[Dermatomyositis-like syndrome revealing statin-induced necrotizing autoimmune myopathy with anti-HMGCR antibodies]. / Myopathie nécrosante auto-immune à anticorps anti-HMGCR induite par les statines et révélée par un tableau évocateur de dermatomyosite.

BACKGROUND: Statin-induced necrotizing autoimmune myopathy (NAM) has been recently characterized. Herein we report an accurate description of the clinical and histological characteristics of cutaneous rash associated with NAM. PATIENTS AND METHODS: A 61-year-old woman presented a skin rash involving the face, the chest and the back of the hands with heliotropic distribution coupled with proximal symmetrical muscle weakness. Rosuvastatin had been introduced 8 months earlier. Creatinine kinase levels were dramatically raised. Screening for lupus and dermatomyositis antibodies were negative. The cutaneous histology was consistent with neutrophilic lupus while a muscle biopsy revealed no inflammation but showed necrotic and regenerative myofibres. Finally, antibodies directed against 3-hydroxy-3-methylglutaryl-coenzyme A reductase (HMGCR) were found at high levels (1658UA/ml vs. normal<13.0UA/ml), resulting in diagnosis of necrotizing autoimmune myopathy (NAM). Intensive immunosuppressive therapy resulted in excellent improvement. DISCUSSION: NAM is a severe acquired autoimmune myopathy characterised by severe proximal weakness and specific positive antibodies (anti-HMGCR or anti-signal recognition particle). It is classically associated with statin use. Some extra-muscular symptoms have been described in previous studies. We report the third accurate description of cutaneous rash associated with statin-induced NAM involving HMGCR antibodies. The skin rash was evocative of connective tissue disease and our diagnosis was based on immunology and muscle histology. CONCLUSION: Dermatologists must be able to recognise this rare entity of "pseudo-dermatomyositis" and then discontinue statin intake if present and carry out further investigations consisting of muscle biopsy and serological tests.

[Pemphigus and herpes: Multicentre survey and literature review]. / Pemphigus et herpès : enquête multicentrique et revue de la littérature.

BACKGROUND: Although herpes superinfection is a well-known complication of pemphigus, it has not been widely investigated. AIM: To investigate the frequency and features of herpes infection in patients with ongoing pemphigus. PATIENTS AND METHODS: We carried out a multicenter retrospective study between 2008 and 2016 in patients with newly diagnosed pemphigus presenting active herpes infection. Clinical, virological, immunological and therapeutic data were collated. We performed a literature review for pemphigus and herpes. RESULTS: Among the 191 pemphigus patients, screening for herpes (PCR or culture) was carried out in 11 to 71 % of subjects, depending on the center in question. Twenty-four patients (12 women, mean age 58 years) presented at least one episode of herpes infection. The frequency of positivity ranged from 0 to 42 % by center. Twenty-one cases consisted of pemphigus vulgaris and infection occurred at a mucosal site in 19 patients. Herpes infection was identified at the time of diagnosis in 15 patients and 17 patients received no specific treatment for their pemphigus. The virus was identified using PCR in 23 cases. Ten patients subsequently received prophylactic treatment for herpes. The mean duration of follow-up was 36 months (0-89 months). Thirteen of the 24 patients had 23 relapses of pemphigus; PCR testing for herpes was performed 19 times and was positive in 6 cases (31.5 %). CONCLUSION: Our study showed wide variation in the incidence of herpes superinfection in patients with pemphigus, reflecting the different screening approach at each center (being performed either routinely or only in the event of strong suspicion). The prognostic value of routine screening for herpes in patients with active pemphigus lesions remains to be demonstrated by further prospective investigations.

Bacterial chondritis complications following ear piercing.

BACKGROUND: Body piercing has become widespread and is associated with increased complications. Post-piercing chondritis may lead to severe residual deformity. We aimed to report case patients presenting with post-piercing chondritis in our department and to describe clinical features and treatment. PATIENTS AND METHODS: We conducted a retrospective study of patients presenting with post-piercing chondritis in the infectious disease department of Tenon Hospital, Paris, France. RESULTS: We included 21 patients. Fifteen bacteriological cultures were positive (7 Pseudomonas aeruginosa, 5 Staphylococcus aureus, and three other). Dual intravenous antibiotic therapy was administered to 13 patients for a median duration of six days [2-8], replaced by an oral antibiotic therapy for a median duration of 15 days [7-40]. Eight patients received oral antibiotic monotherapy for 10 days [7-30]. Median duration of antibiotic therapy was 16 days. Earring removal was performed for 18 patients. No ear deformity or general complication was reported. CONCLUSION: Transcartilaginous ear piercing may lead to infectious complications or deformity. In case of chondritis, early administration of an antibiotic therapy active against P. aeruginosa and S. aureus is recommended. Specific guidelines are needed.