Clinical efficacy of intravenous immunoglobulins for the treatment of dermatomyositis skin lesions without muscle disease.

BACKGROUND: Treating dermatomyositis (DM) with isolated skin involvement is difficult and inconsistently performed. Intravenous immunoglobulins (IVIg) are recommended for corticoresistant or corticodependant DM, but only a few cases of IVIg use in DM with isolated skin involvement have been reported. DESIGN: We performed a retrospective monocentric study of 27 patients who were treated with IVIg for severe DM skin lesions (no or minor muscle involvement) after failure of photoprotection and at least one line of treatment. RESULTS: Nineteen patients (70%) exhibited a major response, four patients exhibited a partial response and four patients exhibited no response, including two patients with grade 3 side effects (headaches). The mean number of IVIg courses was 4.8 (range 1-15). Ten patients (53%) relapsed, with a median time of 6.2 months after the last IVIg course. Six of these patients were successfully treated with a new IVIg course. Muscle disease developed in six patients. CONCLUSION: IVIg may be an effective and safe treatment for DM with isolated skin involvement. Relapse occurred frequently, but treatment with a new course of IVIg was successful. Controlled studies are required to confirm these results.

[Cutaneous revelation of Rosai-Dorfman disease: 7 cases]. / Maladie de Rosai-Dorfman à révélation cutanée: 7 observations.

BACKGROUND: Rosai-Dorfman disease (RDD) is a benign form of non-Langerhans-cell histiocytosis. It is identified by a particular histological profile first observed in febrile lymph nodes. Extranodal sites are frequent. The most common site is the skin, which can reveal the disease despite a difficult and delayed diagnosis. Seven cases of cutaneous revelation of RDD were studied retrospectively in order to delineate the clinical characteristics and facilitate diagnosis and treatment of this extremely rare disease. PATIENTS AND METHODS: Six cases of RDD from 1990 to 2011 were identified in the photographic and histopathological records of the Saint-Louis Hospital and one case came from a Bichat Hospital consultation. The diagnosis was based in all cases on histopathology results. RESULTS: Patients consisted of four men and three women aged between 31 and 69 years. Cutaneous lesions (3 to 20) revealed the disease in all of them and the time from disease onset to diagnosis ranged from six months to five years. The clinical presentation was erythematous or orange popular nodules or plaques, usually on the face. Microscopically, a dense dermal infiltration was observed, in some cases extending into the subcutaneous tissue, with pale histiocytic cells characterised by emperipolesis, plasma cells, lymphocytes, some neutrophils and variable fibrosis. The diagnosis, initially erroneous in 4 cases, was rectified by a second reading of histopathology slides, and immunohistochemical studies showed expression of S-100 protein in histiocytes but not CD1a. Three patients had pure cutaneous RDD. Two neurological sites and one nasal site were also found, with one ENT site and sequelae of previous uveitis in one patient. All extra-cutaneous sites were identified by clinical examination. Different treatments were proposed according to the sites and impact of the disease. In one case, the lesions regressed spontaneously after 18 months. COMMENTS: Few RDD series have been published and they mainly concern Asian patients. The ethnic origin of our patients was varied. The main findings were: 1) common clinical findings (orange or erythematous papules or nodules, mostly on the upper body), which should alert the dermatologist and histopathologist to the possible diagnosis of RRD; 2) the possibility, already mentioned in the literature, of spontaneous regression and a good prognosis; 3) the need for thorough evaluation by thoracic, abdominal and cerebral CT (computed tomography) or more a PET (positron emission tomography) scan to screen for potentially dangerous visceral sites, and also clinical follow up.

[Cutaneous manifestations revealing cryofibrinogenaemia associated with monoclonal gammopathy]. / Manifestations cutanées révélant une cryofibrinogénémie associée à une gammapathie monoclonale.

BACKGROUND: Cryofibrinogenaemia is an under-recognized cutaneous thrombotic vasculopathy that may be revealed by purpura or chronic necrotic ulcerations. We report two original cases characterized by their severity, their association with a monoclonal gammopathy and their excellent response to treatment. PATIENTS: A 38-year-old woman was admitted for large necrotic leg ulcers appearing 1 year earlier and already investigated. Non-specific signs were seen on a previous skin biopsy and the diagnosis of a factitious disorder was considered at that time. Further investigations revealed circulating cryofibrinogen associated with IgG kappa monoclonal gammopathy without cryoglobulinaemia. Plasmapheresis followed by bortezomid-dexamethasone to treat the monoclonal gammopathy resulted in rapid and complete healing of the ulcers, militating in favour of its involvement in cryofibrinogen formation. The second patient, a 91-year-old woman, was referred to our department for acute necrotic purpura of the legs. Skin biopsy revealed leukocytoclastic vasculitis. Glomerular nephropathy with acute renal failure and multiple arterial thromboses were associated with the skin condition. The cryofibrinogen assay was positive without cryoglobulinaemia and other causes of vasculitis were ruled out. The main component was monoclonal IgG lambda. Prednisone-cyclophosphamide treatment led to complete healing of the skin lesions and to recovery from the systemic consequences of cryofibrinogen without sequelae. CONCLUSION: Routine screening for cryofibrinogen in plasma should be performed to explore cutaneous symptoms of unexplained thrombotic vasculopathy, even in the presence of a non-specific skin biopsy. Specific treatment of cryofibrinogenaemia associated monoclonal gammopathy appears to be highly effective against manifestations of cryofibrinogenaemia.

Management of cutaneous adverse events induced by anti-EGFR (epidermal growth factor receptor): a French interdisciplinary therapeutic algorithm.

PURPOSE: Cutaneous adverse events induced by epidermal growth factor receptor (EGFR) inhibitors can hamper the patients' quality of life. The aim of our work was to draft an algorithm for the optimised management of this skin toxicity. METHODS: This algorithm was built in three steps under the responsibility of a steering committee. Step I: a systematic literature analysis (SLA) has been performed. Step II: the collection of information about practices was performed through a questionnaire.These questions were asked during regional meetings to which oncologists, gastro-enterologists, radiotherapists, and dermatologists were invited. Step III: a final meeting was organised involving the bibliography group and the steering committee and regional scientific committees for proposing a final algorithm. RESULTS: Step I: 14 publications were selected to evaluate the use of cyclines as curative or prophylactic treatment of the folliculitis induced by EGFR inhibitors. Nineteen publications were retained for the topical treatment of the folliculitis. Forty-six articles were selected for the management of the cutaneous lesions in link with appendages and 12 for xerosis and pruritus. Step II: 96 delegates attended the seven regional meetings and 67 questionnaires were analysed. Step III: a final algorithm was proposed on the basis of the conclusions of the first two steps and expert opinions present at this final meeting. The different propositions were unanimously approved by the 14 experts who voted. CONCLUSIONS: This multidisciplinary study summarising published data and current practices produced a therapeutic algorithm, which should facilitate the standardised, optimised management of skin toxicity associated with EGFR inhibitors in France.

[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.

[Tobacco contact dermatitis caused by sensitivity to sorbic acid]. / Eczéma de contact au tabac par sensibilisation à l'acide sorbique.

BACKGROUND: Contact dermatitis caused by tobacco is rare and poorly understood. In this paper, we report what is to our knowledge, the first case of tobacco contact dermatitis with identification of the causative agent. PATIENTS AND METHODS: A 46-year-old man was hospitalised for diffuse dermatitis originating in the left leg. Subsequent epicutaneous tests indicated that this dermatitis was ascribable to sensitisation to the sorbic acid present in Fucidin cream applied around a chronic wound. A persistent plaque of eczema observed on the day of examination opposite the patient's right trouser pocket suggested the implication of tobacco powder with which the pocket was soiled. An epicutaneous test using tobacco (moistened in water) proved positive (++ at 48 and 72 h). This tobacco contained sorbic acid, used as a preservative. DISCUSSION: The positive test for sorbic acid was relevant not only regarding the lesions on the patient's legs (application of a topical cream containing sorbic acid) but also for the lesion on the patient's thigh and for palmar dyshidrosis (through contact with strands of loose rolling tobacco). To our knowledge, this is the first recorded case of contact dermatitis caused by sorbic acid in tobacco.