[Necrotic leg ulcers induced by vitamin K antagonists: five cases]. / Ulcères de jambe nécrotiques induits par les anti-vitamines K : à propos de cinq cas.

BACKGROUND: Vitamin K antagonists (VKAs) are widely used in thromboembolic diseases. We report five cases of necrotic leg ulcers having a particularly severe course and in which withdrawal of VKA treatment alone enabled healing. CASE REPORTS: Five patients presented with necrotic leg ulcers clinically evocative of necrotic angiodermatitis or vasculitis. Histological features were variable, including inconstantly inflammatory lesions (leukocytoclastic vasculitis) and microthrombosis. None of the patients had laboratory signs of autoimmune disease. Healing occurred in all patients only after withdrawal of VKA therapy (fluindione or acenocoumarol). Associated vascular diseases included superficial venous, distal arterial insufficiency and postphlebitic disease. In three cases, thrombotic factors were observed: hyperhomocysteinaemia or heterozygous Factor V Leiden mutation. DISCUSSION: Although the causative role of VKAs is based solely on chronological criteria, this potential side effect deserves publication because of its practical therapeutic consequences. The physiopathological mechanisms accounting for the role of VKAs, including immunoallergic phenomena and, above all, microcirculatory thrombotic processes, are hypothetical and not universally accepted.

[Polymorphic eruption of pregnancy and acquired hemophilia A]. / Éruption polymorphe de la grossesse associée à une hémophilie acquise A.

BACKGROUND: Acquired haemophilia A (AHA) is a rare and serious disease, and instances of association with skin diseases have been described. We report a case of postpartum AHA associated with atypical polymorphic eruption of pregnancy (PEP). PATIENTS AND METHODS: Following delivery of her second child, a 27-year-old woman developed a generalised pruritic erythematous papular and vesicular rash in plaques. The diagnosis of pemphigoid gestationis was ruled out on the basis of negative immunopathology results and a diagnosis of PEP was made. Lengthening of activated cephalin time was observed, without correction by addition of control plasma, and prothrombin time was normal. AHA was confirmed by the very low levels of factor VIII and the presence of antifactor VIII antibody. The patient was given intravenous activated recombinant factor VII for epistaxis and gingival bleeding, followed by an infusion of polyvalent immunoglobulins and systemic corticosteroids. Both diseases regressed within a few weeks. DISCUSSION: This case is original in terms of the atypical presentation of AHA associated with severe PEP. AHA was associated with the presence of antifactor VIII Ab. Although the disease generally occurs alone, it has already been reported during pregnancy and the postpartum period, and in association with various forms of dermatosis, including bullous pemphigoid, although to our knowledge, never in association with PEP or pemphigoid gestationis. However, neither the underlying mechanisms of this association of PEP and AHA, which was probably not a chance occurrence, nor the risks of relapse of these conditions during subsequent pregnancies have been elucidated.

[Tumoral calcinosis at an unusual site in a haemodialysis patient]. / Calcinose pseudotumorale de localisation inhabituelle chez un patient hémodialysé.

BACKGROUND: Tumour-like calcinosis is a rare cause of tissue calcification in patients on maintenance haemodialysis for chronic renal failure. Its estimated incidence is between 0.5 and 7% of haemodialysis patients. PATIENTS AND METHODS: A 29-year-old male patient was referred to our department with a large cervical mass lesion increasing in size for two months. The patient had been on maintenance haemodialysis for one year for chronic renal failure during which time he reported multiple episodes of cervical trauma. Cervical MRI demonstrated a 11 x 9 cm calcified tumoral mass extending to the cervical muscles and the lower cervical spine (C6, C7, T1), accompanied by C6 osteolysis. Laboratory studies revealed secondary hyperparathyroidism with elevated calcium-phosphorus product. The patient underwent parathyroidectomy and several weeks later, there was a dramatic regression of the tumoral calcinosis. Renal transplantation was performed secondarily with no recurrence of the tumoral calcification after six years of follow-up. DISCUSSION: Tumour-like calcinosis of the lower cervical spine with osteolysis of the cervical vertebrae is very rare. The principal contributing factors are hyperparathyroidism, elevation of calcium-phosphorus product and local trauma. Optimal treatment of these calcifications remains controversial. While surgical resection of the mass is commonly recommended, in our case report, despite the initial aggressiveness of the lesion, surgery was not performed and treatment of hyperparathyroidism alone was sufficient to ensure dramatic improvement with complete resolution of the calcinosis within few weeks.

[Bullous amyloïdis revealing a light chains lambda myeloma]. / Amylose bulleuse révélant un myélome à chaînes légères lambda.

INTRODUCTION: Although rare, skin lesions are regularly reported in patients with systemic amyloidosis. The existence of bullous skin lesions however is very rare; only thirty cases have been previously reported. We report a new case of bullous amyloidosis revealing a light chains lambda myeloma, and underline the usual characteristics of this type of systemic amyloidosis. EXEGESIS: An 85-year-old man was hospitalised for a bullous eruption associated with a general asthenia. Bullous amyloidosis revealing a light chains lambda myeloma was diagnosed and confirmed by histopathological examination of a skin biopsy specimen. The patient died of a severe congestive heart failure, 15 days later, due to cardiac involvement of the amyloidosis. CONCLUSION: Bullous amyloidosis lesions can be an early manifestation of occult dysglobulinemia. Early diagnosis would allow rapid treatment, before onset of systemic amyloidosis, which is often lethal.