R-spondins in cutaneous biology: nails and cancer.
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The R-spondins are a relatively recently identified family of secreted proteins linked to the Wnt signaling pathway. Recently, human phenotypes have been associated with mutations in two human R-spondins. Germline RSPO4 and RSPO1 mutations result in recessive anonychia (absence of all fingernails and toenails) and a recessive syndrome with XX-male sex reversal, PPK and a predisposition to SCC, respectively. This review discusses the key roles R-spondins play in embryogenesis, adult tissue maintenance and skin carcinogenesis. (+info)
Psoriatic onycho-pachydermo periostitis.
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A 46-year-old woman presented with onycholysis and swollen, painful digits. No other stigmata of psoriasis were present. Magnetic resonance imaging of the hands showed an extensive periosteal reaction of the phalangeal tuft. Psoriatic onycho-pachydermo periostitis (POPP) is a rare subset of psoriatic arthritis that is characterized by psoriatic onychodystrophy, connective-tissue thickening above the distal phalanx, and a periosteal reaction. Treatment of POPP is difficult; however, low-dose methotrexate and anti-TNF-alpha agents may be beneficial. In patients who are unresponsive or intolerant of these medications, other biologic and non-biologic disease modifying anti-rheumatic drugs need to be considered. (+info)
Health-related quality of life and nail disorders.
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Health-related quality of life (HRQoL) measurements in clinical practice ensure that treatment and evaluations focus on the patient rather than the disease. To verify whether these measurements are also relevant in a minor dermatological disease and whether a generic routine assessment is sufficient to capture different aspects of quality of life, we studied patients affected by nail disorders. The Skindex-29, the 12-item General Health Questionnaire and the 36-item Short Form of the Medical Outcomes Study questionnaires were used. A total of 114 patients were enrolled: 31% men aged 48 +/- 15 years and 69% women aged 49 +/- 14 years (mean +/- SD). The mean values of SF-36 scales in nail disorders were higher than those reported for minor skin diseases, and Skindex-29 values were lower. A duration of disease > or =1 year was associated with higher Skindex-29 scores on the symptom and emotions scales. Patients with onychomycosis have higher values on the Skindex-29 social functioning scale compared with other nail disorders. The use of HRQoL measurements in routine clinical activities allows us to depict how nail disorders affect patients and show how relevant some neglected aspects, such as pain or emotional trauma, are and offers us the opportunity to address these issues in a patient-centred approach to treatment. (+info)
Comparison of clinical features of HLA-Cw*0602-positive and -negative psoriasis patients in a Han Chinese population.
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HLA-Cw6 is strongly associated with psoriasis and has been suggested to be the PSORS1 gene that confers susceptibility to early-onset psoriasis. In this study of the clinical features of HLA-Cw*0602-positive and -negative psoriasis patients in a Han Chinese population, we typed HLA-C in a cohort of 679 patients and compared the two groups. Cw*0602-positive patients (n=345) had an earlier disease onset (p < 1 x 10(-5)), more severe disease (p < 1 x 10(-3)), higher frequency of guttate psoriasis (p < 1 x 10(-9)), more affected legs and trunk (p < 1 x 10(-5)), higher incidence of Kobner's phenomenon (p=0.005) and of trauma history (p=0.009). Cw*0602-negative patients (n= 334) had more palmoplantar pustulosis (p=0.004), nail changes (p=0.001) and scalp involvement (p=0.007). However, there was no statistically significant difference between the two groups regarding age, gender, incidence of plaque psoriasis, erythrodermic, inverse, psoriatic arthritis, and the precipitation factors stress and infection. The study showed that Cw*0602-positive patients had some obvious clinical differences from Cw*0602-negative patients in a Han Chinese population, which provides evidence for an HLA-Cw*0602-associated phenotype in psoriasis. (+info)
Idiopathic yellow nail syndrome successfully treated with OK-432.
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A 70-year-old female presented with yellow discoloration of the nail beds of all fingers and toes, as well as bilateral pleural effusions. The patient was diagnosed as having the yellow nail syndrome based on the triad of yellow nails, lymphedema, and pleural effusions. The patient's intractable bilateral pleural effusion was treated with pleurodesis using OK-432. The treatment prevented the accumulation of pleural fluid for a long period of time. Pleural effusion associated with yellow nail syndrome is thought to be difficult to treat; however, this patient's excellent clinical course suggests that pleurodesis with OK-432 could be used to treat the disease in the future. (+info)
Transungual iontophoretic transport of polar neutral and positively charged model permeants: effects of electrophoresis and electroosmosis.
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Transungual iontophoretic transport of model neutral permeants mannitol (MA), urea (UR), and positively charged permeant tetraethylammonium ion (TEA) across fully hydrated human nail plates at pH 7.4 were investigated in vitro. Four protocols were involved in the transport experiments with each protocol divided into stages including passive and iontophoresis transport of 0.1 and 0.3 mA. Water and permeant uptake experiments of nail clippings were also conducted to characterize the hydration and binding effects of the permeants to the nails. Iontophoresis enhanced the transport of MA and UR from anode to cathode, but this effect (electroosmosis) was marginal. The transport of TEA was significantly enhanced by anodal iontophoresis and the experimental enhancement factors were consistent with the Nernst-Planck theory predictions. Hindered transport was also observed and believed to be critical in transungual delivery. The barrier of the nail plates was stable over the time course of the study, and no significant electric field-induced alteration of the barrier was observed. The present results with hydrated nail plates are consistent with electrophoresis-dominant (the direct field effect) transungual iontophoretic transport of small ionic permeants with small contribution from electroosmosis. (+info)
First-line single-agent cetuximab in patients with advanced colorectal cancer.
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BACKGROUND: The anti-epidermal growth factor receptor (EGFR) antibody cetuximab is active in heavily pretreated patients with metastatic colorectal cancer (mCRC) both in monotherapy and in combination with chemotherapy (CT). This study assesses the antitumor activity of single-agent cetuximab in CT-naive patients. PATIENTS AND METHODS: Phase II clinical trial was used. Patients were EGFR positive by immunohistochemistry and were not candidate for radical surgery, even in the case of substantial tumor shrinkage. Cetuximab was administered weekly. RESULTS: Thirty-nine patients were treated and evaluated. The most common adverse event was skin toxicity (89% any grade; 48% grade 1; 31% grade 2; 10% grade 3). One patient had a complete response and three obtained partial responses (10% overall response rate). Thirteen patients had stable disease (34%). Twenty-two patients experienced progressive disease (56%). Overall median time to progression (TTP) was 2 months, and the responders individual TTP was 12, 9, 9, and 6 months. CONCLUSIONS: Even in chemo-naive patients, cetuximab as single agent is active only in a small fraction of mCRC, similarly to what has been reported for heavily pretreated patients. The extent of benefit when response occurs is, however, such that it is mandatory to intensify the search for the predictive markers of response to cetuximab therapy. (+info)
Matched case-control phase 2 study to evaluate the use of a frozen sock to prevent docetaxel-induced onycholysis and cutaneous toxicity of the foot.
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