Antiphospholipid antibody syndrome presenting as pyoderma gangrenosum

2022 ◽  
Vol 42 (1) ◽  
pp. 77
Author(s):  
MansoorC Abdulla
2020 ◽  
Author(s):  
metin kaya gürgöze ◽  
Aslıhan Kara ◽  
Mehmet yusuf sarı ◽  
İlknur Çalık ◽  
Saadet Akarsu

Abstract Background: Although pyoderma gangrenosum (PG) -like lesions have been rarely described in adults with the antiphospholipid antibody syndrome (APS) and systemic lupus erythematosus (SLE), the occurrence of PG as a preceding manifestation of APS in children with SLE has not been reported until. We present a young girl with SLE and APS who developed progressive extstensive ulcerations that were consistent with PG.Case presentation: A 14-year-old girl with a 2-year history of SLE was admitted to our department, complaining painful crusted ulcerations on her legs. Skin biopsy was reported as PG. However, she did not respond to immunosuppressive therapy administered. When her skin biopsy findings is reassessed in keeping with the positive anticardiolipin antibody results, superficial small vessel microthrombosis was observed. Diagnosis of APS and PG developing secondary to SLE were made. It was resulted in marked clinical improvement with anticoagulation therapy in addition to immunosuppressives as is recommended in APS. Conclusions: Based in clinical, pathological and response to proposed treatment, we can state that PG -like lesions in children with SLE could be considered as a secondary form of APS.


2019 ◽  
Vol 25 (3) ◽  
pp. 498-499 ◽  
Author(s):  
Justine Figurelli ◽  
Karim Kolsi ◽  
Camille Franchet ◽  
Karen Delavigne ◽  
Marc Soule‐Tholy ◽  
...  

1994 ◽  
Vol 71 (04) ◽  
pp. 424-427 ◽  
Author(s):  
Masahide Yamazaki ◽  
Hidesaku Asakura ◽  
Hiroshi Jokaji ◽  
Masanori Saito ◽  
Chika Uotani ◽  
...  

SummaryThe mechanisms underlying clinical abnormalities associated with the antiphospholipid antibody syndrome (APAS) have not been elucidated. We measured plasma levels of lipoprotein(a) [Lp(a)], the active form of plasminogen activator inhibitor (active PAI), thrombin-antithrombin III complex (TAT) and soluble thrombomodulin (TM), to investigate the relationship of these factors to thrombotic events in APAS. Mean plasma levels of Lp(a), TAT, active PAI and TM were all significantly higher in patients with aPL than in a control group of subjects. Plasma levels of Lp(a) and active PAI were significantly higher in patients with aPL and arterial thromboses than in patients with aPL but only venous thromboses. There was a significant correlation between plasma levels of Lp(a) and active PAI in patients with aPL. These findings suggest that patients with aPL are in hypercoagulable state. High levels of Lp(a) in plasma may impair the fibrinolytic system resulting in thromboses, especially in the arterial system.


1997 ◽  
Vol 77 (04) ◽  
pp. 624-628 ◽  
Author(s):  
Sabine Eichinger ◽  
Ingrid Pabinger ◽  
Andreas Stümpfien ◽  
Mirko Hirschl ◽  
Christine Bialonczyk ◽  
...  

SummaryThromboprophylaxis with oral anticoagulants up to six months is established in patients after a first venous thromboembolic event (VTE). The risk of recurrent VTE is still considerable thereafter, and it is uncertain whether some patients might benefit from extended anticoagulation. We performed a prospective, multicenter trial (4 thrombosis centers) and evaluated in 380 patients with a first or recurrent VTE (patients with a deficiency of antithrombin, protein C, protein S or plasminogen; cancer; or an antiphospholipid antibody syndrome were excluded) the risk of recurrence after discontinuation of secondary thromboprophylaxis with oral anticoagulants. It was the aim of the study to evaluate whether patients with factor V Leiden are at an increased risk of recurrent VTE. 112 (29.5%) patients were carriers of factor V Leiden (26.9% heterozygous, 2.6% homozygous). After a median observation time of 19.3 months the overall recurrence rate of VTE was 9.9%. Recurrent deep vein thrombosis and/or pulmonary embolism occurred in 26 of 268 patients without factor V Leiden (9.7%) and in 10 of 112 patients with factor V Leiden (8.9%). The probability of recurrent VTE two years after discontinuation of oral anticoagulants was 12.4% (95% Cl 7.8-17) in patients without factor V Leiden and was 10.6% (95% Cl 3.8-17.4) in carriers of the mutation. This difference was statistically not significant. Patients with factor V Leiden are not at a higher risk of recurrent VTE within two years after discontinuation of oral anticoagulants than patients without factor V Leiden. Balancing the risk of recurrent VTE and bleeding from oral. anticoagulants, patients with factor V Leiden are not likely to benefit from oral anticoagulant therapy extended beyond six months.


2001 ◽  
Vol 63 (3) ◽  
pp. 230-234
Author(s):  
Ryo TANAKA ◽  
Takenobu YAMAMOTO ◽  
Osamu YAMASAKI ◽  
Joji TADA ◽  
Jiro ARATA ◽  
...  

2001 ◽  
Vol 77 (6) ◽  
pp. 517-21
Author(s):  
Célia S. Macedo ◽  
Roberta S. Martinez ◽  
Márcia C. Riyuzo ◽  
Herculano D. Bastos

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1029.2-1030
Author(s):  
A. Colpo ◽  
P. Marson ◽  
T. Tison ◽  
A. Zambon ◽  
A. La Rosa ◽  
...  

Background:Therapeutic apheresis (TA) represents a therapeutic option in pre-existing conditions or rheumatic diseases that occur during gestation. Although pregnancy is not a contraindication itself, due to the lack of evidence-based guidelines and the alleged risk of maternal and/or fetal adverse events, there is general resistance to its application during pregnancy.Objectives:In this observational study we aimed to evaluate the efficacy and safety of TA in high-risk pregnancies in patients with rheumatic diseases, followed over a decade in a tertiary Center.Methods:Between January 2005 and April 2019, 843 TA procedures were performed during 51 pregnancies in 43 patients: 745 plasma exchange sessions and 98 immunoabsorption sessions. TA was performed in 29 (57%) pregnancies of 21 (48.8%) patients with antiphospholipid antibody syndrome (APS), in 20 (39.2%) pregnancies of 20 (46.5%) patients with congenital heart block (CHB), in 1 (1.9%) pregnancy of 1 (2.3%) patient with systemic sclerosis (SSc) and 1 (1.9%) pregnancy of 1 (2.3%) patient affected by lupic nephritis (SLE).Results:During the period considered, apheresis sessions applied to pregnant women were 7.1% of the total (n = 13.251). The average age at the first treatment was 33 years (range 24-43). The mean management age at the first apheretic treatment was 21 weeks (range 4-32). Twelve (1.4%) apheresis sessions were complicated by adverse events, none required or prolonged hospitalization. There were 44 (86.3%) live births, 3 (5.9%) spontaneous abortions and 2 (3.9%) voluntary terminations of pregnancy, 2 (3.9%) lost to follow-up. The average gestational age at birth was 35 weeks (range 24-37) and cesarean section was performed in 41 (80.4%) cases. TA was added to conventional therapy in 24/29 (82.7%) patients with APS, to the detection of fetal cardiac activity, while in 5/26 (17.3%) it was introduced when the first signs of pregnancy complications such as mild preclampsia, HELLP and IUGR were detected. TA was started within 24 hours of atrioventricular block (AVB) detection; 10/20 (50%) mothers with CHB were diagnosed with 2nd degree AVB, 9/20 (45%) with 3rd degree AVB and one (5%) with sinus bradycardia and endocardial fibroelastosis. The patient with SSc was treated with TA twice a week from the 32nd SG until delivery, which occurred at the 36th SG, due to severe IUGR and oligohydramnios. The patient with SLE complicated by lupic nephritis was treated with TA twice a week, from the 26th SG until the birth, which took place at the 31st SG.Conclusion:Our data have shown that TA in pregnancy is well tolerated. Close collaboration between rheumatologist, obstetrician and specialist in TA is essential to ensure a successful outcome of high-risk pregnancies.Disclosure of Interests: :Anna Colpo: None declared, Piero Marson: None declared, Tiziana Tison: None declared, Alessandra Zambon: None declared, Annalisa La Rosa: None declared, Ermella Zanetti: None declared, Amelia Ruffatti: None declared, Giustina De Silvestro: None declared, Ariela Hoxha Speakers bureau: Celgene, UCB, Novartis, Sanofi, Werfen


2019 ◽  
Vol 19 (1) ◽  
pp. 36-38
Author(s):  
Babak Choobi Anzali ◽  
Maryam Bahreini ◽  
Behnaz Habibi ◽  
Noorieh Sharifi Sistani

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