Genital pyoderma gangrenosum revealing Behçet’s disease: a case report

Abstract Background Pyoderma gangrenosum (PG) is a rare and chronic neutrophilic dermatosis. It is clinically characterized by aseptic ulcerations preferentially located in the lower limbs. Its location in the genital area is unusual and could be a source of diagnostic difficulties. In half of the cases, PG is associated with an underlying disease. The association with Behçet’s disease is exceptional. Case presentation We report an original observation of a patient who presented a vulvar PG which revealed a Behçet’s disease. Conclusion The distinction between these two pathologies was difficult because of the similarity of the cutaneous-mucous lesions on the one hand, and the absence of histological specificity of these two pathologies on the other hand.

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Endovascular Aneurysm Exclusion along a Femorodistal Venous Bypass in Active Behçet's Disease

Journal of Endovascular Therapy ◽

10.1177/152660280200900523 ◽

2002 ◽

Vol 9 (5) ◽

pp. 694-698 ◽

Cited By ~ 6

Author(s):

Silvia B. Gretener ◽

Dai-Do Do ◽

Iris Baumgartner ◽

Hans-Peter Dinkel ◽

Jürg Schmidli ◽

...

Keyword(s):

Behçet’S Disease ◽

Behcet’S Disease ◽

Behçet's Disease ◽

Bypass Graft ◽

Underlying Disease ◽

European Ancestry ◽

Stent Grafts ◽

Venous Bypass ◽

Behcet's Disease ◽

Active Nature

Purpose: To report the endovascular repair of dual aneurysms along a femorodistal venous bypass graft in a patient with Behçet's disease. Case Report: A 55-year-old man of middle European ancestry with Behçet's disease had dual aneurysms evolve along the proximal segment of a femorodistal venous bypass that had been implanted 2.5 years earlier for recurrent false aneurysm formation. Owing to the lack of suitable venous conduits and the active nature of the disease, the aneurysms were successfully excluded with overlapping Hemobahn and Jostent endografts; the immunosuppressive therapy was intensified. Rupture of the aneurysms was successfully prevented, but the stent-grafts thrombosed 6 weeks later owing to exacerbation of the underlying disease. Conclusions: Endovascular exclusion of aneurysm in venous bypass grafts in Behçet's disease is feasible. Although the stent-grafts thrombosed, they did prevent rupture of the aneurysms.

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Behçet’s disease: a case report about a rare cause of intra-cardiac mass

European Heart Journal - Case Reports ◽

10.1093/ehjcr/ytab299 ◽

2021 ◽

Vol 5 (10) ◽

Author(s):

Alexandra Briosa ◽

Ana Catarina Gomes ◽

Ana CastelBranco ◽

Margarida Cunha ◽

Sandra Sousa ◽

...

Keyword(s):

Right Ventricular ◽

Behçet’S Disease ◽

Behcet’S Disease ◽

Behçet's Disease ◽

Underlying Disease ◽

Cardiovascular Complications ◽

Initial Manifestation ◽

Behcet's Disease ◽

Ventricular Mass ◽

Right Ventricular Mass

Abstract Background Intra-cardiac masses are always a challenging diagnosis, especially when it involves the right side of the heart. There are multiples aetiologies that can be responsible for these masses, namely thrombosis, neoplasm, or vegetations. Occasionally, these may be related to an autoimmune process not yet diagnosed. We present a case of a 17-year-old patient with an exuberant right ventricular mass due to a not yet diagnosed Behçet’s disease. The best approach and treatment for these patients remains uncertain. Case summary The authors present a case of a 17-year-old patient with a right ventricular mass who presented as an initial manifestation of Behçet’s disease. It was firstly assumed as a thrombotic mass and medicated with anticoagulation, with no resolution. After performing a cardiac magnetic resonance, the case was discussed in a multidisciplinary team, including cardiology, paediatrics, and rheumatology, and the diagnosis of Behçet’s disease with cardiac complication was established. The patient started immunosuppressive therapy with clinical and echocardiographic response. Discussion Behçet’s disease is a multi-systemic autoimmune vasculitis that usually manifests by recurrent oral and genital ulcers as well as ocular symptoms. Cardiac manifestations are rare but important aspects of the course of the disease, especially in what concerns morbidity burden. The treatment of these cardiovascular complications is generally empirical and involves the treatment of the underlying disease.

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Case of pyoderma gangrenosum showing oral and genital ulcers, misdiagnosed as Behcet's disease at first medical examination

The Journal of Dermatology ◽

10.1111/j.1346-8138.2008.00468.x ◽

2008 ◽

Vol 35 (5) ◽

pp. 289-292 ◽

Cited By ~ 13

Author(s):

Hiromi TSUBOI

Keyword(s):

Behçet’S Disease ◽

Pyoderma Gangrenosum ◽

Behcet’S Disease ◽

Behçet's Disease ◽

Medical Examination ◽

Behcet's Disease ◽

Genital Ulcers

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Behçet’s disease – diagnostic difficulties

Polish Archives of Internal Medicine ◽

10.20452/pamw.208 ◽

2012 ◽

Vol 117 (9) ◽

pp. 420-425

Author(s):

Anna Niedzielska ◽

Katarzyna Chelminska ◽

Bogdan Jaremin

Keyword(s):

Behçet’S Disease ◽

Behcet’S Disease ◽

Behçet's Disease ◽

Behcet's Disease ◽

Diagnostic Difficulties

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Vegetative Pyoderma Gangrenosum in Behçet’s Disease

Acta Dermato Venereologica ◽

10.2340/00015555-0221 ◽

2007 ◽

Vol 87 (4) ◽

pp. 365-367 ◽

Cited By ~ 8

Author(s):

JW Kim ◽

JH Park ◽

D Lee ◽

SW Hwang ◽

SW Park

Keyword(s):

Behçet’S Disease ◽

Pyoderma Gangrenosum ◽

Behcet’S Disease ◽

Behçet's Disease ◽

Behcet's Disease

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Pediatric Behçet’s Disease and Thromboses

The Journal of Rheumatology ◽

10.3899/jrheum.100257 ◽

2010 ◽

Vol 38 (2) ◽

pp. 387-390 ◽

Cited By ~ 23

Author(s):

BRICE KRUPA ◽

ROLANDO CIMAZ ◽

SEZA OZEN ◽

MICHEL FISCHBACH ◽

PIERRE COCHAT ◽

...

Keyword(s):

Behçet’S Disease ◽

Behcet’S Disease ◽

Behçet's Disease ◽

International Study ◽

Retrospective Chart Review ◽

Lower Limbs ◽

Disease Course ◽

Protein C Deficiency ◽

Behcet's Disease ◽

Increased Risk

Objective.To describe the characteristics of a group of pediatric patients with Behçet’s disease (BD) who presented at least 1 episode of thrombosis during their disease course.Methods.We made a retrospective chart review of the clinical, biological, and radiological data of children with BD who presented at least 1 episode of either arterial or venous thrombosis. Data were extracted from both an international pediatric Behçet cohort and files referred from 7 French centers.Results.Twenty-one patients were included. Diagnosis of BD was based on the criteria of the International Study Group for BD. Main locations for thrombosis were the cerebral sinuses, in 11 patients (52.4%); and lower limbs, in 9 patients (40.9%). Recurrent episodes were observed in 4 patients (21%). Thrombophilia measurements were normal in 14 patients out of 21, while anticardiolipin antibodies were positive in 4 patients, and 2 out of 21 had protein C deficiency. One patient had lupus anticoagulant. All patients were treated with colchicine. Corticosteroids were also added for variable periods in 13 patients. Five patients out of 21 were treated with anticoagulants (heparin, then anti-vitamin K) and 3 with antiplatelets (acetylsalicylic acid).Conclusion.Thromboses are a serious complication of BD and may occur early in the disease course. The presence of thrombophilic markers could increase the risk of thrombosis in BD, but the size of our population does not allow any conclusion. An international cohort (PED-BD) is currently in place and will allow study of such cases longitudinally, as well as assessment of the elements that correlate with an increased risk of thrombosis in children with BD.

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