Rituximab, a new treatment for difficult-to-treat chronic erythema multiforme major? Five cases.

BACKGROUND: Erythema multiforme major (EMM) is an inflammatory disease affecting skin and mucosae, often triggered by infection with Herpes simplex virus. Some patients have a chronic disease associated with antidesmoplakin autoantibodies, but the pathophysiology remains to be elucidated. First-line treatment is antiviral therapy. With treatment failure or in patients without herpes-triggered disease, thalidomide is effective but has neurological side-effects. Alternatives (dapsone, immunosuppressant agents) are not codified. For many patients, systemic steroids use is chronic. The immunosuppressant drug rituximab (RTX) may be effective. OBJECTIVES: We report five cases of severe chronic EMM treated with rituximab (RTX). METHODS: Five patients with severe chronic EMM for 9-20 years received RTX after failure or side-effects of several treatments, especially antiviral therapy and thalidomide. All had chronic use of steroids. Four patients had antidesmoplakin autoantibodies. RESULTS: Four patients experienced complete or quasi-complete remission of EMM with withdrawal of steroids and one patient partial remission, for 3-11 months. Disease relapsed in all patients, and three received a second cycle of RTX with shorter duration of efficacy. Two patients received a third cycle, one without efficacy. CONCLUSION: The use of RTX for many autoimmune diseases, especially pemphigus, is increasing. Chronic EMM, especially EMM associated to antidesmoplakin autoantibodies, is an inflammatory disease in which the role of B cells is not well understood. However, we report a favourable benefit of RTX treatment for months in five patients with severe disease. RTX could be a therapeutic option in severe, difficult-to-treat EMM.

Dermatosurgery: total quality management in a dermatology department.

BACKGROUND: Dermatosurgery (DS) is a growing sector in dermatology. Performance measurement is organized worldwide to improve the quality of health care. Clinical audit relies on self-assessment, comparison with guidelines, frames of references and implementation of improvement actions. OBJECTIVE: To assess the efficiency of our DS department. METHODS: A clinical audit focusing on the organization of the DS unit, patient routing, continuing medical education and training for students was conducted by two external auditors. After an initial evaluation, improvements were implemented and reassessed 1 year later by the same auditors. RESULTS: The audit resulted in the implementation of preoperative consultation, improved pre- and postoperative information leaflets for patients, standardizing of surgery reports, earmarking of funds for materials, and patient satisfaction survey. The training of residents was organized. CONCLUSION: This audit was a driving force for communication among the medical and paramedical teams and helped improve patient care and training of residents in DS. It also highlighted areas needing further improvement.

[Bilateral elastofibroma dorsi]. / Élastofibrome dorsal bilatéral.

BACKGROUND: Elastofibroma dorsi (EFD) is a rare form of benign soft-tissue tumour that is almost always located at the lower pole of the scapula, deep to the serratus anterior muscle. There are few reports in the dermatological literature since it rarely develops in skin or subcutaneous tissue. CASE REPORT: We present the case of a 59-year-old man with bilateral elastofibroma dorsi. Physical examination showed two bilateral scapular masses, better visualized in abduction or antepulsion. They were firm, painless, mobile, covered by normal skin, and measured 40/35 mm on the right and 50/55 mm on the left. Ultrasound of soft tissue and a chest CT scan showed two heterogeneous masses (tissue and fat) within the serratus anterior muscle measuring 30/15 mm on the right and 60/19 mm on the left. Histopathological study of a deep biopsy done on the right helped to confirm the diagnosis of EFD. Tumour resection was done on the right. DISCUSSION: Elastofibroma dorsi is an uncommon, benign, non-encapsulated pseudo-tumour occurring in connective tissue of the infrascapular region of elderly patients. Another mass must be sought in all cases in the corresponding contralateral site since this form of tumour is often bilateral. CONCLUSION: EFD must be considered in the differential diagnosis of shoulder masses in elderly patients.

[Severe necrotizing myopathy subsequent to Merkel cell carcinoma]. / Myopathie nécrosante sévère consécutive à un carcinome à cellules de Merkel.

BACKGROUND: Merkel cell carcinoma (MCC) is a rare tumour with a poor prognosis. Rare cases of paraneoplastic neurological syndrome have been associated with this type of tumour, namely myasthenic syndrome of Lambert-Eaton and encephalomyelitis. We report the first case of severe necrotizing myopathy with anti-Hu antibodies complicating MCC. CASE REPORT: We describe the case of a 58-year-old woman with Merkel cell carcinoma (MCC) of the forearm complicated by severe necrotizing myopathy associated with the presence of anti-Hu antibodies. This myopathy occurred 3 months after complete remission of MCC. The patient was treated with high-dose corticosteroids combined with two intravenous infusions of immunoglobulins. Her neurological status deteriorated despite this treatment. Pararaneoplastic syndrome (anti-Hu antibodies, necrotizing myopathy) complicating MCC was suspected. There was no visible tumour relapse. After multidisciplinary discussion, it was decided to supplement treatment with chemotherapy (carboplatin and VP-16). The patient died 20 days after the first course of chemotherapy. DISCUSSION: Severe necrotizing myopathy with anti-Hu antibodies may be added to the list of possible paraneoplastic syndromes associated with Merkel cell carcinoma.

[Cutaneous drug reactions: pharmacogenetic news]. / Réactions médicamenteuses cutanées: nouveautés pharmacogénétiques.

Identifying genetic factors in severe drug eruption should improve efficacy and safety of drug prescription. The first pharmacogenetic studies concerned the polymorphism of genes encoding drug metabolism enzymes (especially for sulfamides reactions in AIDS and anticonvulsants reactions). Recent studies did not confirm the role of genotypic variation in metabolism enzymes in drug reaction. Genes controlling immune response are another research issue. Certain MHC phenotypes are linked to a higher risk of toxidermy, such as in abacavir hypersensitivity or fixed drug eruption. Indeed, severe drug eruptions seem to be linked to a specific immune response controlled by T lymphocytes.