported. Three patients presented with typical features of recurrent or severe cutaneous viral infections, atopic dermatitis, and recurrent respiratory or gastrointestinal tract infections. However, four patients presented with other features. Patient 1-1 featured sclerosing cholangitis and colitis; patient 2-1, granulomatous soft tissue lesion and central nervous system involvement, with primary central nervous system lymphoma found on follow-up; patient 3-1, a fatal metastatic leiomyosarcoma; and patient 4-2 showed no other symptoms initially besides atopic dermatitis. Similar to other previously reported Turkish patients, but in contrast to patients of non-Turkish ethnicity, the patients' lymphopenia was primarily restricted to CD4(+) T cells. Patients had homozygous mutations in DOCK8 that altered splicing, introduced premature terminations, destabilized protein, or involved large deletions within the gene. Genotyping of remaining family members showed that DOCK8 deficiency is a fully penetrant, autosomal recessive disease. In our patients, bone marrow transplantation resulted in rapid improvement followed by disappearance of viral skin lesions, including lesions resembling epidermodysplasia verruciformis, atopic dermatitis, and recurrent infections. Particularly for patients who feature unusual clinical manifestations, immunological testing, in conjunction with genetic testing, can prove invaluable in diagnosing DOCK8 deficiency and providing potentially curative treatment.

Objective: Leukoclastic vasculitis is an inflammatory disease of the small vessels which frequently presents with skin and joint signs. The aim of the present study is to determine the clinical and demographical characteristics of the patients diagnosed as leukocytoclastic vasculitis in our clinic. Materials and Methods: Patients diagnosed to have leukocytoclastic vasculitis in our clinic between 2000 and 2009 were included in the study. Demographic characteristics of the patients as well as localization and type of the cutaneous lesions, etiology, symptoms, presence of extracutaneous symptoms, histopathological evaluation and treatment options, were retrospectively evaluated.Results: Median age of the 60 patients (36 female, 24 male) was 44 years. Pruritus (25%, n=15) and pain (18.3%, n=11) were the most frequent symptoms. Cutaneous lesions were most frequently localized in the lower extremity and usually presented as palpable pruritic papules and plaques. The most frequent etiologic factor was drugs (25%, n=15). Systemic corticosteroids were the most frequently used therapy of choice (38%, n=23). Conclusion: Leukocytoclastic vasculitis is a benign self limited disease which is frequently triggered by drugs. The most frequent clinical form is palpable pruritic papules or plaques localized in the lower extremity. Defining the etiologic cause is an important step to therapy, while systemic corticosteroid is an effective therapeutic choice.

Ectrodactyly, ectodermal dysplasia, clefting (EEC) syndrome is the prototype of several p63 conditions, which include ankyloblepharon, ectodermal dysplasia, clefting (AEC) syndrome, limb-mammary syndrome (LMS), Rapp-Hodgkin syndrome (RHS), ADULT syndrome, and others. All these disorders include combinations of ectodermal dysplasia, orofacial clefting and limb malformations in variable severity. A newborn patient is presented with diffuse erythematous and desquamating skin lesions and anal atresia. She also had sparse and lightly colored thin hair, deeply set eyes, hypoplastic alae nasi, and a short philtrum. Cleft lip/palate and ankyloblepharon were not present. Complete cutaneous syndactyly was present on both hands in between the third and fourth fingers. Mild ectrodactyly was evident on all four extremities in between first and second digits. There was post-axial polydactyly on both feet. Anal atresia was present and defecation occurred through a rectovaginal fistula. The patient represented an interesting overlapping clinical condition between AEC and EEC syndromes. Diffuse skin lesions with excoriation and desquamation suggest AEC syndrome, despite the absence of ankyloblepharon, however; ectrodactyly and polydactyly strongly suggest the EEC syndrome. C308Y mutation in exon 8 of TP63 gene was detected, which was previously described to lead only to EEC syndrome and not to any of the other allelic conditions. These data emphasize the large degree of clinical variability that may be seen for specific TP63 mutations.

Leiomyosarcoma and epidermodysplasia verruciformis are rarely encountered in children. The association of either leiomyosarcoma or epidermodysplasia verruciformis with immune deficiency has previously been documented. A 6-year-old girl, who has had multifocal leiomyosarcomatosis after the previous diagnoses of epidermodysplasia verruciformis and immune defect, represents an interesting association of these features.

Current knowledge about the efficacy of narrowband UVB therapy for the treatment of pityriasis lichenoides is limited.The aim of this study was to investigate the efficacy of narrowband UVB therapy for the treatment of pityriasis lichenoides chronica (PLC).Data were retrospectively collected following a review of patient phototherapy and medical charts, and telephone interviews were performed for follow-up information.The study included 25 patients (14 male, 11 female) with a mean age of 34 +/- 13 years. Diagnosis was confirmed by histopathological examination in 92% (n = 23) of the patients. The median duration of the disease was 24 months (range: 2-192 months). The median number of sessions until response was 25 (8 weeks) (range: 9-77 sessions), with a median cumulative dose of 15 J/cm(2) (range: 2-158 J/cm(2)). Complete response and partial response were achieved in 48% and 44% of the patients, respectively, while 8% of the patients achieved no response. In those patients for whom follow-up data were available (n = 17), 10 (58%) relapsed within a median of 9.5 months (range: 1-44 months).Our results suggest that narrowband UVB is an effective and well-tolerated treatment option for PLC.

Background Pityriasis lichenoides (PL) occurs in all age groups, although predominantly in younger individuals. Objective We sought to study the clinical features of PL in children followed up at our institution. Methods The records of 124 children who were given the diagnosis of PL at our institution between 1993 and 2003 were retrospectively reviewed. Results PL chronica (PLC) was recorded in 37% of the cases, PL et varioliformis acuta (PLEVA) in 57.3%, and clinical features of both disorders were seen simultaneously in the remaining. The median age of onset was 60 months (range: 6-180 months), although the median age of onset of PLEVA (median: 60 months) was significantly younger than that of PLC (median: 72 months) ( P = .03). The age distribution showed peaks at 2 to 3 years (24.8%) and 5 to 7 years (32%). A history of infection or drug intake preceded the skin manifestations in 30% and 11.2% of patients with PLC and PLEVA, respectively. The disease began most commonly during winter (35%) or fall (30%). The median duration was 20 months (range: 3-132 months) in patients with PLC and 18 months (range: 4-108 months) in patients with PLEVA. Involvement was diffuse in 74.2% of the patients, peripheral in 20.2%, and central in the remainder. The disease was recurrent in 77% of the patients (n = 80). Of the patients, 59% had pruritus, whereas 32% reported no symptoms; the remainder had fever, arthralgia, or both. Erythromycin estolate or ethylsuccinate was administered to 79.7% of the affected children; 66.6% of these showed at least a partial response. Limitations The analyzed data were collected retrospectively and biopsies were not performed in all patients. Conclusions PL is not an uncommon disease in childhood, with age peaks in the preschool and early school-age years. It is usually recurrent, and shows a seasonal variation with onset most often in the fall or winter. In childhood PL, erythromycin is an effective initial treatment choice.

Bullous pemfigoid (BP), an autoimmune disorder, can also be induced by some medications. Vildagliptin is a new drug used to treat diabetes mellitus (DM). Recently, a few cases of vildagliptin-induced BP have been described in the literature. We report a patient with BP in which vildagliptin was thought to be as a possible causative agent. The awareness of BP development risk during gliptin therapy can prevent unnecessary usage of systemic drugs with serious side effects.

Since the introduction of biologic therapies for tuberculosis (TB), screening for latent TB infection has increased in importance, especially in countries in which TB is endemic. The aim of this study was to evaluate the effect of psoriasis on tuberculin skin test (TST) results and to compare two TB screening tests, the TST and QuantiFERON(®)-TB Gold In-Tube (QFT-GIT) test, in psoriasis and psoriatic arthritis (PA) patients living in a TB-endemic country (Turkey). This prospective study included 61 psoriasis and 40 PA patients, and 58 healthy controls. Demographic data, medical history, human immunodeficiency virus (HIV) status, level of education, smoking status, exposure to TB, personal and family histories of TB, and bacillus Calmette-Guerin (BCG) vaccination status were recorded for all participants. The TST and QFT-GIT were performed in all participants. The mean ± standard deviation TST indurations in the patient and control groups were 12.6 ± 6.4 mm and 10.2 ± 6.5 mm, respectively (P = 0.051). The TST positivity rate was higher in patients than in controls (86.1% vs. 37.9%; P < 0.001), whereas QFT-GIT positivity did not differ significantly (patients: 20.8%; controls: 17.2%; P = 0.737). False positive results can lead to unnecessary prophylactic TB treatment; therefore, the cut-off point for TST positivity in psoriasis and PA patients should be re-evaluated, or other tests, such as the QFT-GIT, should be used.