[Paroxysmal finger haematoma (Achenbach syndrome)]. / Hématomes digitaux spontanés paroxystiques (syndrome d'Achenbach).

BACKGROUND: Paroxysmal finger haematoma (also known as "Achenbach syndrome") is a benign condition resulting in the sudden appearance of bruising on one or more fingers, either spontaneously or after minimal trauma, and resolving without treatment. To date, less than 40 cases have been reported. PATIENTS AND METHODS: We report two cases of women aged over 50 years presenting for sudden onset of digital haematomas occurring spontaneously without any prior trauma. Laboratory and radiological tests appeared to be normal for both patients. History-taking and clinical and laboratory data pointed towards a diagnosis of spontaneous paroxysmal finger haematoma. Each episode resolved spontaneously but, as is generally seen, recurrences occurred during follow-up. DISCUSSION: In the absence of known aetiologies and/or treatments for spontaneous paroxysmal finger haematomas, a knowledge of this rare condition can at least help doctors reassure their patients by diagnosing their condition and pointing out the benign nature thereof. It also helps avoid costly and unwarranted additional investigations.

[Peripheral neuropathy with autonomous nervous system involvement in the course of dermatomyositis]. / Neuropathie périphérique avec atteinte du système nerveux autonome au cours d'une dermatomyosite.

A peripheral neuropathy occurs rarely during the course of an inflammatory myopathy. Once the classical aetiologies of peripheral neuropathies are ruled out, the diagnosis of neuromyositis can be accepted. We report a patient with dermatomyositis who presented a peripheral neuropathy revealed by dysautonomia. The presence of associated vasculitis led us to consider that this constituted the mechanism of the neurological involvement.

[Cholesterol crystal emboli presenting as a mononeuropathy]. / Maladie des embolies de cristaux de cholestérol révélée par un tableau de mononeuropathie.

Cholesterol crystal embolism is observed in polyvascular patients usually following a medical procedure. We report a 93-year-old patient with lower limb mononeuropathy in whom cholesterol crystal embolism was confirmed by neuromuscular biopsy. Previously, an ophthalmoscopy and a livedo biopsy had been performed and the results were normal. Mononeuropathy simplex or multiplex is a clinical expression for cholesterol crystal embolism that may thus be confounded with polyarteritis nodosa. Neuromuscular biopsy can provide accurate diagnosis if other investigations are inconclusive.

[DRESS syndrome caused by phenylbutazone]. / DRESS syndrome à la phénylbutazone.

The drug rash with hypereosinophilia and systemic symptoms (DRESS) syndrome is a severe drug-induced hypersensitivity syndrome. We report a 57-year-old woman suffering from a DRESS syndrome 15 days after phenylbutazone exposure. She had a skin eruption, liver involvement and hypereosinophilia. She fully recovered after drug withdrawal.

Infliximab efficacy and safety against refractory systemic necrotising vasculitides: long-term follow-up of 15 patients.

BACKGROUND: Results of uncontrolled studies have suggested that infliximab is efficacious against systemic necrotising vasculitides (SNV) refractory to conventional treatment. However, its safety and ability to induce and maintain remission over the long term remain unknown. OBJECTIVES: To report the use of infliximab to treat refractory SNV, focusing on patients' longer-term outcomes. METHODS: The medical charts of patients given adjunctive infliximab for refractory SNV >/=2 years before this evaluation were reviewed retrospectively. RESULTS: The 15 patients (median age 46 (range 20-69) years, median follow-up 35 (24-41) months) included 10 with Wegener's granulomatosis, 1 microscopic polyangiitis, 3 rheumatoid arthritis-associated and 1 cryoglobulinaemia-related vasculitides. Infliximab was taken for a median time of 8 (2-31) months; 2 patients are still being treated. By day 45, 11 patients had entered remission (Birmingham Vasculitis Activity Score (BVAS) = 0) and 4 others had responded (BVAS decrease >/=50%). Five patients achieved sustained remissions (>/=6 months, corticosteroids