Michael A. McGrath

Lymphocytic Thrombophilic Arteritis: A Newly Described Medium-Sized Vessel Arteritis of the Skin

BACKGROUNDnWe encountered a distinct arteriolar histopathologic finding of lymphocytic vasculitis associated with a hyalinized fibrin ring in vessel lumina. Identical histologic findings have previously been described as macular arteritis.nnnOBSERVATIONSnWe describe 5 women (mean age, 25 years; age range, 20-34 years) with persistent, slowly progressive, patchy and reticular hyperpigmentation associated with livedo racemosa affecting predominantly the lower limbs. In the biopsy samples, infiltration of muscular vessel wall by inflammatory cells, affecting small arteries of the dermosubcutaneous junction or superficial subcutis, was present. Of the infiltrate, 90% or more consisted of mononuclear cells, mainly lymphocytes with an admixture of histiocytes. Neutrophils and eosinophils were absent or scant. Inflammation was confined to the vicinity of the vessel and the immediate surrounding panniculus. A concentric fibrin ring involving the entire periphery of the lumina of affected vessels was present in all the patients. Laboratory investigation results revealed that 4 patients had antiphospholipid antibodies in their serum. One of these patients had a heterozygous mutation of the factor V Leiden gene. Conclusion We term this arteritis lymphocytic thrombophilic arteritis to reflect the histologic features that combine lymphocytic vascular inflammation with changes representing a thrombophilic endovasculitis.

A single-centre prospective study of clinical and haemostatic risk factors for venous thromboembolism following lower limb arthroplasty

Previous studies report conflicting results concerning the potential significance of thrombophilic genotypes in postarthroplasty venous thromboembolism (VTE). This study assessed thrombophilic genotypes, haemostatic and clinical variables as independent risk factors for VTE postarthroplasty. A total number of 569 patients undergoing elective lower limb arthroplasty at a single centre were prospectively studied. All patients were interviewed and had blood samples collected preoperatively. Bilateral lower limb ultrasonography was performed at day 7u2003±u20032 postoperatively in all patients (ventilation/perfusion lung scanning in symptomatic patients only). The incidence of inhospital postoperative VTE was 26%. In univariate analysis – increased age, knee arthroplasty, recent surgery, general anaesthesia, shorter operation time, non‐receipt of blood transfusion and differences in surgical practice (including use of pneumatic calf compression, surgical drains and postoperative bandaging techniques) were significantly associated with VTE. Factor V Leiden, prothrombin G20210A and MTHFR C677T mutations were not significant risk factors for VTE, and of all haemostatic variables tested, only median activated partial thromboplastin time showed significant difference between VTE and non‐VTE patients (34u2003s vs. 33u2003s). Multiple logistic regression analysis demonstrated that increased age, knee arthroplasty and individual surgeons routine practices were the only significant independent risks for VTE; hence routine preoperative blood screening for a potential hypercoaguable state is not indicated in this surgical setting.

The natural history of chronic leg ischemia

The onset of leg ischemia may be sudden or gradual, occurring over a wide age spectrum and as a result of multiple causes. The clinical outcome is extremely variable, and only about 1 patient in 8 will progress to the stage of ischemic gangrene. Many patients have the capacity to show marked clinical improvement, or to maintain a comfortable life with claudication that is not severely disabling. Of over 2,000 patients referred to a vascular unit, only about 30% required operative intervention. Rigorous conservative measures should be given a trial for at least 3 months in all patients except those whose limbs are severely threatened, since that period of time is necessary to judge the capacity for spontaneous improvement, before the patient is subjected to the invasive technique of aortography. A flow chart of management decisions is suggested for patients with intermittent claudication. Le début de lischémie de la jambe peut Être brutal ou progressif. Lischémie se manifeste à tous les âges et elle est due à des causes multiples. Lévolution clinique est extrÊmement variable. En pratique une fois sur huit seulement elle aboutit à la gangrène. De nombreux malades présentent une amélioration certaine grâce au traitement médical ou peuvent mener une vie acceptable quand la claudication intermittente nest pas trop sévère. Sur 2000 malades qui ont été adressés à lunité de chirurgie vasculaire, 30% seulement ont eu besoin dune intervention chirurgicale. Le traitement médical conservateur suivi rigoureusement doit Être observé pendant 3 mois, sauf si la gangrène est menaÇante, cette période dessai étant nécessaire pour apprécier la possibilité dune amélioration spontanée et avant de recourir à laortographie. Sont envisagées dans larticle les modalités de traitement de lischémie caractérisée par une claudication intermittente. El comienzo de la isquemia de la pierna puede ser sÚbito o gradual, y puede ocurrir dentro de amplios límites de edades y debido a mÚltiples causas. La evolución clínica es extremadamente variable, y solo uno de cada 8 pacientes avanza hasta el estado de gangrena isquémica. Muchos pacientes tienen la capacidad para exhibir notoria mejoría, o para mantener una vida confortable con claudicación que no causa incapasidad severa. Sobre 2.000 pacientes referidos a una unidad vascular, apenas el 30% requirió intervención operatoria. Debe hacerse un ensayo con rigurosas medidas terapeÚticas conservadoras por un período por lo menos de 3 meses en todos los casos, excepto aquellos cuyas extremidades se encuentren severamente amenazadas, por cuanto éste es el lapso de tiempo necesario para juzgar la capacidad de mejoría espontánea antes de que el paciente sea sometido a la técnica invasiva de aortografía. Se sugiere un flujograma de decisiones de manejo para pacientes con claudicación intermitente.The onset of leg ischemia may be sudden or gradual, occurring over a wide age spectrum and as a result of multiple causes. The clinical outcome is extremely variable, and only about 1 patient in 8 will progress to the stage of ischemic gangrene. Many patients have the capacity to show marked clinical improvement, or to maintain a comfortable life with claudication that is not severely disabling.Of over 2,000 patients referred to a vascular unit, only about 30% required operative intervention. Rigorous conservative measures should be given a trial for at least 3 months in all patients except those whose limbs are severely threatened, since that period of time is necessary to judge the capacity for spontaneous improvement, before the patient is subjected to the invasive technique of aortography.A flow chart of management decisions is suggested for patients with intermittent claudication.RésuméLe début de lischémie de la jambe peut Être brutal ou progressif. Lischémie se manifeste à tous les âges et elle est due à des causes multiples. Lévolution clinique est extrÊmement variable. En pratique une fois sur huit seulement elle aboutit à la gangrène. De nombreux malades présentent une amélioration certaine grâce au traitement médical ou peuvent mener une vie acceptable quand la claudication intermittente nest pas trop sévère.Sur 2000 malades qui ont été adressés à lunité de chirurgie vasculaire, 30% seulement ont eu besoin dune intervention chirurgicale. Le traitement médical conservateur suivi rigoureusement doit Être observé pendant 3 mois, sauf si la gangrène est menaÇante, cette période dessai étant nécessaire pour apprécier la possibilité dune amélioration spontanée et avant de recourir à laortographie.Sont envisagées dans larticle les modalités de traitement de lischémie caractérisée par une claudication intermittente.AbstractoEl comienzo de la isquemia de la pierna puede ser sÚbito o gradual, y puede ocurrir dentro de amplios límites de edades y debido a mÚltiples causas.La evolución clínica es extremadamente variable, y solo uno de cada 8 pacientes avanza hasta el estado de gangrena isquémica. Muchos pacientes tienen la capacidad para exhibir notoria mejoría, o para mantener una vida confortable con claudicación que no causa incapasidad severa.Sobre 2.000 pacientes referidos a una unidad vascular, apenas el 30% requirió intervención operatoria. Debe hacerse un ensayo con rigurosas medidas terapeÚticas conservadoras por un período por lo menos de 3 meses en todos los casos, excepto aquellos cuyas extremidades se encuentren severamente amenazadas, por cuanto éste es el lapso de tiempo necesario para juzgar la capacidad de mejoría espontánea antes de que el paciente sea sometido a la técnica invasiva de aortografía.Se sugiere un flujograma de decisiones de manejo para pacientes con claudicación intermitente.

Abdominal wall ulceration and mucinosis secondary to recombinant human interferon-beta-1b.

A 46‐year‐old woman developed painful ulcers over her lower abdomen in the form of reticulate erythema after injecting interferon β‐1b subcutaneously for multiple sclerosis. Skin biopsy revealed multiple superficial thrombosed vessels with focal epidermal necrosis as well as prominent interstitial mucinosis. Treatment with low‐molecular‐weight heparin followed by a heparinoid resulted in slow healing of the ulcers but also allowed the subcutaneous interferon injections to be continued.

Macular lymphocytic arteritis.

To the Editor, We read with interest the article by Saleh and Mutasim1 on macular lymphocytic arterititis. They propose that this is the most appropriate term for this recently recognized vasculitis, rather than lymphocytic thrombophilic arteritis which we chose as it defines the distinctive histopathology seen in this setting. Although the clinical presentation may appear macular, it is also often reticular and arborizing in a pattern seen with livedo racemosa. This is a useful clinical clue and would be expected for a vasculitis which is targeting the medium-sized arteries within the deep dermis and subcutis. Furthermore, despite the macular appearance of the skin lesions palpation often reveals the presence of subtle subcutaneous induration or frank nodules. We chose the term lymphocytic thrombophilic arteritis2 to highlight the striking histopathological features that had been observed in previous cases described by the authors as macular arteritis,3,4 namely, the presence of a striking thick fibrin ring that can be appreciated on low power microscopy within arterioles in the deep dermis and subcutis in association with a prominent lymphocytic infiltrate (Fig. 1A and B). All of our five published cases had this feature as a defining criterion for inclusion and represents an unusual thrombophilic endarteritis. The authors have not highlighted this aspect in their illustrated histopathology in their additional cases but included narrowed arterioles that may be seen as an end result of a large range of vasculopathies including cutaneous polyarteritis nodosa.5 The striking cover illustration of a markedly inflamed arteriole with intramural lymphocytes may be important as it may represent the early phase of this lymphocytic arteritis prior to the development of the distinctive fibrin ring. This observation raises the question of whether the fibrin ring is primarily deposited on the inner aspect of the vessels as a result of lymphocytic-mediated injury to the subendothelial zone of the arterioles. One of the intriguing aspects in these patients is the prolonged course, often over years without the presence of ulceration or cutaneous infarction and may be dependent on the location of A