IntroductionAnti-endothelial cell antibodies (AECA) recognize endothelial cell proteins and are thought to play an important role in vascular damage observed in systemic scleroderma (SSc) and many other autoimmune diseases. In SSc, AECA were found to be more common in patients with pulmonary hypertension, digital ulcers and nailfold capillaroscopic changes. Until now, there have been no studies examining the association between AECA positivity with the activity and duration of the disease.AimTo evaluate associations between the presence of AECA in sera of patients with SSc and internal organs involvement as well as disease activity.Material and methodsSera of 58 patients with SSc (50 with localized subtype and 8 with diffuse subtype) were examined for AECA presence using an indirect immunofluorescence technique. Several clinical and laboratory features were also evaluated as well as disease activity and disease duration.ResultsA significant association between positive AECA and a subtype of SSc (p = 0.021) was found, as well as between presence of digital ulcers and digital scars (p = 0.001), calcinosis (p = 0.02), acroosteolysis (p = 0.028) and a nearly significant association between AECA and lung fibrosis (p = 0.47). No association between disease duration, disease activity and AECA (p = 1.000 and 0.191, respectively) was present.ConclusionsAnti-endothelial cell antibodies are not associated with the activity of SSc. Digital ulcers, calcinosis and acroosteolysis are more common among AECA-positive patients suggesting that the presence of AECA might be an indicator of vascular complications development in SSc. Positive AECA among patients with lung fibrosis indicate their possible role in the development of lung disease. Further prospective studies including a greater number of patients are required.
Programmed-death 1 (PD-1) is a co-receptor that inhibits the inflammatory response, and thus helps in maintenance of peripheral immunotolerance. Impairment in the PD-1/PD-L1 pathway is believed to play an important role in many immune-mediated diseases, including systemic lupus erythematosus, rheumatoid arthritis, systemic sclerosis and autoimmune hepatitis, and, as emphasized recently, in psoriasis and psoriatic arthritis. Biologic drugs targeting immune checkpoint regulators may be associated with new-onset psoriasis or exacerbations of pre-existing dermatosis. In this review we discuss the role of PD-1/PD-L1 pathway in psoriasis basing on data published to date.
Steatocystoma multiplex (SM) is an unusual benign disorder of the pilosebaceous duct characterized by multiple cysts with little or no nail and hair involvement. We report a 30-year-old woman with multiple cystic nodules located on the neck, axillae and forearms as well as patchy scalp alopecia. Histopathological examination of the lesions was diagnostic of SM. Trichoscopy revealed pili torti and pili canaliculi. This patient represents an unusual clinical presentation of SM because of the presence of hair abnormalities.
Przegląd Dermatologiczny 2016/4 303 StreSzczenie Łuszczyca pospolita, szczególnie jej ciężka postać, często współwystę-puje z otyłością i innymi składowymi zespołu metabolicznego. Prawdopodobnie wiąże się to z prozapalnym działaniem hormonów i cytokin nazywanych adipokinami, które są wydzielane przez nadmiar tkanki tłuszczowej. Pacjenci otyli z łuszczycą mają zwykle większe nasilenie zmian skórnych, ponadto gorzej odpowiadają na terapie systemowe. W tej grupie chorych częściej obserwuje się powikłania sercowo-naczyniowe i metaboliczne, a także następstwa psychologiczne i psychiatryczne, takie jak depresja, obniżone poczucie własnej wartości i zaburzenia lękowe. Poradnictwo dietetyczne i modyfikacja stylu życia ukierunkowane na redukcję masy ciała powinny być integralną częścią postępo-wania terapeutycznego u pacjentów otyłych cierpiących na łuszczycę. AbStrActMany psoriatic patients, especially those suffering from severe forms of the disease, are obese and present other features of metabolic syndrome. It is likely due to proinflammatory activity of hormones and cytokines, called adipokines, which are produced by excess of fat tissue. Obese psoriatic patients usually have more severe skin lesions. They also respond poorly to systemic antipsoriatic therapies. Furthermore, this group of patients has a higher frequency of cardiovascular events and metabolic complications as well as psychological disturbances, such as depression, low self-esteem and anxiety disorders. Dietary counseling and lifestyle modification aimed at weight loss should be an integral part of therapeutic management in obese patients with psoriasis. Nadwaga to nadmierna masa ciała, która wyraża się współczynnikiem masy ciała (ang. body mass index -BMI) > 25 kg/m 2 , natomiast otyłość rozpoznaje się, gdy BMI wynosi powyżej 30 kg/m 2 (tab. 1). Nieco inaczej definiuje się otyłość brzuszną (inaczej trzewną), którą stwierdza się, gdy obwód talii wynosi powyżej 94 cm u mężczyzn i 80 cm u kobiet (dla populacji amerykańskiej wartości te są odpowiednio wyższe: 102 i 88 cm). Pomocne jest również określe-nie współczynnika talia-biodra (ang. waist-hip ratio -WHR). To właśnie trzewna tkanka tłuszczowa odpowiada za większość negatywnych następstw metabolicznych otyłości [1].W USA na otyłość cierpi ponad jedna trzecia społeczeństwa [2], a w skali świata jest to ponad 1 miliard ludzi, co czyni otyłość najczęstszą chorobą cywilizacyjną [3]. Nadmierna masa ciała wiąże się z licznymi powikłaniami -kardiologicznymi, naPrzegląd Dermatologiczny 2016/4 304 Dorota Krasowska, Michał Adamczyk czyniowymi i metabolicznymi, ponadto negatywnie wpływa na funkcjonowanie psychospołeczne, co prowadzi do obniżonego poczucia własnej wartości, depresji i zaburzeń lękowych, zwłaszcza w populacji dziecięcej (ryc. 1) [4,5].Otyłość sprzyja rozwojowi różnych chorób dermatologicznych, takich jak owrzodzenia podudzi w przebiegu niewydolności żylnej kończyn dolnych, obrzęki limfatyczne, zapalenie tkanki podskórnej, wyprzenia, infekcje skórne lub trądzik odwrócony. U osób otyłych częściej ni...
The present multi-center, long-term, real-life study made an attempt to assess the efficacy of risankizumab in the treatment of moderate-to-severe plaque psoriasis. The study comprised 185 patients from 10 Polish dermatologic departments undergoing risankizumab treatment. The disease severity was measured using the Psoriasis Area and Severity Index (PASI) before the start of the risankizumab treatment and next at the defined timepoints, i.e., 4, 16, 28, 40, 52 and 96 weeks of treatment. The percentage of patients achieving PASI90 and PASI100 responses as well as the PASI percentage decrease at the defined timepoints were calculated, and correlations with clinical characteristics and therapeutic effect were analyzed. The number of patients evaluated at the defined timepoints was: 136, 145, 100, 93, 62, and 22 at 4, 16, 28, 40, 52 and 96 weeks of treatment, respectively. At 4, 16, 28, 40, 52 and 96 weeks, the PASI90 response was achieved in 13.2%, 81.4%, 87.0%, 86.0%, 88.7% and 81.8% of patients, whereas the PASI100 response was achieved in 2.9%, 53.1%, 67.0%, 68.8%, 71.0% and 68.2% of patients, respectively. Our study revealed a significant negative correlation between a decrease in the PASI and the presence of psoriatic arthritis as well as the patient’s age and duration of psoriasis at several timepoints throughout the observation period.
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