Klinefelter‐Syndrom mit Mischkollagenose (Sharp‐Syndrom) und postthrombotischem Syndrom bei Thrombophilie

A 43‐year‐old male with eunuchoid body proportions and a history of deep venous thromboses in the right leg presented with recurrent ulcers in the right perimalleolar region for 6 years. Karyotyping revealed a 47 XXY Klinefelter's syndrome, while serologic testing showed protein S deficiency, hyperhomocysteinemia and positive lupus anticoagulant. He also had mixed connective tissue disease (Sharp's syndrome) with acrosclerosis, proximal finger edema, Raynaud's phenomenon, and high titers of ANA and U1‐RNP‐antibodies, as well as osteoporosis. There is evidence that patients with Klinefelter's syndrome are prone to develop connective tissue diseases and thrombophilia as a result of low androgen levels. Substitution of testosterone in Klinefelter's syndrome can have a favorable therapeutic effect on the associated connective tissue disease, thrombophilia and osteoporosis.     

Oral etoposide as first‐line therapy in the treatment of patients with advanced classic Kaposi’s sarcoma (CKS): a single‐arm trial (oral etoposide in CKS)

Background Classic Kaposi’s sarcoma (CKS) affects an elderly population; it is important to have effective treatment options with high activity and relatively low toxicity, and availability to be used for long periods. Objective We investigated the activity and safety of single‐agent etoposide with an oral administration schedule in patients with advanced CKS. Methods Histologically confirmed, CKS patients were eligible for study. All had a negative test for HIV and good performance status. All patients received oral etoposide 50 mg twice daily for 10 days every 3 weeks. Results Thirty patients (median age 66 and 22 males) were enrolled into the study. The majority of them had non‐metastatic, local advanced disease and symptoms in nearly half of patients. Complete and partial responses were observed in 10% and 77% of patients, respectively, giving an overall response rate of 87%. Stable disease occurred in the other 13% of patients. Treatment was well tolerated. Grade IV toxicity was not observed. Haematological toxicity was the principal dose‐limiting side effect. Severe leucopaenia and neutropaenia were observed in 7% and 10% of patients respectively. No patient was complicated by febrile neutropaenia. Mild‐moderate anaemia observed frequently, but only 3% of patients had severe anaemia and severe thrombocytopaenia was not observed. The 5‐year overall survival rate was 92%. Conclusions Single‐agent oral etoposide is an effective treatment option and is acceptably toxic and easily administered. Therefore, we recommend the single agent of oral etoposide as the first‐line chemotherapy for advanced CKS.     

Methicillin‐resistenter Staphylococcus aureus (MRSA) in chronischen Wunden: Therapeutische Optionen und Perspektiven

The contamination of chronic wounds with Methicillin‐resistant Staphylococcus aureus (MRSA) represents a world‐wide increasing problem. Although it is still unclear whether bacterial contamination is a relevant factor for delayed wound‐healing, verification of MRSA contamination has significant logistic consequences for the medical institution as well as for the patient. In particular, if MRSA contamination progresses towards a systemic infection, options for antibiotic therapy are greatly limited. In this article, we discuss current therapeutic standards and potential alternatives for eradication of MRSA. There is evident need for effective, novel approaches for elimination of MRSA from chronic wounds that avoid the development of bacterial resistance; otherwise therapeutic alternatives for antibacterial treatment of chronic wounds will become more limited.     

Photodynamische Therapie mit Methylaminooxopentanoat (Metvix®) und einer Breitbandlichtquelle (PhotoDyn 501): Praktische Erfahrungen bei Problem‐Patienten mit Aktinischen Keratosen und Basalzellkarzinomen

Background: Actinic keratoses (AK) and basal cell carcinomas (BCC) may represent a therapeutic challenge because of special subtypes, location, previous therapy or accompanying diseases. Photodynamic therapy (PDT) offers a semi‐conservative treatment option for selected indications. Patients and methods: 28 outpatients who had been admitted as complicated dermato‐oncologic cases because of AK (n = 22) and BCC (n = 6) were treated with PDT, using methylaminooxopentanoate (MAOP, Methyl‐Ala, Metvix®) and a broad band light source (PhotoDyn 501). The treatment was evaluated for efficacy and subjective tolerance (local discomfort and pain). Results: A complete remission (CR) was achieved in 11/22 AK (50 %) and 4/6 BCC (67 %) cases. All three cases of a superficial BCC subtype underwent a CR. Among responders, tolerance was good in 12/15 cases (80 %), as compared to 4/13 cases (31 %) in non‐responders. Focusing on 16/28 patients with good tolerance (57 %), there was a CR in 12 cases (75 % rate), whereas for the 12/28 patients with moderate to poor tolerance a CR was achieved in only 3 cases (25 % rate). In a subgroup of 8 patients who, partly due to secondary diseases, were taking systemic retinoids or immunosuppressive‐cytostatic medications, a CR was achieved in 3/8 cases (38 %) with a good tolerance in only 1/8 cases (13 %). Conclusion: These observations confirm a good efficacy and tolerance of PDT in ≥ 50 % of a AK/BCC problem patient cohort. We found indications for 1) a positive correlation between efficacy and subjective tolerance as well as 2) the presumptive existence of a retinoid‐dependent cutaneous PDT hyperalgesia. Effective pain control seems to be an essential cofactor for the success of PDT.     

High‐concentration (20 μg g−1) tacalcitol ointment in the treatment of facial psoriasis: an 8‐week open‐label clinical trial

Background Facial psoriasis gives rise to considerable concern because of associated cosmetic problems and psychosocial distress. It requires a treatment approach other than topical corticosteroids, which bear a risk of cutaneous adverse reactions. Recently, topical tacalcitol has been shown to be effective in psoriasis. Objectives The aim of this open‐label single‐centre study is to investigate the efficacy and safety of high‐concentration (20 μg g^?1) tacalcitol ointment (Bonalfa‐high^®, Teijin Pharma, Tokyo, Japan) in patients with facial psoriasis and to evaluate clinical response according to the distribution of facial psoriatic lesions. Patients and methods Thirty‐seven patients were enrolled to this clinical trial. Tacalcitol 20 μg g^?1 ointment was applied once daily to psoriatic lesions of the face over an 8‐week period. Patients were also categorized into three subtypes according to facial lesion distribution. Efficacy was evaluated by the facial Psoriasis Area and Severity Index (facial PASI) and the Physician’s Global Assessment (PGA) score at weeks 2, 4 and 8. The Subjective Global Assessment (SGA) was also determined at the end of the study. Results Thirty‐three patients completed the clinical trial. Mean facial PASI of 33 patients at baseline was 9·58 and after 8 weeks of treatment the mean facial PASI decreased significantly to 3·88. By using PGA, patients showed the following responses to treatment: clearance (n = 1); excellent (6); good (16); fair (4); slight (5); no change (1). The response rate among the three facial psoriasis types showed no difference. Using the SGA, 27 (82%) of the patients presented excellent (15%) or good (67%) effect with tacalcitol 20 μg g^?1 ointment. No serious adverse reactions were observed. Conclusions This is the first clinical study reporting a relevant therapeutic effect and favourable safety profile of tacalcitol 20 μg g^?1 ointment in facial psoriasis. These results suggest that tacalcitol 20 μg g^?1 ointment can be used as the first‐line treatment in patients with facial psoriasis.     

CLinical Experience Acquired with Raptiva® (CLEAR) trial in patients with moderate‐to‐severe plaque psoriasis: results from extended treatment in an international,Phase III,placebo‐controlled trial

Background: The 12‐week, double‐blind, placebo‐controlled, first‐treatment (FT) CLEAR trial period demonstrated the efficacy/safety of efalizumab in moderate‐to‐severe plaque psoriasis, including refractory or contraindicated patients unsuitable for other systemic treatments. This study assessed the efficacy/safety of open‐label extended treatment (up to 24 weeks' continuous treatment) in patients not achieving ≥75% improvement in Psoriasis Area and Severity Index (PASI‐75) at week 12 of the FT period. Time to relapse after treatment cessation, and efficacy/safety of 12 weeks' open‐label re‐treatment in patients achieving PASI‐75 at week 12 FT were also assessed. Patients and methods: Patients with PASI‐75 at week 12 FT were observed without treatment until relapse, then re‐treated with open‐label efalizumab (1.0 mg/kg/week for 12 weeks). Others received open‐label extended treatment without intervening observation. Results: Among efalizumab‐treated patients (n = 308) who had < 75% PASI improvement at week 12 FT, extended treatment led to PASI‐75 in 26.6%. Among patients with between ≥ 50 and < 75% PASI improvement at week 12 FT (n = 118), 47.5% improved to PASI‐75 with extended treatment. Forpatients achieving PASI‐75 at week 12 FT (n = 164), median time to relapse was 58 days. Re‐treatment after relapse led to mean PASI improvement of 62.3% from study baseline (n = 145). Safety results were consistent with pre‐vious studies, with no new safety concerns. Conclusions: These results demonstrate additional benefit of continuing efali‐zumab. Re‐treatment re‐established disease control in patients with PASI‐75 who relapsed following treatment cessation. The safety profile remained consistent with that seen at 12 weeks.     

Towards an international language for incontinence‐associated dermatitis (IAD): design and evaluation of psychometric properties of the Ghent Global IAD Categorization Tool (GLOBIAD) in 30 countries

The best way to prevent and treat diaper dermatitis (nappy rash) in babies has been the topic of research for decades. However this skin problem can also occur in adults, where it is known as incontinence‐associated dermatitis (IAD). It is a specific type of irritant contact dermatitis ‐ a type of eczema caused when contact with something causes irritation (rather than an allergy), in this case prolonged contact of the skin with urine or faeces. It is hard to know exactly how many people suffer from IAD as there is not one set of internationally agreed diagnostic criteria (e.g. a list of specific symptoms and criteria that identify the disease) that help differentiate it from other skin disorders with similar symptoms, such as pressure ulcers. Ten different instruments / tools (meaning ways of diagnosing and ranking the severity of the disease) exist, but some are complex, and a single one is needed to make sure study results are comparable. Therefore, a group of 34 experts in 13 countries worked together to develop a tool that was started in 2015, called GLOBIAD. It was then translated into 14 different languages. Its accuracy was tested by asking 823 experts in 30 countries to categorize 34 photos of IAD using GLOBIAD to see if their results matched. The study found that the development of the GLOBIAD is a major step towards a better systematic assessment of IAD in clinical practice and research worldwide. However, further validation is needed.