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Assessment of the efficacy and safety of omalizumab in chronic urticaria refractory to conventional treatment (H1-antihistamines at high dosage and montelukast) in real-life practice.A retrospective, descriptive, single-centre study was performed of the data for all patients presenting refractory chronic spontaneous urticaria or inducible urticaria and receiving omalizumab (300mg every four weeks) from November 2012 to June 2016.In all, 23 patients were included. Omalizumab led to complete or significant remission in 19 patients (83%) with chronic urticaria, with remission in 9 patients (47%) occurring within 72hours of the first injection. One patient had a partial response and 3 (13%) showed no response. Only 2 patients (9%) in complete remission stopped their treatment at 1 and 3 years. 52% of patients presented non-serious adverse events, which in one case resulted in treatment withdrawal.Omalizumab exhibited good real-life efficacy in a small series of chronic urticaria patients in France.

Fingolimod is an oral immunomodulator approved for relapsing-remitting multiple sclerosis. We report a case of a primary cutaneous CD30+ T-cell lymphoproliferation occurring 6 months after initiation of fingolimod. Based on a systematic literature review, the characteristics of these fingolimod-induced lymphoproliferative disorders are described.A 56-year-old woman developed cutaneous indurated and ulcerated nodular lesions 6 months after starting fingolimod for active relapsing-remitting multiple sclerosis. Histological examination of a punch biopsy sample demonstrated a polymorphous dermal infiltrate containing large atypical CD30+ cells, leading to diagnosis of primary cutaneous CD30+ anaplastic large-cell lymphoma. Chest-abdomen-pelvis CT scans were performed to rule out secondary cutaneous anaplastic large-cell lymphoma. Spontaneous clinical regression was observed and after assessing the benefit/risk ratio, it was decided to continue fingolimod under strict surveillance, with no relapse occurring by month 18.A systematic review of PUBMED/Medline and Embase identified seven other cases of lymphoproliferative disorders occurring during fingolimod treatment, including two other cases of primitive cutaneous CD30+ lymphoproliferative disorders.Even if cutaneous CD30+ lymphoproliferative disorders occur only rarely during fingolimod treatment, dermatologists should nevertheless be aware of this association for which strict dermatological surveillance is required. We would also stress that these CD30+ lymphoproliferative disorders can disappear spontaneously, as in our case, even if treatment by fingolimod is continued.

Lipedematous scalp, with or without alopecia, is a poorly known and rarely reported entity. It was first described in 1935 by Cornbleet. It involves increased thickness of the subcutaneous tissue of the scalp, responsible for an overall thickening of the scalp, which may be associated with alopecia, pruritus or painful sensations. Currently, fewer than 50 cases of lipedematous scalp, both with and without alopecia, have been reported in the literature.Herein we present the case of a 36-year-old woman from the Ivory Coast, who presented scalp pain associated with infiltration of the entire subcutaneous tissue of the scalp seen clinically and confirmed at MRI. Histology added nothing.We diagnosed a new case of lipedematous scalp in an African woman. No cause was found. Therapeutic abstention appears the best management strategy.

The incidence of cancer is increased in patients with systemic sclerosis (SSc). Further, recent studies have also shown that the presence of anti-RNA polymerase III antibodies is associated with a higher incidence of cancer in this population.Herein we present the cases of two men aged 56 and 23 years presenting SSc without anti-Scl70 or anti-centromere antibodies but with anti-RNA polymerase III antibodies. Clinical symptoms led us to prescribe more laboratory exams and both patients were diagnosed with cancer of the nasopharyngeal area.Anti-RNA polymerase III antibodies are useful for SSc diagnosis in patients without anti-centromere or anti-Scl70 antibodies. Their presence must lead physicians to screen for associated cancer, even in the absence of clinical signs.

The rate of hypersensitivity reactions to platinum salts (PS) and taxanes (TX) is on the increase. The aim of our study was to show the value of skin testing and efficacy of rapid drug desensitization.This was a retrospective study conducted between January 2007 and February 2016 in patients consulting for immediate or delayed hypersensitivity to PS and TX. Skin prick tests (pT) and intradermal reaction tests (IDR) were performed according to the ENDA/EAACI recommendations. We used a 12-step desensitization protocol for rapid drug desensitization.Among the 99 patients included (30 men, 69 women, age 60.4) PS were suspected in 86 cases and taxanes in 13 cases. Skin tests were positive in 25 patients (7 pT, 18 IDR), 23 for platinum salts and 2 for taxanes. Rapid drug desensitization was proposed in 50 patients and performed in 33 (30 PS and 3 TX), proved effective in 29 patients, with protocol adaptation being necessary in 7 cases, and was ineffective in 4 patients. The skin tests for the latter 4 patients were positive. Seventy-five percent of patients with positive skin tests to oxaliplatin presented hypersensitivity reactions during desensitization, i.e. twice as many as patients having negative skin tests. Two percent of patient for PS and 7% for TX had cross reactivity.This French study confirms the efficacy of the 12-step protocol that allows patients to receive chemotherapy after hypersensitivity reaction. Skin test permits the detection of cross-reactions but their practice must be considered based on the patient's history.

Introduction L’argan est tres utilise en cosmetologie depuis quelques annees, notamment pour ses proprietes supposees benefiques pour la sante. Nous rapportons ici les premiers cas de rhinites et d’asthmes professionnels decrit chez des agents de production d’une societe de fabrication d’actifs pour cosmetiques, en lien avec la manipulation de poudre d’argan. Methode Neuf patients travaillant dans une entreprise de production d’actifs pour cosmetiques ont ete explores suite a l’apparition chez 4 d’entre eux de symptomes ORL et/ou respiratoires en relation avec la manipulation de poudre d’argan. Tous etaient occasionnellement amene a manipuler de l’argan sous forme de poudre et de liquide, sans mesure de protection respiratoire specifique. Tous ont repondu a un questionnaire medical et professionnel, puis ont beneficie d’examens complementaires comprenant : un suivi de debit expiratoire de pointe (DEP), un test de provocation bronchique a la metacholine, une recherche d’IgE specifique de l’argan par immunoblot , des prick-tests a l’argan et a divers autres allergenes vegetaux. Pour ceux suspects de presenter un asthme professionnel, un test de provocation bronchique realiste avec de la poudre d’argan a ete realise. Enfin, une recherche de sequences peptidiques analogues a d’autres vegetaux a ete realisee par analyse en spectrometrie de masse. Resultats Sur les 4 agents symptomatiques, 3 presentaient des prick-tests positifs pour au moins un allergene standard et la noisette. Deux des 3 avaient egalement un prick-test positif a l’argan. Les 3 agents rapportant des symptomes d’asthme presentaient un test de provocation bronchique realiste positif a la poudre d’argan. Sept sequences proteiques etaient identifiees par immunoblotting de la poudre d’argan avec le serum de patients sensibilises. En revanche, aucune sequence n’etait mise en evidence lors de l’application de la meme technique sur un extrait liquide d’argan, suggerant la denaturation des proteines allergeniques par le processus de transformation. L’identification des proteines par comparaison avec des banques de donnees d’extraits allergeniques vegetaux, la sensibilisation associee a la noisette documentee par prick-test et les tests Elisa d’inhibition des IgE de l’argan par un extrait de noisette suggeraient le role allergenique de 2 proteines en particulier : une viciline et une globuline 11 S. Conclusion Une exposition habituelle a de fortes concentrations d’argan sous forme de poudre semble pouvoir favoriser l’apparition d’allergies respiratoires IgE mediees. Il semble important de proteger efficacement et de surveiller medicalement les salaries amenes a manipuler ce type de substance.

To evaluate the vaccine coverage of psoriasis patients prior to initiating or changing immunosuppressant therapy, and to verify that the prescribed vaccines have been administered.We conducted a bi-centre, observational, cross-sectional study over 9 months. Psoriasis patients in whom immunosuppressant therapy (comprising cyclosporine, methotrexate, etanercept, infliximab, adalimumab or ustekinumab) was indicated were included. Medical history, previous treatments, vaccination status, viral serology results (for hepatitis B, measles, and chickenpox), and reasons for non-vaccination were assessed via questionnaire.Sixty-eight patients were included. One third brought their immunization records. Overall, 54.4% had already received immunosuppressant therapy; of these, 9 were up to date for influenza and 3 were up to date for pneumococcus. Only one patient was up to date for all of the recommended vaccinations. A total of 61% of patients were seronegative for hepatitis B. The following vaccines were updated: DTP (in 2 patients), DTP-pertussis (12), influenza (22), pneumococcus (45), and hepatitis B (6). None of the three patients with plans to travel to yellow fever-endemic countries had been vaccinated. In all, 53 (78%) stated that they had already had chickenpox and 43 (63.2%) stated that they had had one of the following three diseases: measles, rubella, or mumps. Fifty-two patients were serologically tested for chickenpox, and 98% were immunized. The most common reasons for not updating the immunization schedule were the absence of any notification or proposal by the patient's doctor and oversight.This study should help raise awareness among patients and health professionals concerning the new vaccination recommendations for a population particularly at risk of infection.

Skin patch-tests in dermatology-allergology practice require good preparation. To this end, the dermatology-allergology group of the French Society of Dermatology introduced an information notice informing patients about patch testing procedures. The aim of this study was to evaluate the utility and understanding of the notice.The information notice was sent out to patients before testing. On the day of the test, a questionnaire was submitted to patients to evaluate their comprehension of the notice. Another questionnaire was submitted simultaneously to the dermatology-allergology practitioner to evaluate whether the patient had complied with the guidelines given in the information notice. Paired questionnaires were analyzed for this study.Eight dermatology-allergology hospital departments participated in the study and collected 921 paired questionnaires over a period of 18months. Among the vast majority (96.2%) of patients who had read the information notice, most found it useful (98.8%), easy to read (97.4%), and appropriate (91.5%). Ten percent of patients had difficulty understanding.This study shows that the information notice was clear and explicit for the immense majority of patients. Thanks to the feedback of a number of patients, the information notice was further improved to enhance patient understanding.

Leukocytoclastic vasculitis is characterized by necrotizing inflammation around small blood vessels, composed mainly of neutrophils. Skin lesions in leukocytoclastic vasculitis are polymorphous, but a rare annular variant exists of which dermatologists must be aware. Herein we present a new case of this entity.We report the case of a 74-year-old man who developed annular purpuric infiltrated lesions mainly on the lower limbs, with leukocytoclastic vasculitis being confirmed by histology. This annular leukocytoclastic vasculitis regressed spontaneously with no recurrence after 6 months. The aetiology was not established.Annular leukocytoclastic vasculitis is rare, and the mechanisms underlying annularity and peripheral spread as well as the aetiology remain unexplained. This form of vasculitis with unusual clinical features may constitute a new entity in the subclass of immune complex small vessel vasculitis within the Chapel Hill classification. While the aetiology and physiopathology of this vasculitis are unknown, despite the possibility of recurrence, skin involvement appears isolated and the condition carries a good prognosis.

Dermatomyositis (DM) is an inflammatory disease associated with auto-antibodies in 50 to 70% of cases. A new antibody, anti MDA-5, has been described in association with a specific type of DM involving severe interstitial lung disease and minimal muscle disease. We report the first case of DM with MDA-5 antibodies and with interstitial lung disease and rapidly extensive digital necrosis.A 28-year-old male was hospitalized for asthenia, myalgia and subacute dyspnea. Examination demonstrated skin lesions with edema on every digit associated with purpuric and cyanotic lesions, as well as erythematous papules on the helix and the elbows, and Gottron's papules. Systemic corticosteroid therapy was initiated. The immunoprecipitation results indicated the presence of anti-MDA-5 antibodies. Despite corticosteroid therapy, the patient's respiratory status gradually deteriorated towards pulmonary fibrosis and rapidly extensive necrosis appeared on all fingers and toes. Theses effects were resistant to cyclophosphamide and immunoglobulin but were stabilized by cyclosporine.Anti-MDA-5 antibodies are specific to DM and constitute a risk factor for severe interstitial lung disease (70% of cases) with a higher risk of mortality (40%). The cutaneous presentation of this DM is specific with palmar papules and mucocutaneous ulceration. Rapidly extensive digital necrosis has not been previously reported. No treatment has demonstrated superiority.We report the first case of DM with anti-MDA-5 antibodies involving interstitial lung disease and massive digital necrosis. Because of the pulmonary risk, in the presence of clinical lesions containing anti-MDA-5 DM, screening for these antibodies should be carried out.

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