http://www.medicaljournals.se/acta/ Acta Dermato-Venereologica is an international peer-review journal for clinical and experimental research in the field of dermatology and venereology. http://www.medicaljournals.se/acta/content/?doi=10.2340/00015555-1734
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Atypical teratoid/rhabdoid tumour is a rare and highly malignant tumour of the posterior fossae nervous system that occurs in children especially in the first few years of life. Cutaneous location is not previously reported. A newborn boy was referred for both aqueductal stenosis detected antenatally and skin tags mimicking hamartoma. The cerebral tumour increased in size during a few months leading to both skin and cerebral biopsies. Integrase Interactor-1 (INI-1) immunostaining and tumoural and leukocytes INI-1 gene sequencing confirmed the atypical teratoid/rhabdoid tumour nature of the cerebral tumour. INI-1 immunostaining in skin biopsy confirmed the dermal location of rhabdoid tumour. Thus, unusual cutaneous lesions may be part of atypical teratoid/rhabdoid tumour. The loss of Integrase INI-1 on immunohistochemical staining is characteristic.]]> 2013-11-05 Clinical http://www.medicaljournals.se/acta/content/?doi=10.2340/00015555-1690
Self-harm of the skin is a complex problem encountered mainly in adolescents and young adults. The aim of the study was to assess the prevalence of deliberate cutaneous self-harm without suicidal intent among secondary school teenagers of the Lodz region. A self-administered specially designed anonymous questionnaire was delivered to 1,448 secondary school teenagers, aged 12–19 years. The lifetime prevalence of self-reported deliberate self-harm was 19.5%, out of which 14.4% confirmed isolated cutaneous self-injury (self-cutting in the vast majority of cases), 1.7% ingested a substance or drug in excessive amounts and 3.5% declared both behaviours. Our results indicate that skin is the organ most commonly involved in deliberate self-harm. Dermatologists, especially those focussed on dermatosurgery and aesthetic dermatology, should understand the special issues relating to such patients before taking decisions concerning performing any procedures on these individuals, since deliberate self-harm has been recognised as one of the main risk factors of suicide. ]]> 2013-09-24 Clinical http://www.medicaljournals.se/acta/content/?doi=10.2340/00015555-1758
Defining the health-related quality of life (HRQoL) in patients suffering from the heritable blistering disease epidermolysis bullosa (EB) is important in assessing the efficacy of new treatments. The quality of life in EB questionnaire (QOLEB) is an English 17-item EB-specific HRQoL measurement tool. The aim of this study was to develop a validated and reliable QOLEB in Dutch and assess the HRQoL in Dutch EB patients. The QOLEB was translated to Dutch according to protocol. Fifty-five adult patients across 4 EB subtypes participated. The QOLEB had excellant correlation with the Skindex-29 (ρs = 0.86), good correlation with the SF-36 physical score (ρs = –0.75), and moderate correlation with the SF-36 mental score (ρs = –0.43). The discriminative validity between the 4 different EB subtypes was significant (p = 0.002). The internal consistency was excellent (α = 0.905), and the test–retest reliability strong (ρs = 0.88). In conclusion, the Dutch QOLEB is a reliable and valid instrument for the assessment of the HRQoL in adult EB patients.]]> 2013-12-11 Clinical http://www.medicaljournals.se/acta/content/?doi=10.2340/00015555-1743
Experiences from a Danish patient cohort with hereditary angioedema are reported with focus on home therapy
and burden of illness. Eighty patients have been prospectively followed over 11 years, having experienced a total of 7,809 attacks over 469 patient years. More than half of the patients stopped long-term prophylaxis with danazol or tranexamic acid and changed treatment regimen to on-demand treatment with C1 inhibitor concentrate or icatibant. At least 10% of the attacks remained un-treated. More than half of the patients felt that hereditary angioedema had a significant psychological impact on their lives and restricted their physical activities. By December 2012, a total of 39 patients (49%) were practicing home treatment of acute attacks. Home therapy reduced the mean number of acute hospital visits by 84% and significantly improved burden of illness items. In conclusion, home therapy has profoundly improved the lives of hereditary angioedema patients. ]]> 2013-10-24 Clinical http://www.medicaljournals.se/acta/content/?doi=10.2340/00015555-1771
Bowen’s disease is an in situ squamous cell carcinoma of the skin with various treatment modalities available. A major advantage of surgical excision is the opportunity to histologically examine the resection margins. There is no consensus about the most appropriate margin. This retrospective study evaluates the clearance rates achieved by excision with a 5 mm margin and estimates how that might change after fictitiously reducing the resection margin by 1 or 2 mm. Patients with histologically confirmed Bowen’s disease were selected at the Maastricht University Medical Centre from 2002 until 2007. Surgical margins and complete excision rates were evaluated and histological slides were re-examined. To our knowledge this is the first study investigating the safety margin for Bowen’s disease. As Bowen’s disease is not an invasive disease, minimisation of healthy tissue excision is desirable. Our data show that a hypothetical reduction
of the safety margin from 5 mm to 4 or 3 mm decreases the complete excision rate from 94.4% to 87% and 74.1%, respectively.]]> 2013-12-09 Clinical http://www.medicaljournals.se/acta/content/?doi=10.2340/00015555-1753
Phototherapy is an effective and widely used treatment for generalised plaque psoriasis. Despite the mutagenic effects of UVB this type of therapy is still assumed to be a safe treatment option. We have performed a cross sectional study to assess the risk of skin cancer in the cohort of psoriasis patients treated with UVB. A total of 162 white patients (116 men and 46 women, mean ± standard deviation age 56.0 ± 13.5 years) were included in the study. All patients have previously been treated with UVB at least 100 times in the last 5 years. Eight patients (4.9%, 0.95 CI: 2.2–9.5%) out of the 162 included in the study had histopathologically verified skin cancer. We found that the risk of skin cancer in psoriasis patients treated with UVB correlated with the number of treatments (controlling for age) but not with the type of UVB lamp. How-ever, the overall risk of malignancy in the UVB-treated patients was not greater than in the general population. ]]> 2013-11-28 Clinical http://www.medicaljournals.se/acta/content/?doi=10.2340/00015555-1741
A few key publications report on the frequency of skin disorders in paediatric organ transplant recipients in Southern and Central Europe presenting cumulative incidences. We aimed to estimate frequencies of skin disorders both as cumulative incidences and prevalence data, and describe skin problems in paediatric renal transplant recipients in a Norwegian renal transplant population. Clinical examination and review of post-transplant skin diseases were conducted in 70 patients having performed renal transplantation before the age of 16 in the period 1983–2006. Viral warts were a common and persistent problem, whereas bacterial and fungal infections in the skin were few. Drug-related skin disorders were rather frequent, but usually reversible on dose reduction or change of medication. Pre-malignant and malignant skin disorders appeared only in patients > 30 years of age. Relatively high cumulative incidences and low prevalence data of most skin disorders were found in the examined patient cohort. ]]> 2013-11-21 Clinical http://www.medicaljournals.se/acta/content/?doi=10.2340/00015555-1735
New treatment modalities are needed in atopic dermatitis. We evaluated the pharmacokinetics, safety, tolerability, and efficacy of topical cis-urocanic acid (cis-UCA) cream in randomised vehicle-controlled double-blinded clinical trials. The subjects received 5% cis-UCA emulsion cream and control vehicle on volar forearms after right–left randomisation. Study 1: 16 healthy subjects received one dose on the skin and, a week later, on DMSO-irritated skin. Study 2: 16 healthy subjects received 2 daily doses for 10 days. Study 3: 13 patients with mild to moderate disease were treated on selected skin lesions twice daily for 28 days. Study treatments were well tolerated. cis-UCA remained close to endogenous levels in plasma and urine. cis-UCA reduced transepidermal water loss (TEWL) both in healthy subjects and in the patients. Eczema area severity index and physician’s global assessment improved from baseline with both treat-ments. cis-UCA cream improved skin barrier func-tion and suppressed inflammation in the human skin.]]> 2013-11-11 Clinical http://www.medicaljournals.se/acta/content/?doi=10.2340/00015555-1731
The aim of this study was to assess quality of life in patients with scalp dermatitis using the Italian version of the Scalpdex, and to validate the instrument in Italian. The survey was conducted in outpatients with psoriasis, seborrhoeic dermatitis, alopecia, or follicular lichen. Data were completed on 194 patients, 78% of whom had psoriasis. Scalpdex scores were always higher in women than in men, and in younger people compared to elderly people. The most frequent items were: being ashamed, embarrassed, bleeding scalp, feeling self-conscious, bothered that the condition is incurable, having the choice of colour of clothes affected, having a negative effect on daily life. The Italian Scalpdex showed good internal consistency, test-retest reliability, convergent validity, and responsiveness. In conclusion, the Italian version of the Scalpdex is a useful instrument to measure quality of life in patients with a scalp condition. ]]> 2013-11-25 Investigative http://www.medicaljournals.se/acta/content/?doi=10.2340/00015555-1748
In a multicentre study consecutively patch-tested dermatitis patients were tested simultaneously with 1.0% and 2.0% (w/v) formaldehyde in aqua applied with a micropipette (15 µl) to the filter paper disc in Finn Chambers (0.30 mg/cm2 and 0.60 mg/cm2, respectively). A total of 2,122 dermatitis patients were patch-tested. In all, 77 (3.6%) patients reacted positively to formaldehyde; 37 reacted only to 2.0%, 35 reacted to both concentrations and 5 patients reacted only to 1.0%. Significantly more patients were thus diagnosed with contact allergy to formaldehyde with 2.0% compared to 1.0% (p < 0.001) without causing more irritant reactions. The detected number of isolated allergic reactions to the 2 formaldehyde-releasers in the Swedish baseline series and not to formaldehyde itself raises the question whether quaternium-15 1.0% and diazolidinyl urea 2.0% should be present in the Swedish baseline series. ]]> 2013-12-10 Investigative http://www.medicaljournals.se/acta/content/?doi=10.2340/00015555-1770
Wounds represent a growing healthcare problem due to an aging population. Nurses play a key role in wound management and their theoretical understanding of basic wound management may be expected to influence the quality of wound therapy fundamentally. In this study, we evaluated the level of knowledge of wound management in 136 Danish nurses working in 3 different settings: advanced wound care clinics, home care and general hospital departments. We found that hospital nurses had less theoretical knowledge than home care nurses and nurses working at advanced wound care clinics. We also found that the length of experience (adjusted for workplace and education) did not have any impact on nurses’ knowledge. Nurses’ knowledge of clinical investigations was consistently lower than their knowledge of therapy and clinical symptoms. This study provides benchmarking information about the current status of wound management in Denmark and suggests how improvements might be achieved. ]]> 2013-12-10 Investigative http://www.medicaljournals.se/acta/content/?doi=10.2340/00015555-1725
Atranol and chloroatranol are strong contact allergens in oak moss absolute, a lichen extract used in perfumery. Fifteen subjects with contact allergy to oak moss absolute underwent a repeated open application test (ROAT) using solutions of an untreated oak moss absolute (sample A) and an oak moss absolute with reduced content of atranol and chloroatranol (sample B). All subjects were in addition patch-tested with serial dilutions of samples A and B. Statistically significantly more subjects reacted to sample A than to sample B in the patch tests. No corresponding difference was observed in the ROAT, though there was a significant difference in the time required to elicit a positive reaction. Still, the ROAT indicates that the use of a cosmetic product containing oak moss absolute with reduced levels of atranol and chloroatranol is capable of eliciting an allergic reaction in previously sensitised individuals. ]]> 2013-11-20 Investigative http://www.medicaljournals.se/acta/content/?doi=10.2340/00015555-1764
Dermatitis herpetiformis (DH) is an extraintestinal manifestation of coeliac disease. Untreated coeliac disease patients are known to have transglutaminase 2 (TG2)-targeted IgA deposits in the small bowel mucosa. To evaluate whether similar intestinal IgA deposits are also present in DH and whether the deposits disappear with gluten-free diet, 47 untreated and 27 treated DH patients were studied. Seventy-nine percent of untreated and 41% of the treated DH patients had TG2-specific IgA deposits in the small bowel, and the presence of the deposits showed a significant association with the degree of small bowel villous atrophy (p < 0.001). Other coeliac-disease related inflammatory markers were also investigated, and the density of small bowel mucosal intraepithelial γδ+ T cells was increased in 91% of untreated and 73% of treated DH patients. The results show that the majority of untreated DH patients have similar gluten-dependent TG2-specific IgA deposits the small bowel mucosa as coeliac disease patients. ]]> 2013-12-11 Investigative http://www.medicaljournals.se/acta/content/?doi=10.2340/00015555-1717
Immune regulation of the skin plays an important role in susceptibility and development of illnesses. The aim of our study was to localise the interleukin (IL)-10 family of cytokines, in children’s skin and to determine possible age-related differences in the expression level. The mRNA expression level of IL10, IL19, IL20, IL22, IL24, IL26, IL28B, IL29 and their receptors IL10RA, IL10RB, IL20RA, IL20RB, IL22RA1, IL22RA2, IL28RA was compared in skin biopsies of children and adults and in childrens’ skin cells by quantitative real-time PCR (qRT-PCR). Immunohistochemistry was performed to confirm the qRT-PCR findings. We found age-related differences in the expression of IL10RB, IL20, IL20RA, IL22RA1, IL22RA2, IL26 and IL28RA genes. Cell type-dependent expression of IL10 family cytokines was apparent in the skin. In addition to previously known differences in systemic immunological response of adults and children, the present results reveal differences in immune profile of adult and juvenile skin. ]]> 2013-11-14 Investigative http://www.medicaljournals.se/acta/content/?doi=10.2340/00015555-1708
The non-involved, healthy-looking skin of psoriatic patients displays inherent characteristics that make it prone to develop typical psoriatic symptoms. Our primary aim was to identify genes and proteins that are differentially regulated in the non-involved psoriatic and the normal epidermis, and to discover regulatory networks responsible for these differences. A cDNA microarray experiment was performed to compare the gene expression profiles of 4 healthy and 4 psoriatic non-involved epidermis samples in response to T-cell lymphokine induction in organotypic cultures. We identified 61 annotated genes and another 11 expressed transcripts that were differentially regulated in the psoriatic tissues. Bioinformatics analysis suggested that the regulation of cell morphology, development and cell death is abnormal, and that the metabolism of small molecules and lipids is differentially regulated in psoriatic epidermis. Our results indicate that one of the early steps of psoriasis pathogenesis may be the abnormal regulation of IL-23A and IL-1B genes in psoriatic keratinocytes. ]]> 2013-12-16 Clinical http://www.medicaljournals.se/acta/content/?doi=10.2340/00015555-1737
Psoriasis is a common chronic inflammatory disease of the skin. Its pathogenesis has not been completely elucidated. Phosphoinositide-3 kinase/protein kinase-B/mammalian target of rapamycin (PI3K/Akt/mTOR) pathway has been identified as a key signaling pathway for important cellular functions. The data collected in this review suggest that overexpression of the PI3K/Akt/mTOR pathway may play an important role in the pathogenesis of psoriasis by mediating the immune-pathogenesis, the epidermal hyperplasia or/and the angiogenesis in the disease. Advances in understanding the pathogenesis of psoriasis has provided new insight into potential therapeutic targets, including the development of biological therapies, resulting in remarkable clinical responses in patients with severe psoriasis. More recently, small molecule oral preparations targeting intracellular signaling that may prove effective have been developed. Data suggest that PI3K/Akt/mTOR pathway may be a potential target for treatment of psoriasis.]]> 2013-10-23 Review http://www.medicaljournals.se/acta/content/?doi=10.2340/00015555-1628
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