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Annales de Dermatologie et de Vénéréologie
Volume 142, n° 8-9
pages 506-512 (août 2015)
Doi : 10.1016/j.annder.2015.06.009
L’artériolopathie calcifiante urémique et non urémique : urgence diagnostique et thérapeutique
Uraemic and non-uraemic calcific arteriopathy: The need for rapid diagnosis and therapy
 

G. Perceau
 Service de dermatologie, hôpital Robert-Debré, CHU de Reims, avenue du Général-Koenig, 51092 Reims cedex, France 

Résumé

L’artériolopathie calcifiante (AC), souvent nommée aussi calciphylaxie, est une pathologie de mauvais pronostic. Elle se rencontre principalement chez les patients insuffisants rénaux chroniques (IRC), en particulier hémodialysés (HD), où elle est nommée artériolopathie calcifiante urémique (ACU), mais aussi en dehors de cette situation où elle est nommée artériolopathie calcifiante non urémique (ACNU). Elle correspond à une microangiopathie artériolaire thrombosante cutanée en rapport avec des dépôts calciques fins de la média associés à une hyperplasie de l’intima des artérioles dermiques et hypodermiques. Elle évolue en 2 phases : une phase silencieuse d’installation des anomalies artériolaires, puis souvent à la faveur d’éléments déclenchants, une phase symptomatique avec apparition brutale de placards ischémiques nécrotiques cutanés. Il existe plusieurs formes cliniques : proximales, distales et mixtes en fonction de la localisation préférentielle des lésions. Le pronostic est sombre, en raison de complications septiques et de l’atteinte d’autres organes. Son diagnostic repose sur le terrain, la clinique, les examens biologiques et la biopsie cutanée avec coloration de Von Kossa. La physiopathologie est de mieux en mieux connue et complexe, faisant intervenir un produit phosphocalcique élevé, une hyperparathyroïdie mais aussi d’autres facteurs (inflammation, facteurs pro-calcifiants, facteurs inhibiteurs de la calcification, troubles de la coagulation, traumatismes). Son traitement est multidisciplinaire et médico-chirurgical.

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Summary

Calcific arteriopathy (CA), also commonly known as calciphylaxis, is a disease carrying a poor prognosis. It is seen primarily in patients with chronic renal insufficiency (CRI), particularly those on haemodialysis (HD), where it is referred to as uraemic calcific arteriopathy (UCA), but it also occurs outside this setting, in which case it is known as non-uraemic calcific arteriopathy (NUCA). It is caused by thrombotic cutaneous arteriolar microangiopathy associated with fine calcium deposits in the media associated with hyperplasia of the intima of the dermal and hypodermal arterioles. Its course comprises two phases: a silent phase in which the arteriolar abnormalities begin, followed by a symptomatic phase, frequently triggered by specific factors, with sudden appearance of necrotic ischaemic plaques on the skin. Several clinical forms exist, proximal, distal and mixed, depending on the main site of the lesions. The prognosis is poor due to septic complications and the involvement of other organs. Diagnosis is based on the patient's history, clinical examination, laboratory examinations and skin biopsy with Von Kossa staining. The physiopathology, which is complex and is becoming increasingly well understood, involves high phosphorous and calcium levels and hyperparathyroidism, as well as other factors (inflammation, factors promoting or inhibiting calcification, coagulation disorders and traumatisms). Treatment involves a multidisciplinary and medical-surgical approach.

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Mots clés : Artériolopathie calcifiante urémique, Artériolopathie calcifiante non urémique, Calciphylaxie, Nécrose cutanée

Keywords : Uraemic calcific arteriopathy, Non-uraemic calcific arteriopathy, Calciphylaxis, Skin necrosis




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