Blog
About

4
views
0
recommends
+1 Recommend
0 collections
    0
    shares
      • Record: found
      • Abstract: found
      • Article: found
      Is Open Access

      Atypical Behçet disease with endocarditis, pyoderma gangrenosum–like ulcers and methicillin-resistant Staphylococcus aureus–positive skin abscesses

      Read this article at

      Bookmark
          There is no author summary for this article yet. Authors can add summaries to their articles on ScienceOpen to make them more accessible to a non-specialist audience.

          Abstract

          Introduction Behçet disease (BD) is a neutrophilic dermatosis characterized by relapsing flares of orogenital aphthous ulcers, uveitis, and skin inflammation.1, 2, 3 Extracutaneous manifestations involve gastrointestinal, neurologic, pulmonary, and cardiac systems. 1 The spectrum of skin manifestations includes erythema nodosum, papulopustular lesions, and pathergy. Skin abscesses, leukocytoclastic vasculitis, bullae, or lesions mimicking Sweet syndrome or pyoderma gangrenosum (PG) are rare.1, 2, 3 We describe a patient with atypical BD with endocarditis, PG-like ulcerations, and methicillin-resistant Staphylococcus aureus (MRSA)-positive abscesses, highlighting a unique constellation of infection and immune dysregulation. Case report A 33-year-old man with BD presented with a several-week history of persistent fever, abscesses, and oral and skin ulcers despite escalating his dapsone dose to 100 mg and prednisone dose to 100 mg daily. He was a marijuana farmer with exposure to animals and denied trauma and intravenous drug use. He had discontinued heavy alcohol use 6 weeks before presentation. His prior Behçet flares included fever, orogenital ulcers, and symmetric papulopustular eruptions on his extremities. Physical examination found an ill-appearing, febrile male with a 3/6 systolic murmur at the lower left sternal border. An extensive hemorrhagic ulcer with undermined violaceous borders and serosanguinous exudate was noted on the right dorsal thumb (Fig 1). Scattered 3- to 4-cm abscesses with surrounding erythema and warmth were present on the abdomen (Fig 2) and left anterolateral leg (Fig 3). Cellulitic plaques were present on the right elbow and dorsal right foot. Multiple 1-cm hyperpigmented macules were observed at the extensor forearms bilaterally, which the patient stated reflected a recent flare more typical of his BD skin disease (Fig 4). There were no signs of uveitis or neurologic deficits. Fig 1 Atypical PG-like ulcer at the dorsal aspect of the right proximal first digit. Clinical examination found an extensive hemorrhagic ulcer with undermined violaceous borders and serosanguinous exudate. Fig 2 MRSA-positive abscess with surrounding cellulitic plaque at the right lower abdominal quadrant. Fig 3 MRSA-positive abscess with surrounding cellulitic plaque at the left anterolateral leg. Fig 4 Hyperpigmented postinflammatory macular eruption on the extensor forearms bilaterally after a recent typical Behçet disease flare of papulopustules. Diagnostic evaluation found a white blood cell count of 19,000/μL, erythrocyte sedimentation rate of 3 mm/h, C-reactive protein level of 24 μg/mL, and methemoglobinemia of 12% (normal, 0-1.5%). Toxicology screening was positive for opiates and tetrahydrocannabinol. Additional laboratory test results were normal. The patient was admitted to the surgical service for suspicion of infection or Behçet flare, was treated with vancomycin and ertapenem, and underwent debridement and incision and drainage. Skin biopsy findings showed a dense neutrophilic dermal infiltrate, edema, and ulceration. Fite and Periodic acid–Schiff diastase stains were negative. Positive Brown-Brenn staining (identifies gram-positive and gram-negative bacteria within tissue) and cultures taken from multiple skin lesions confirmed MRSA. Three blood cultures and a nasal culture, taken after the initiation of antibiotics, were negative. Transthoracic echocardiogram found a mitral valve vegetation. After emergent treatment with methylene blue for his methemoglobinemia, the patient was treated with prednisone 1 mg/kg, dapsone 50 mg, and colchicine 1.2 mg, with rapid improvement of his skin lesions, and completed 1 month of vancomycin treatment for his skin lesions and possible infectious endocarditis (IE) without complications. Discussion The pathophysiology of BD is a complex relationship of genetics, immune dysregulation, and a possible important role for infection. Infectious triggers are suspected to act as auto-antigens in genetically predisposed (HLA-B51+) persons with BD.1, 4 Herpes simplex virus-1, Streptococcus, and oral flora are associated with neutrophilic ulcers and multiorgan disease. 1 Importantly, inflammatory manifestations of BD can also mimic infection, making the diagnosis and definitive treatment challenging. This case illustrates a unique discordance between cultures, transthoracic echocardiogram, and skin findings; his multiple MRSA-positive skin lesions and mitral valve vegetation suggested systemic infection, although negative blood cultures and clinical improvement with corticosteroids supported an equally plausible role for inflammation leading to his presentation. Typical mucocutaneous presentations of BD occur in 38% to 99% of patients, including orogenital aphthous ulcers, erythema nodosum, and papulopustular lesions resembling folliculitis. 1 Atypical skin presentations, including abscesses and lesions resembling PG, Sweet syndrome, or necrotizing fasciitis, are rare.1, 4, 5, 6 Infectious mucocutaneous lesions have also been described, including a BD patient with MRSA-positive gingivae 4 and a second case with methicillin-sensitive S aureus–positive necrotizing fasciitis. 7 Our patient presented with MRSA-positive lesions resembling abscesses, PG, and cellulitis. MRSA-positive skin lesions have not been previously reported in BD. This patient had a mitral valve vegetation with negative blood cultures in the setting of systemic antibiotic treatment, raising the question of whether he had infectious versus inflammatory endocarditis. Aseptic cardiac involvement occurs in 6% to 46% of BD patients; endocardial biopsy shows neutrophilic infiltration.2, 3, 8, 9 The prognosis of cardiac BD is poor, associated with 15.4% compared with 5.4% BD-related deaths without cardiac involvement. 3 The prevalence of culture-negative endocarditis in BD patients with cardiac lesions is 26.9%. 3 Culture-negative endocarditis in BD may be a result of antibiotics, a result of infection with intracellular organisms difficult to culture, or reflect sterile inflammation.2, 3, 8, 9 The latter is supported by reports of improvement with corticosteroid treatment alone.2, 3 Importantly, blood cultures for IE are often negative, with a sensitivity and specificity of 61% and 52%, respectively. 10 Although the literature suggests that valve vegetation in BD is more strongly associated with BD cardiac involvement than with IE, an infectious etiology of a cardiac lesion must be actively excluded.8, 9 BD is a clinical diagnosis, classified by an International Criteria for BD score of ≥4 1 ; our patient had a calculated score of 5. This patient's unique presentation of MRSA-positive skin lesions was distinct from his typical BD acral papulopustules. His cardiac murmur, negative nasal cultures, and MRSA-positive skin cultures from multiple scattered skin lesions prompted a diagnostic evaluation for IE. The role of MRSA in our case is unclear; his presentation may reflect sequelae of IE, an atypical manifestation of his BD with skin and cardiac involvement, or a combination of conditions. We hypothesize that his presentation reflected an interplay of MRSA endocarditis as an infectious trigger in a patient with known immune dysregulation, resulting in his unique systemic and cutaneous presentation. An equally plausible alternative hypothesis is that this patient had sterile endocarditis with multifocal MRSA-positive skin lesions that are rarely seen in BD. Given the complex interplay of infection, genetics, and immune dysregulation in patients with BD, atypical presentations may pose a diagnostic challenge and may warrant additional evaluation for complicating factors. Currently, to our knowledge, there are no documented cases of severe BD with this unique constellation of concurrent clinical features. Diagnostic evaluation to distinguish infectious versus inflammatory signs, especially in an immunosuppressed patient with BD, is paramount.

          Related collections

          Most cited references 10

          • Record: found
          • Abstract: found
          • Article: not found

          Behçet's disease: A comprehensive review with a focus on epidemiology, etiology and clinical features, and management of mucocutaneous lesions.

           Erkan Alpsoy (2016)
          Behçet's disease (BD) is a chronic, relapsing, inflammatory multisystem disease of unknown etiology. Oral ulcers, genital ulcers, cutaneous lesions, and ocular and articular involvement are the most frequent features of the disease. Mucocutaneous lesions are considered hallmarks of the disease, and often precede other manifestations. Therefore, their recognition may permit earlier diagnosis and treatment with beneficial results for prognosis. BD is particularly prevalent in "Silk Route" populations but has a global distribution. The disease usually starts around the third or fourth decade of life. Sex distribution is roughly equal. The diagnosis is based on clinical criteria, as there is no pathognomonic test. Genetic factors have been investigated extensively, and association with human leukocyte antigen (HLA)-B51 is still known as the strongest genetic susceptibility factor. The T-helper 17 and interleukin (IL)-17 pathways are active, and play an important role, particularly in acute attacks of BD. Neutrophil activity is increased in BD, and the affected organs show a significant neutrophil and lymphocyte infiltration. HLA-B51 association and increased IL-17 response are thought to play a role in neutrophil activation. Treatment is mainly based on the suppression of inflammatory attacks of the disease using immunomodulatory and immunosuppressive agents. Although treatment has become much more effective in recent years with the introduction of newer drugs, BD is still associated with considerable morbidity and increased mortality. Male sex, younger age of onset and increased number of organs involved at the diagnosis are associated with a more severe disease and, therefore, require more aggressive treatment.
            Bookmark
            • Record: found
            • Abstract: found
            • Article: not found

            Spectrum of cardiac lesions in Behçet disease: a series of 52 patients and review of the literature.

            Cardiac abnormalities in patients with Behçet disease (BD) include pericarditis, myocarditis, endocarditis with valvular regurgitation, intracardiac thrombosis, endomyocardial fibrosis, coronary arteritis with or without myocardial infarction, and aneurysms of the coronary arteries or sinus of Valsalva. Data regarding the clinical spectrum, prevalence, and outcome of cardiac lesions in BD are lacking. In this study, we report the main characteristics, treatment, and long-term outcomes of 52 patients with cardiac lesions from a cohort of 807 (6%) BD patients. Forty-five (86.5%) patients were male, with a mean (±SD) age at BD diagnosis of 29.3 ± 10.3 years.Cardiac involvement was the first feature of BD in 17 (32.7%) patients. Cardiac lesions included pericarditis (n = 20; 38.5%), endocarditis (mostly aortic insufficiency) (n = 14; 26.9%), intracardiac thrombosis (n = 10; 19.2%), myocardial infarction (n = 9; 17.3%), endomyocardial fibrosis (n = 4; 7.7%) and myocardial aneurysm (n = 1; 1.9%). Patients with cardiac involvement were more frequently male (86.5% vs. 64.9%; p < 0.01) and had more arterial (42.3% vs. 11.1%; p < 0.01) and venous lesions (59.6% vs. 35.8%; p < 0.01) compared to those without cardiac manifestations. Factors associated with complete remission of cardiac involvement were treatment regimens with oral anticoagulants, immunosuppressants, and colchicine. The 5-year survival rate was 83.6% and 95.8% (p = 0.03) in BD patients with and without cardiac involvement, respectively. After a median (Q1-Q3) follow-up of 3.0 (1.75-4.2) years, 8 patients had died, in 3 cases directly related to cardiac involvement.In conclusion, cardiac lesions affected 6% of our large cohort of BD patients. The prognosis of cardiac involvement in BD is poor and improves with oral anticoagulation, immunosuppressive therapy, and colchicine.
              Bookmark
              • Record: found
              • Abstract: found
              • Article: not found

              Cardiac manifestations in Behcet's disease.

              Behcet's disease (BD) is a chronic inflammatory disorder, with vasculitis underlying the pathophysiology of its multisystemic effects. Venous pathology and thrombotic complications are hallmarks of BD. However, it has been increasingly recognised that cardiac involvement and arterial complications are also important aspects of the course of the disease. Cardiac lesions include pericarditis, endocarditis, intracardiac thrombosis, myocardial infarction, endomyocardial fibrosis, and myocardial aneurysm. Treatment of cardiovascular involvement in BD is largely empirical, and is aimed towards suppressing the vasculitis. The most challenging aspect seems to be the treatment of arterial aneurysms and thromboses due to the associated risk of bleeding. When the prognosis of cardiac involvement in BD is not good, recovery can be achieved through oral anticoagulation, immunosuppressive therapy, and colchicine use. In this review, we summarise the cardiovascular involvement, different manifestations, and treatment of BD.
                Bookmark

                Author and article information

                Contributors
                Journal
                JAAD Case Rep
                JAAD Case Rep
                JAAD Case Reports
                Elsevier
                2352-5126
                30 April 2018
                June 2018
                30 April 2018
                : 4
                : 5
                : 449-451
                Affiliations
                Department of Dermatology, University of California San Francisco, San Francisco, California
                Author notes
                []Correspondence to: Kanade Shinkai, MD, PhD, 1701 Divisadero St, San Francisco, CA 94115. kanade.shinkai@ 123456ucsf.edu
                Article
                S2352-5126(18)30009-2
                10.1016/j.jdcr.2018.01.003
                6031566
                © 2018 Elsevier Inc.

                This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).

                Categories
                Case Report

                Comments

                Comment on this article