Macular lymphocytic arteritis: three cases questioning its classification as primary lymphocytic vasculitis.

BACKGROUND: Macular arteritis, macular lymphocytic arteritis (MLA) or lymphocytic thrombophilic arteritis all correspond to an identical new clinicopathological entity. Its individualization as a primary cutaneous lymphocytic arteritis is still controversial for certain authors as it could represent a latent form of cutaneous polyarteritis nodosa. MATERIALS AND METHODS: We report here 3 additional cases of MLA, present a review of the literature and discuss the disease's nosology. RESULTS: MLA is characterized clinically by a benign skin eruption consisting in bilateral asymptomatic erythematous/hyperpigmented macules mainly located on the lower legs and histologically by a medium-sized cutaneous lymphocytic prominent arteritis present in early cutaneous lesions. CONCLUSION: These findings support that MLA may be considered as a chronic and indolent primary lymphocytic cutaneous arteritis. Nevertheless, in some cases the objective obliteration of cutaneous vessels underlines the need for continuous monitoring in MLA patients.

[Ultrasonography in Large-Vessel Arteritis].

Vasculitides are a group of diseases in which inflammation occurs in various vascular walls of the whole body, and ischemic symptoms are caused by stenoses and occlusions of blood vessels. Various parts of blood vessels of the whole body are affected, and the clinical manifestations are diverse. In the Chapel Hill Consensus Conference (CHCC) 2012, vasculitides are classified into seven categories. Takayasu arteritis and giant cell arteritis are included in large-vessel vasculitis. Large-vessel arteritis is defined as vasculitis affecting the aorta and its major branches more often than other vasculitides, but any sized artery may be affected. Ultrasonography has been progressing rapidly, so we can easily depict vessels of the surface of the body, in 0.1-mm units, and indicate the blood flow noninvasively. Ultrasonography has been used for the diagnosis of and estimation of the treatment for large-vessel vasculitis, and its importance has been increasing.

Cannabis and skin diseases.

From time out of mind, man has grown hemp for both "industrial" and "recreational" use (it is then referred to as cannabis). Of course, cannabis has strong psychoactive properties and is one of the most commonly used "soft drugs" in the world. Clinicians should know the adverse effects on mucous membranes and on skin, which may sometimes entail an absolutely necessary stopping of consumption. Raynaud's phenomenon, as well as arteritis due to cannabis consumption may be extremely severe and result in worrying situations for both clinicians and patients.

Disseminated visceral giant cell arteritis: histopathologic description and differentiation from other granulomatous vasculitides.

Disseminated visceral giant cell arteristic, a previously unknown entity, was observed in four autopsied patients, all men, aged 33, 67, 59 and 45 years. None of the patients had temporal arteritis, collagen disease, sarcoidosis, hepatitis or other infections, and vasculitis was diagnosed only after death. All had giant cell arteritis of extracranial arteries and arterioles in at least three of the following organs: the heart, lungs, kidneys, liver, pancreas, and stomach in various combinations. Despite some histopathologic similarities, disseminated visceral giant cell arteritis can be distinguished from other necrotizing and granulomatous vasculitides by the type of vessels principally affected and the presence or absence of giant cells, vascular fibrinoid necrosis and eosinophilic infiltrates. The observations suggest that it is a distinctive type of systemic vasculitis.

Systemic vasculitis.

The systemic vasculitides are comparatively rare but important disorders of childhood. Apart from Henoch-Schönlein purpura, which is the commonest example in paediatric practice, Kawasaki disease is most often encountered. Polyarteritis and Wagener's granulomatosis have a lower incidence but are associated with a high morbidity and mortality. Newer investigative procedures and therapeutic approaches have led to more accurate diagnoses and improved outcome but further advances await a better understanding of the vasculitides: polyarteritis; Kawasaki disease; Wagener's granulomatosis; and ANCA in childhood.

Coronary vasculitis. A review in the current scheme of classification of vasculitis.

Coronary artery disease is overwhelmingly atherosclerotic in nature, but inflammatory disease of the coronary arteries can be just as life-threatening a cause of ischemic heart disease in all age groups. Coronary vasculitis is not short in variety; as a clinicopathologic entity it spans the entire spectrum of systemic vasculitides. Coronary vasculitis is most closely associated with the polyarteritis group of necrotizing angiitis, vasculitis of collagen-vascular disease, and granulomatous giant cell arteritis. This article provides an overview of coronary vasculitis as an independent entity as well as a manifestation of systemic vasculitis, both the common and the uncommon varieties.

Parietal inflammatory infiltrate in peripheral aneurysms of atherosclerotic origin.

Clinical and histological analyses were made of 18 consecutive cases of peripheral artery atherosclerotic aneurysms (PAAA) (common, deep femoral and popliteal arteries) and compared to a group of 10 specimens obtained from the atherosclerotic, non aneurysmal femoral arteries of 10 cadavers with similar characteristics to the 18 patients. Although neither the clinical nor the macroscopic morphological data indicated the presence of an inflammatory process in the PAAA, the histological examination revealed the presence of a considerable infiltrate (greater than 11 inflammatory cells/2116 microns2) in a surprisingly high percentage of cases (4 cases, 22.8%). In 5 other cases (27.7%) the presence of lymphomonoplasmonocytic cells, although less pronounced, was greater than normally seen in atherosclerotic arterial walls (greater than 4 and less than 11 inflammatory cells/2116 microns2). The median number of inflammatory cells present in the media and adventitia varied from 2.2 +/- 0.3 to 13.2 +/- 0.3 per 2116 microns2. These values are significantly different compared to the results of atherosclerotic arteries at the same level (P less than 0.001 Mann-Whitney's U test). These findings of lymphomonoplasmocytic infiltrates in the absence of other pathology, together with an analysis of the data in the literature, makes it possible to presume that the inflammation present is associated with atherosclerosis and is more common in aneurysmal rather than stenotic forms. The pathogenesis may be determined by immune reaction phenomena.

[A proposed classification for diabetic arteritis based on functional and arteriographic data (author's transl)]. / Essai de classification des artérites du diabétique à la lumière des données artériographiques et fonctionnnelles.

Three main types of arteriopathy in diabetic patients can be distinguished by comparing arteriographic and "irrigraphic" data. High forms, with non-specific irrigraphic findings, fall within the framework of typical arteriosclerotic types of arteritis. Distal femorotibial forms, with characteristics in irrigraphic tracings found only in diabetes, differ from the previous forms. Finally, there are hyperirrigation syndromes in which the functional element is dominant, and which can be considered as early forms of diabetic arteriopathy. These conclusions are based on a study of 180 cases, arteriographic and irrigraphic data being compared in 90 of these patients.

[Drug evaluation methods in lower limb arteriopathy]. / Méthodes d'évaluation des médicaments dans l'artériopathie des membres inférieurs.

The prevalence and severity of chronic vascular leg disease explains the necessity to clarify methods for assessing it. Today the methods are both clinical and paraclinical. Clinically, they are based on the Fontaine and Leriche classification and appearance of ischaemic pain. Paraclinically, they are measuring distal blood pressure, artery output, micro-circulation, rheology, and tissular metabolism. But this approach must be also global, assessing coronary and carotid disease. Trials methodology includes a first step of explanatory studies by paraclinical ways and lastly pragmatic efficacy and tolerability studies. We recommend an accurate selection of patients and a stratification. The disease must be stable and the treatment and diet too. The trials should be randomised vs placebo. In conclusion we propose a multiparametric classification of the disease.

Eosinophilic coronary periarteritis (vasospastic angina and sudden death), a new type of coronary arteritis: report of seven autopsy cases and a review of the literature.

A previously reported autopsy case of eosinophilic coronary periarteritis (ECPA, or isolated eosinophilic coronary periarteritis, IECPA), and an additional six autopsy cases of ECPA are reported. In addition, another four autopsy cases of ECPA reported in the literature are discussed. Fifteen cases of ECPA with spontaneous coronary dissection (hematoma), which appeared in the literature from 1987 to 2011, are also reviewed. The characteristic clinico-pathological findings of ECPA are: (a) variant angina (Prinzmetal's vasospastic angina) appeared mainly from evening to early in the morning; (b) allergy or allergic history could be identified in only three of a total of 11 cases; (c) sudden unexpected death (sudden cardiac death) usually occurred early in the morning; (d) eosinophilic inflammation limited to the adventitia and periadventitial soft issue appeared in the epicardial large coronary arteries, chiefly in the left coronary anterior descending artery; (e) fibrinoid necrosis or granuloma could not be found in or around the inflammatory area; (f) no type of vasculitis could be found in any other tissues or organs (i.e., localized and non-systemic periarteritis); (g) ECPA was frequently accompanied by spontaneous coronary arterial dissection (SCAD) in the affected wall; and (h) ECPA without SCAD appeared mainly in men (male/female ratio was 8:3), while EPCA with SCAD appeared in almost all female cases (male/female ratio was 1:14). Although the etiology and pathogenesis are still unknown, we believe that ECPA (with or without SCAD) might be a distinct new type of coronary arteritis.