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Resume Introduction Le pemphigus paraneoplasique est associe a la maladie de Castleman. Nous rapportons un cas de pemphigus paraneoplasique survenu a un stade de transformation sarcomateuse d’une maladie de Castleman qui etait presente depuis des annees sans pemphigus paraneoplasique associe. Ce pemphigus s’est manifeste par une atteinte muqueuse buccale et pulmonaire exclusive, tout a fait inhabituelle. Observation Un homme de 32 ans souffrant de vastes ulcerations de la muqueuse buccale etait hospitalise en Decembre 2000 pour alteration de l’etat general et insuffisance respiratoire aigue. La recherche d’anticorps anti-substance intercellulaire sur vessie de rat etait positive, correspondant en immunoempreinte a des IgG anti envoplakine, permettant le diagnostic de pemphigus paraneoplasique. Une radio de thorax et un scanner thoracique montraient une tumeur hilaire dont l’examen histologique permettait le diagnostic de maladie de Castleman associee a une transformation sarcomateuse. Apres traitement chirurgical, l’insuffisance respiratoire s’aggravait. Le malade etait alors tres ameliore par une corticotherapie generale a 2 mg/kg/jour, et une chimiotherapie etait debutee. Il decedait brutalement dans un tableau d’insuffisance respiratoire aigue, trois mois apres la chirurgie. Commentaires Il s’agissait d’un pemphigus paraneoplasique d’expression clinique et biologique particuliere : atteinte muqueuse exclusive avec bronchiolite obliterante expliquee par la presence isolee d’anticorps reconnaissant l’envoplakine, sans antidesmogleine. La transformation de la tumeur de Castleman en sarcome a pu demasquer des antigenes intracellulaires (les plakines), initiant la reaction immunologique specifique.

Resume Introduction Le lymphœdeme de membre resulte d’une incompetence du systeme lymphatique. Qu’il soit primitif ou acquis, la prise en charge est le drainage lymphatique manuel et la contention. L’efficacite de ce traitement varie beaucoup selon les malades, sans caractere previsible. Buts de l’etude Chercher des criteres cliniques ou paracliniques permettant de prevoir la reponse au traitement physique combine (drainage lymphatique et bandage multicouches) pratique en hospitalisation. Malades et methodes L’etude retrospective a porte sur 45 malades, tous hospitalises une semaine dans l’unite de lymphologie du service de Dermatologie du CHU de Tours pour traitement intensif d’un lymphœdeme de membre. Tous ces malades avaient eu une lymphoscintigraphie du membre lymphœdemateux en debut d’hospitalisation. Pour evaluer l’efficacite des drainages, nous avons etudie la perte de volume du membre atteint. Resultats Deux criteres se sont averes predictifs d’une resistance au traitement combine : l’existence d’une insuffisance veineuse et une activite ganglionnaire a la lymphoscintigraphie a 4 heures. Ces deux criteres etaient lies entre eux de facon tres significative. Conclusion L’insuffisance veineuse semble etre un facteur de resistance au traitement physique combine. Pour optimiser les resultats de ce traitement, il convient donc de la depister et de la traiter, en particulier chez tous les patients ayant une activite ganglionnaire sur les cliches tardifs de lymphoscintigraphie.

Paraneoplastic pemphigus is associated with Castleman's disease. We report a case of paraneoplastic pemphigus at the stage of the sarcomatous transformation of Castleman's disease, present for many years but without concomitant paraneoplastic pemphigus. The pemphigus was manifested by the most unusual, exclusive, involvement of the mucosa of the mouth and lung.A 32 year-old man suffering from extensive ulceration of the oral mucosa was hospitalized in December 2000 for alteration in his general status of health and acute respiratory failure. The search for intercellular anti-substance antibodies on rat spleen was positive, corresponding to anti-envoplakin IgG and leading to the diagnosis of paraneoplastic pemphigus. The thoracic x-ray and scan revealed a hilum tumor, the histological examination of which confirmed the diagnosis of Castleman's disease concomitant to sarcomatous transformation. Following surgical treatment, the respiratory failure worsened. The patient improved with systemic corticosteroids at the dose of 2 mg/kg/d and chemotherapy was initiated. The patient died suddenly within the context of acute respiratory failure, three months after surgery.This is a case of paraneoplastic pemphigus of unusual clinical and biological expression: exclusively mucosal involvement with obliterating bronchiolitis, explained by the isolated presence of antibodies recognizing envoplakin, without anti-desmoglein. The transformation of the Castleman tumor into a sarcoma may have unmasked intra-cellular antigens (plakins), initiating the specific immune reaction.

Limb lymphedema results from incompetence of the lymphatic system, and treatment of both primary and secondary forms involves manual lymphatic drainage and support. The effectiveness of treatment varies from patient to patient and can be unpredictable.To investigate clinical and paraclinical criteria able to predict responses to combined physical treatment (lymphatic drainage and multilayered support) during hospitalization.A retrospective study was performed in 45 patients admitted for one week's intensive treatment of limb lymphedema in the Lymphology Unit of the Department of Dermatology of the University Hospital of Tours. Lymphoscintigraphy was performed for all patients on admission. Loss of volume in affected limbs was studied to evaluate the effectiveness of treatment.Two criteria were predictive of resistance to combined treatment, i.e. venous insufficiency and continuing lymph node evidence of scintigraphic activity four hours after lymphoscintigraphy. The two factors were significantly related.Failure of combined physical treatment for lymphedema appears to be related to venous insufficiency. It is therefore essential to investigate and treat venous insufficiency, particularly in patients with continuing evidence of scintigraphic activity in lymph nodes.

The objectives of this work were a) to assess the feasibility of lymphoscintigraphy in very young children, and b) to assess its usefulness in understanding the underlying pathophysiology.Forty-one children (age range 3 months-16 years) with primary lymphedema of the lower and/or upper limbs underwent lymphosinctigraphy using 99mTC-labeled colloid (Nanocis or Nanocol) injected subcutaneous at the foot and/or hand level. Recording time, which depended on the child's age, included at least static images during the thirty minutes following injection and four hours later.Abnormal activity of the lymph collectors, nodes, and interstitial space were analyzed according to age (or2 years). Abnormal findings were classed as simple hypoplasia (n=18) or other type of lymph disease: diffuse lymphatic dysplasia (n=6), mixed vascular dysmorphism (n=5), amniotic bride disease (n=1), and hypoplasia revealed by occurrence of a minor factor (n=11). These observations demonstrate the role of lymphoscintigraphy in the diagnosis and prognostic evaluation of lymphedema in children.Lymphoscintigraphy is a useful technique for very young children with lymphedema providing further information contributing to overall exploration.

The association of manifestations in the mouth and Vitamin B12 deficiency is already known. The signs are not specific to Vitamin B12 deficiency, however they may reveal the deficiency and this is often ignored and leads to delays in diagnosis. We report two cases of Vitamin B12 deficiency revealed by stomatodynia, glossitis and erosions in the mouth.Two women aged 51 and 76 years consulted, one for atrophic glossitis and the other for stomatodynia that had progressed over the past few months. No other symptoms were present. The biological examinations revealed isolated macrocytosis without anemia. Vitamin B12 could not be measured in the serum. The symptoms regressed in less than one month following replacement therapy.As with our two patients, the majority of cases of Vitamin B12 deficiency reported in the literature were only discovered several months or years after their onset, or were treated for other causes. Vitamin B12 deficiency leads to severe neuropathies. The neurological damage is reversible when replacement therapy is initiated early. Since the oral signs appear before the modification in the systemic markers of deficiency, it is crucial that these signs be recognized and diagnosed before the occurrence of severe after effects.

Erysipelas and lymphangitis are frequent complications of lymphedemas (20 to 30%). The most important risk factor for erysipelas is lymphedema since this is a protein rich edema that contributes to the risk of infection. In case of lymphedema the treatment is the usual consensus treatment for erysipelas. A prophylactic treatment with penicillin is requested as soon as the first recurrence. This prophylactic treatment includes skin care, particularly treatment of injuries and intertrigos. Hyperplastic skin leads to maceration and then mycoses. Physiotherapy does not increase the risk for infection. Moreover an infection needs a complex decongestive physiotherapy which decreases risks of recurrence.

Radiodermatitis after cardiac catheterization procedures has been reported in only a few cases in the literature. This side effect may not however be uncommon after long or repeated endocavitary explorations.We observed a case of chronic radiodermatitis which developed after cardiac catheterization, in a patient treated with corticosteroids and ciprofibrate for lupus. The patient had undergone only one heart catheterization. A second catheterization performed when the patient had stopped ciprofibrate did not provoke new lesions.This observations would suggest that ciprofibrate could participate as a radiosensitizing factor in the development of radiodermatitis.