Puffy hand syndrome Chouk, Mickaël; Vidon, Claire; Deveza, Elise ...
Revue du rhumatisme (Ed. française : 1993),
October 2016, 2016-10-00, Volume:
83, Issue:
5
Journal Article
La toxicomanie intraveineuse est responsable de nombreuses complications notamment cutanées et infectieuses. Il existe un syndrome rarement observé en rhumatologie se traduisant par des « grosses ...mains » : le puffy hand syndrome. Nous en rapportons deux cas rencontrés en consultation de rhumatologie chez deux patients toxicomanes. Il s’agit d’un œdème des mains, bilatéral, indolore, ne prenant pas le godet, survenant chez un de nos patients pendant l’intoxication par héroïne, et chez l’autre 2ans après avoir arrêté ses injections. Chez nos deux patients les bilans complémentaires (biologiques, radiologiques, échographiques) étaient sans particularité, ce qui a permis dans le contexte de poser le diagnostic de puffy hand syndrome. La physiopathologie, mal connue, repose en partie sur une toxicité lymphatique des drogues et de leurs excipients. Il n’existe pas de traitement étiologique mais une compression par manchettes nocturnes a permis d’améliorer l’œdème des mains chez un de nos patients.
Angio-oedema is a transitory, localized, noninflammatory oedema of subcutaneous tissue or mucous. When the oedema affects the mouth, lips, tongue or larynx, it can result in fatal asphyxiation in the ...absence of specific treatment. Oedema secondary to plasma extravasation is usually mediated by either histamine or bradykinin. As laboratory tests are not available in an emergency setting, the implicated mediator cannot be readily determined. The challenge for the emergency physician is to determine the aetiological type, evaluate severity and initiate adapted treatment by means of a structured approach. A team of experts from the French Reference Centre for Angio-oedema reached a consensus for recommendations for the diagnostic and therapeutic strategy to be adopted by emergency departments faced with angio-oedema of the upper airways in adults. The experts defined 11 important questions. Responses were rated using a two-round Delphi methodology. The 11 recommendations were related to triage on admission, a step-by-step diagnostic protocol, definition of attack severity, discouragement of instrumental examination, prioritization of treatment for severe attacks according to clinical signs and anticipation of access to specific treatments by the hospital. Angio-oedema of the upper airways can be fatal and requires anticipation by the emergency department. A search for the aetiology, an evaluation of clinical symptoms and the availability of the treatments are challenges justifying these recommendations.
Les angioedèmes (AE) bradykiniques sont soit associés à un déficit héréditaire ou acquis en C1Inhibiteur (C1Inh) soit associés à un C1Inh normal dans certaines formes héréditaires et en cas ...d’iatrogénie (AE secondaire aux inhibiteurs de l’enzyme de conversion de l’angiotensine). Quand il existe une forte suspicion clinique d’angioedème bradykinique, une exploration de C1Inh doit être réalisée. Cette exploration doit toujours comporter un dosage fonctionnel et pondéral de C1Inh ainsi qu’un dosage du C4. Un déficit pathologique en C1Inh peut être affirmé lorsque les taux sont inférieurs à 50 % des valeurs normales sur 2 prélèvements distincts. Si le déficit en C1Inh se confirme, des examens plus approfondis doivent être réalisés afin de déterminer le caractère héréditaire ou acquis du déficit. Le dosage du C1q et la recherche d’un anticorps anti-C1Inh peuvent alors être proposés. La recherche d’une mutation sur le gène SERPING1 s’impose, même en l’absence d’antécédents familiaux. L’absence de déficit en C1Inh n’exclut pas un angioedème héréditaire. Dans ce cas-là, après validation du tableau clinique par un expert du CREAK, la recherche de mutation sur les gènes F12 et PLG sera proposée.
Bradykinin mediated angioedema (BK-AE) can be associated either with C1Inhibitor deficiency (hereditary and acquired forms), either with normal C1Inh (hereditary form and drug induced AE as angiotensin converting enzyme inhibitors…). In case of high clinical suspicion of BK-AE, C1Inh exploration must be done at first: C1Inh function and antigenemy as well as C4 concentration. C1Inh deficiency is significant if the tests are below 50 % of the normal values and controlled a second time. In case of C1Inh deficiency, you have to identify hereditary from acquired forms. C1q and anti-C1Inh antibody tests are useful for acquired BK-AE. SERPING1 gene screening must be done if a hereditary angioedema is suspected, even if there is no family context (de novo mutation 15 %). If a hereditary BK-AE with normal C1Inh is suspected, F12 and PLG gene screening is suitable.
Since the introduction of sentinel lymph node biopsy (SLNB), its use as a standard of care for patients with clinically node-negative cutaneous melanoma remains controversial. We wished to evaluate ...our experience of SLNB for melanoma.
A single center observational cohort of 203 melanoma patients with a primary cutaneous melanoma (tumour thickness > 1 mm) and without clinical evidence of metastasis was investigated from 2002 to 2009. Head and neck melanoma were excluded. SLN was identified following preoperative lymphoscintigraphy and intraoperative gamma probe interrogation.
The SLN identification rate was 97%. The SLN was tumor positive in 44 patients (22%). Positive SLN was significantly associated with primary tumor thickness and microscopic ulceration. The median follow-up was 39.5 (5-97) months. Disease progression was significantly more frequent in SLN positive patients (32% vs 13%, p = 0.002). Five-year DFS and OS of the entire cohort were 79.6% and 84.6%, respectively, with a statistical significant difference between SLN positive (58.7% and 69.7%) and SLN negative (85% and 90.3%) patients (p = 0.0006 and p = 0.0096 respectively). Postoperative complications after SLNB were observed in 12% of patients.
Our data confirm previous studies and support the clinical usefulness of SLNB as a reliable and accurate staging method in patients with cutaneous melanoma. However, the benefit of additional CLND in patients with positive SLN remains to be demonstrated.
Gluten intolerance and skin diseases Humbert, Philippe; Pelletier, Fabien; Dreno, Brigitte ...
EJD. European journal of dermatology,
2006 Jan-Feb, Volume:
16, Issue:
1
Journal Article
Peer reviewed
Gluten sensitivity with or without coeliac disease (CD) symptoms and intestinal pathology has been suggested as a potentially treatable cause of various diseases. CD is a chronic disease which ...improves on withdrawal of wheat gliadins and barley, rye and oat prolamins from the diet. There have been numerous reports linking CD with several skin conditions. A body of evidence shows that dermatitis herpetiformis is actually a cutaneous manifestation of CD. Autoimmune diseases, allergic diseases, psoriasis and miscellaneous diseases have also been described with gluten intolerance. Dermatologists should be familiar with the appraisal of gluten sensitive enteropathy and should be able to search for an underlying gluten intolerance (GI). Serological screening by means of antigliadin, antiendomysial and transglutaminase antibodies should be performed. HLA typing is often useful in association with serologic tests. Intestinal biopsy is usually needed to establish the diagnosis of CD or GI. Thus, gluten intolerance gives rise to a variety of dermatological manifestations which may benefit from a gluten-free diet.
Bradykinin mediated angioedema (BK-AE) can be associated either with C1Inhibitor deficiency (hereditary and acquired forms), either with normal C1Inh (hereditary form and drug induced AE as ...angiotensin converting enzyme inhibitors…). In case of high clinical suspicion of BK-AE, C1Inh exploration must be done at first: C1Inh function and antigenemy as well as C4 concentration. C1Inh deficiency is significant if the tests are below 50 % of the normal values and controlled a second time. In case of C1Inh deficiency, you have to identify hereditary from acquired forms. C1q and anti-C1Inh antibody tests are useful for acquired BK-AE. SERPING1 gene screening must be done if a hereditary angioedema is suspected, even if there is no family context (de novo mutation 15 %). If a hereditary BK-AE with normal C1Inh is suspected, F12 and PLG gene screening is suitable.
Although primary cutaneous lymphomas (PCL) are the second most common group of extra-nodal non-Hodgkin lymphomas, few epidemiological data are available in the literature, and most of them are ...provided by large databases from population-based cancer registries in the US or patients attending a single institution. We conducted this study to investigate the epidemiological and clinical features of PCL diagnosed in the département of Doubs from 1980 to 2003. Data were collected from the Doubs cancer registry from 1980 to 2003. Seventy-one patients with PCL were investigated. 82% were cutaneous T-cell lymphoma (CTCL) and 18% were cutaneous B-cell lymphoma (CBCL). Among CTCL, mycosis fungoides (MF) represented 58% and Sezary syndrome 10%. The standardised incidence rate of PCL was 0.42 for 100 000 person-years and significantly increased from 0.21 in 1980-1984 to 0.70 in 2000-2003 (p <0.05). The incidence rate of CTCL was 0.34 for 100 000 person-year and significantly increased from 0.2 to 0.57 (p <0.05). For MF and CBCL, the incidence rates were 0.20 and 0.08, respectively and did not vary significantly from 1980-1984 to 2000-2003. Five-year survival was 64.5% for PCL patients similar to MF patients. Our results provide updated data on the incidence of PCL in France.
Background. There are few epidemiological data available on rare skin cancer, including Merkel cell carcinoma, Paget's disease, adnexal carcinoma, and sarcoma. We conducted this study to investigate ...the epidemiological of rare skin cancer diagnosed in the département of Doubs from 1980 to 2004. Methods. Data were collected from a population-based cancer registry from 1980 to 2004. Diagnosis was based on the 3rd edition of the International Classification of Diseases for Oncology. The incidence rates were standardized on world population. Results. One hundred and fifty one patients were investigated (88 women and 63 men). Median age for the diagnosed disease was 63 years. The standardized incidence rate was 0.82/100 000 person-year (95% CI = 0.68-0.96) and increased from 0.25 in 1980-1984 to 1.50 in 2000-2004. Fifty nine cases (39%) were sarcomas, 35 (23%) adnexal carcinomas, 27 (18%) Merkel cell carcinoma and 27 (18%) Paget's disease. The standardized incidence rates were 0.37/100 000 (0.27-0.47) for sarcomas, 0.16 (0.10-0.22) for adnexal tumors, 0.13 (0.08-0.18) for Merkel cell carcinoma, and 0.15 (0.09-0.21) for Paget's disease. Conclusions. Our results based on a population-based cancer registry showed an increase of the standardized incidence rate for all types of rare skin tumors. These results may be useful when considering the growing interest in rare diseases in identifying risk factors and planning scientific research programmes.
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