[Mycosis fungoides in a heart transplant recipient]. / Mycosis fongoïde après transplantation cardiaque.

BACKGROUND: Skin cancer occurs frequently in organ transplant patients as a result of induced immunosuppression. Most cases involve carcinomas or B-cell lymphomas induced by the Epstein Barr virus (EBV). Cutaneous T-cell lymphomas remain rare. We report a case of cutaneous T-cell lymphoma of the mycosis fungoides type in a heart transplant recipient. PATIENTS AND METHODS: A 68-year-old man who had received a heart transplant 21years earlier and was being treated with tacrolimus, mycophenolate mofetil and prednisolone had been presenting a psoriasiform rash on his trunk, limbs and head for 4years. The rash was resistant to both PUVA therapy and topical corticosteroids. Histopathological examination suggested epidermotropic cutaneous T-cell lymphoma. There was no impairment of the patient's general state of health nor any adenopathy. Molecular biology revealed TCR rearrangement in both blood and skin. Screening for circulating Sézary cells was negative, and PET scan revealed no signs of extracutaneous localization. Mechlorethamine showed little efficacy, bexarotene was complicated by dysthyroidism, hypertriglyceridemia was ineffective, methotrexate was contraindicated because of calcineurin inhibitor-related chronic kidney failure, and interferon could not be given due to the context of heart transplantation. Finally, we treated our patient with gemcitabine, which initially proved effective but was later complicated by septic shock that resulted in the patient's death. CONCLUSION: The particularities of our observation are the onset of cutaneous T-cell lymphoma of the mycosis fungoides type in a heart transplant patient, and the therapeutic difficulties encountered in a setting of transplantation with immunodepression.

[Syphilitic osteitis in an HIV-negative patient]. / Ostéite syphilitique chez un patient séronégatif pour le VIH.

BACKGROUND: Secondary syphilis with skeletal involvement is rare; herein we report a case concerning an HIV-negative patient. PATIENTS AND METHODS: During the course of secondary syphilis, a 28-year-old male homosexual, HIV-negative and with no medical history, presented intense and localized headaches persisting despite three weeks of antibiotic therapy. Bone scintigraphy revealed three bone lesions evocative of syphilitic osteitis, for which prolonged antibiotic therapy was instituted. DISCUSSION: Few cases of syphilitic osteitis have been described in the recent literature and these are linked to haematogenous diffusion of Treponema pallidum. Skeletal disease is suggested when febrile bone pain is present. Bone scintigraphy remains the best diagnostic tool and treatment comprises prolonged penicillin G or ceftriaxone.

[Annular lipoatrophy of the ankles treated by fat grafting: Case report and review of the literature]. / Lipoatrophie annulaire des chevilles traitée par autogreffe de tissu graisseux : à propos d'un cas et revue de la littérature.

INTRODUCTION: Annular lipoatrophy of the ankles is a rare disease. Eleven cases are described in the literature. CASE REPORT: We report the case of a 10-year-old girl having an annular lipoatrophy of the ankles. The clinical history begins with the appearance of inflammatory infiltrated nodules at the two legs, which have evolved in a few months to a circumferential lipoatrophy of the ankles. Laboratory studies showed a very high antistreptolysin O titer, concluding streptococcal origin of this hypodermitis. After two years of stable lesions, the patient received two sessions of fat injection. RESULT/DISCUSSION: A satisfactory outcome of the adipocyte graft was observed with reconstitution of shapely legs, stable over time. Eleven cases described in the literature are found. It is a pediatric pathology seen predominantly in female children. The evolution towards lipoatrophy is systematic with or without treatment initiated at the inflammatory phase. We first discuss the management of aesthetic sequelae of this disease. CONCLUSION: Fat grafting appears to be a good indication for the treatment of the cosmetic sequelae seen in annular lipoatrophy of the ankles.

[Drug-induced Sweet's syndrome related to hydroxychloroquine: About 2 cases]. / Syndrome de Sweet médicamenteux à l'hydroxychloroquine : à propos de 2cas.

INTRODUCTION: Hydroxychloroquine is widely prescribed in systemic lupus erythematosus. Dermatologic adverse drug reactions are rare but can mimic a disease specific manifestation of lupus. Exceptionally, Sweet's syndrome, or acute febrile neutrophilic dermatosis, may be drug-induced. CASE REPORTS: Two patients aged 31 and 42 years were treated with hydroxychloroquine for systemic lupus and Sjogren's syndrome, respectively. Three weeks after starting treatment, they had a febrile, purple and erythematous papular rash of the trunk and limbs. There was a biological inflammatory syndrome and skin biopsy disclosed an infiltrate of the dermis rich in neutrophils. Lesions regressed after stopping hydroxychloroquine and introducing systemic corticosteroid therapy. Allergologic tests discussed the differential diagnosis with a delayed-type hypersensitivity reaction. CONCLUSION: We report two exceptional cases of drug-induced Sweet's syndrome related to hydroxychloroquine treatment in autoimmune rheumatic diseases.

[Extensive purpura and septic schock in two patients with Capnocytophaga canimorsus]. / Purpura extensif au cours d'un choc septique à Capnocytophaga canimorsus: deux observations.

INTRODUCTION: Capnocytophaga canimorsus is a Gram negative bacillus present in oral flora and in saliva of dogs and cats. It can be responsible for septicaemia and meningitides in some patients after dog or cat bite two patient with a septic shock due to C. canimorsus, who presented with an extensive pupura are reported. CASE REPORT: A 50 year-old man with a past history of splenectomy was referred to an emergency department for a shock with vomiting, abdominal pain and generalized ecchymotic purpura culture samples isolated C. canimorsus in peritoneal fluid. Purpura occurred secondary to a disseminated intravascular coagulation. Despite intensive care and major antibiotherapy, the patient rapidly died. Four days previously, the patient administered pills in his dog mouth. The second patient was a 39 year old alcoholic man who was hospitalised for vomiting and septic shock witch occurred 24 hours after a dog bite. He presented with a livido and a diffuse purpura associated with necrotic lesions. Cultures of blood samples isolated C. canimorsus. CONCLUSION: These severe infections due to C. canimorsus should be avoided by the routine use of early antibiotherapy with amoxicilline and clavulanic acid in patients with dog or cat bite, particularly if patient with associated debilitating disorders.

[Severe alveolar haemorrhage related to a silicone embolism syndrome revealed by a chronic penis ulcer: A case report]. / Une hémorragie intra-alvéolaire sévère liée à un syndrome d'embolie siliconée révélée par une ulcération chronique du pénis : une observation.

INTRODUCTION: Silicone injections, in particular illegal injections, carried out in an aesthetic purpose, can cause serious complications, like silicone embolism syndrome. CASE REPORT: We present a 39-year-old man who presented with a severe acute respiratory distress syndrome related to an alveolar hemorrhage associated with a persistent penis ulcer and a genital lymphedema. It was the complications of silicone injections which revealed a severe personality disorder. Diagnosis of silicone embolism syndrome was made, a few years later, thanks to the histopathology study of a persistent penis ulcer with genital lymphedema. The outcome was favorable. CONCLUSION: A serious alveolar hemorrhage in a young patient should raise suspicion of silicone embolism syndrome, especially if there are cutaneous lesions compatible with injections.

[Chronic meningococcemia]. / Méningococcémie chronique.

INTRODUCTION: Chronic meningococcemia is a rare clinical form of invasive Neisseria meningitidis infection. We report 2 cases. OBSERVATIONS: A 39 year-old man and a 42 year-old woman had developed a widespread, fleeting and painful maculopapular cutaneous eruption over the past few weeks, associated with intermittent fever and inflammatory arthralgia. In both cases blood cultures isolated a serogroup B meningococcus that confirmed the diagnosis. Cutaneous histology revealed a non-specific image of leukocytoclastic vasculitis. Treatment with beta lactamin antibiotics was successful after respectively 3 weeks and 12 days. DISCUSSION: This septicemia is characterized by the clinical triad of cutaneous eruption, fever and arthralgia. It must not be mistaken for connectivitis because inappropriate corticosteroid prescription may provoke severe complications. Confirmation of the diagnosis is provided by the blood cultures, which should be repeated. In the case of strong clinical suspicion, the rapid improvement with antibiotics confirms the diagnosis.

[Hypothesis on the mechanism of cnidocyst discharge (author's transl)]. / Hypothèse sur le mécanisme de l'évagination fu filament urticant des cnidocystes.

The geometic aspects of the cnidocystic filament before invagination, during invagination and after the process of evagination are shown. Experiments are described that allow an approach to study the mechanism of discharge. It is suggested here that the filament is an elastic tube which, when it is intracapsular, is folded and spiraled and thus, under a constraining action. Evagination then consists simply in the release of the constraining force and reversion of the filament to the primitive cylindric shape. The presence of water seems indispensable to the process to take place; however this does not necessarily imply that a hydratation take place in the filament wall. It also seems obvious that if expansion is a normal tendency of the constrained filament, eversion is a result of the continuity between the wall of the filament and that of the capsule. In conclusion, the invagination of the tube during cnidogenesis may be interpreted as a mean of storing energy for the evagination.

Reliable measurement of mouse intraocular pressure by a servo-null micropipette system.

PURPOSE: To develop a reliable technique for measuring intraocular pressure (IOP) in the mouse. METHODS: An electrophysiologic approach-the servo-null micropipette system (SNMS)-for measuring hydrostatic pressure was adapted for the mouse eye. Fine-tipped (5 microm in diameter) micropipettes were advanced across the cornea with a piezoelectric micromanipulator, and the IOP was continuously monitored for up to 46 minutes. RESULTS: The micropipette tip was visualized in the anterior chamber. With the SNMS, the IOP of black Swiss outbred mice under ketamine anesthesia was 17.8 +/- 0.4 mm Hg, higher than values previously estimated in inbred mouse strains by a larger bore microneedle manometric technique. After withdrawal of the micropipette, a second penetration led to a similar level of IOP. Hypotonic solutions increased and hypertonic solutions decreased IOP. Drugs that decrease inflow (acetazolamide, timolol) or increase outflow facility (pilocarpine, latanoprost) in primates and humans lowered steady state IOP in the mouse. The transient initial increase in IOP produced by pilocarpine reported in other animals was also observed in the mouse. Xylazine-ketamine anesthesia lowered IOP substantially in comparison with systemic anesthesia with either ketamine or tribromoethanol alone. CONCLUSIONS: The SNMS is the first reliable, reproducible method for measuring mouse IOP. The mouse IOP is sensitive not only to drugs known to reduce aqueous humor inflow but also to drugs that increase aqueous humor outflow facility in the eyes of primates and humans. The development of the SNMS is an enabling step in the use of the mouse for glaucoma research, including molecular genetics, molecular pharmacology, and the search for novel antiglaucoma drugs.

Halogenated inhalation anesthetic agents decrease transepithelial electrical measurements across the isolated iris-ciliary body.

Transmembrane electrical measurements were performed on the isolated rabbit iris-ciliary body (I-CB) to study the direct effects of halogenated inhalation anesthetic agents on the ciliary epithelium. Addition of either halothane, enflurane, or isoflurane to the control 95% O2:5% CO2 gas mixture resulted in a dose-dependent decrease in the short-circuit current (SCC) and potential difference (PD). This response was reversible after the anesthetic gas was discontinued. Pretreatment with either alpha-adrenergic or beta-adrenergic antagonists (phentolamine or timolol) had no effect on the halothane-induced decrease in SCC. Delivery of the analgestic gas N2O did not alter baseline electrical measurements across the isolated I-CB.

Effects of HCO3- on cell composition of rabbit ciliary epithelium: a new model for aqueous humor secretion.

PURPOSE: To determine whether the Na+-K+-2Cl- symport or the parallel Na+/H+ and Cl-/HCO3- antiports provide the dominant pathway for NaCl uptake into the ciliary epithelium. Both pathways are known to support NaCl entry from the stroma into the pigmented ciliary epithelial (PE) cells, after which Na+ and Cl- diffuse across the gap junctions into the nonpigmented ciliary epithelial (NPE) cells and are released into the aqueous humor. METHODS: Rabbit iris ciliary bodies were preincubated in HCO3-/CO2-containing or HCO3-/CO2-free solutions before quick freezing, cryosectioning, dehydration, and electron probe x-ray microanalysis. RESULTS: The NPE and the PE cells contained more K and Cl when incubated with bicarbonate. Inhibition of carbonic anhydrase with 0.5 mM acetazolamide had little effect in HCO3--free medium but prevented the increase in Cl in both cell types in HCO3-/CO2 solution. Inhibition of the Na+-K+-2Cl- symport with 10 to 500 microM bumetanide caused Cl loss from both cell types in HCO3--free solution, but bumetanide produced a paradoxical increase in Cl and Na in HCO3-/CO2 solution. Together, acetazolamide and bumetanide resulted in significant Cl loss in HCO3--free solution and prevented the gains of Cl and Na in HCO3-/CO2 solution. CONCLUSIONS: The present results indicate that the dominant entry pathway of NaCl from the stroma into the ciliary epithelial syncytium is through an acetazolamide-inhibitable Cl-/HCO3 and a parallel Na+/H+ antiport. The dominant release pathways into the aqueous humor appear to be a Na+-K+-2Cl-symport, which can be outwardly directed under physiological conditions, together with the Na+/K+-exchange pumps and Cl- channels.

Optic disc topography in patients with low-tension and primary open angle glaucoma.

Computerized optic disc topographic measurements (cup-disc ratio, neuroretinal rim area, disc volume) and automated visual field parameters (total field loss, mean sensitivity) were similar in 48 eyes with primary open angle glaucoma and 27 eyes with low-tension glaucoma. In open angle glaucoma, the neuroretinal rim area was negatively correlated with the cup-disc ratio and the disc volume. Total field loss was positively correlated with the cup-disc ratio and the disc volume and negatively correlated with the neuroretinal rim area. Eyes with low-tension glaucoma showed a negative correlation between neuroretinal rim area and cup-disc ratio, and between total field loss and rim area. However, low-tension glaucomatous eyes did not have significant correlations between disc volume and rim area and between total field loss and cup-disc ratio or disc volume. Cupping in low-tension glaucoma was broadly sloping, resulting in less disc volume alteration.

Effect of bicarbonate on intracellular potential of rabbit ciliary epithelium.

Extracellular HCO3- hyperpolarizes the intracellular potential and makes the aqueous medium negative with respect to the stromal surface of the rabbit ciliary epithelial syncytium. The bases for these observations have been unclear. We have been studying the bicarbonate-induced hyperpolarization (BIH) with sustained intracellular recordings for periods as long as 1-2 hrs. The BIH was observed [6.0 +/- 0.4 mV (mean +/- SE, N = 22)] even when the external pH was clamped constant by appropriately changing the CO2 tension. External HCO3- was required since aeration with CO2 at low external pH did not replicate the BIH. DIDS [4,4'-diisothiocyano-2,2'-disulfonic acid] did not abolish the effect. The hyperpolarization is unlikely to reflect the pH dependence of K+ channels alone, since the effect was not reduced by either 2 mM Ba2+ alone or 2 mM Ba2+ together with 50-100 microM quinidine. The BIH depends directly or indirectly on external Na+, since the sign of the polarization response was reversed either by replacing Na+ with N-methyl-D-glucamine or by blocking the Na+,K(+)-exchange pump with 50-100 microM ouabain. Replacement of external Cl- with NO3- or application of the Cl(-)channel blocker NPPB [5-nitro-2-(3-phenylpropylamino)-benzoate] depolarized the membrane and reversed the sign of the BIH. The response of the ciliary epithelium to HCO3- is complex and may arise from several mechanisms. We suggest that one important element is an anion channel whose conductance is reduced by bicarbonate and whose reversal potential is indirectly dependent on the operations of the Na+,K(+)-pump and a Cl(-)-linked symport.

Cell-attached patch clamping of the intact rabbit ciliary epithelium.

Following thorough removal of adhering aqueous humor, we have succeeded in patch clamping the intact rabbit ciliary epithelium in the cell-attached and inside-out excised-patch modes. Rapidly fluctuating currents ("chatter activity') were observed during recordings conducted for as long as 1 h. Chatter activity did not reflect seal instability since interconversion was noted between chatter activity and transitions between stable open and closed states, excision of patches into the bath was associated with substantial shifts in the reversal potential, and chatter activity could be triggered by sustained hyperpolarization, but was insensitive to stretch. The chatter channel was identified as cation-nonselective from the reversal potentials both in the cell-attached and excised-patch modes. The channel's kinetics were similar to those of the cGMP-activated phototransduction channel. The results of PCR amplifications of fragments in cDNA libraries from both human ciliary body and human nonpigmented ciliary epithelial (NPE) cells indicated that human ciliary epithelial cells transcribe message for the retinal phototransduction channel. The possible role of the phototransduction channel in expressing chatter activity was further explored by perfusing preparations with a known activator of that channel (cGMP) and with a known inhibitor (L-cis-diltiazem). Neither agent significantly affected chatter behavior. We conclude that: (1) this is the first demonstration of the feasibility of patch-clamping the intact ciliary epithelium; (2) the NPE cells display chatter activity arising from rapidly fluctuating transitions of a cation-nonselective channel; (3) NPE cells can transcribe message for the cation-nonselective phototransduction channel; and (4) if the observed chatter activity is from a homologue of the photo-transduction channel, the homologue is pharmacologically distinct.

Novel cnidocysts of narcomedusae and a medusivorous ctenophore, and confirmation of kleptocnidism.

Cnidocysts have been examined from the tentacles of the ctenophore Haeckelia rubra (Euchlora rubra) and five species of hydrozoan narcomedusae (Solmundella bitentaculata, Aegina citrea, Solmissus marshalli, Solmissus albescens, and Cunina sp.) using TEM, both in sections and by firing whole cnidocysts onto EM grids. The study revealed that these apotrichous isorhiza cnidocysts have a novel morphology in which the intracapsular inverted tubule has five circumferential pleats when viewed in transverse section, rather than the usual three pleats. Accordingly, the definition of helicoptychoneme cnidocysts has been broadened to include both the usual three-pleated cnidocysts and these new five-pleated cnidocysts. In general, apotrichous isorhizas have subspherical capsules with a thick, bilayered wall, whose interior is nearly filled with the regularly coiled, helically folded, five-pleated inverted tubule. Upon discharge, the everted tubule is several mm long and the five circumferential pleats become manifested as five helical rows of spines running up the tubule, which has three morphologically different segments. The very short basal segment is devoid of ornamentation; the remaining proximal portion is characterized by five spirals of uniform, closely packed short spines; the long distal portion is characterized by a single spiral of regularly spaced large spines that derive from all five spirals-the five spirals are otherwise demarcated in the distal portion by 'scales' that are visible only with the electron microscope.

[Colonic angiodysplasia. Rare cause of low digestive hemorrhage in young patients]. / Angiodysplasie colique. Cause rare d'hémorragie digestive basse du sujet jeune.

We report the following observation of a diffuse kind of colic angiodysplasia in a young subject. Usually to be find in old subjects and regarded as been degenerative origine, angiodysplasiae have been reported in young subjects and we are led to ponder over their nature. The contribution of diagnostic and therapeutic means and their respected place have been specified. In particular the significance of vascular radiology and the angiographic signs of this arterioveinous malformation have been developed.

[Eosinophilic gastroenteritis: an example of gastrointestinal toxicity of eosinophils]. / La gastroenterite a eosinophiles: un exemple de la toxicite digestive de l'eosinophile.

Toxicity of polynuclear eosinophils in the digestive tract results from a cascade of immune responses involving various mediators including mastocytes and T-lymphocyte helpers. Polynuclear eosinophils may be implicated in many digestive disorders. This involvement is well established in eosinophilic gastroenteritis which has become the model for study of eosinophil toxicity on the digestive tract. Considered to be of uncertain etiology up until 1998, this mechanism is well illustrated by the case described in this report involving infiltration of all layers of the small intestinal mucosa in a 23-year-old patients who responded well to corticosteroid treatment. This case provides the opportunity to update physiopathologic and therapeutic data concerning this unpredictable syndrome. Current evidence of transition forms from idiopathic hypereosinophilic syndrome casts doubt on the conventional classification of eosinophilic gastroenteritis and underlines the need for careful diagnostic work-up and prolonged follow-up.

[Acute infectious diarrhea: current treatment and perspectives]. / Diarrhées aiguës infectieuses: traitement actuel et perspectives.

Acute infectious diarrhea is a worldwide public health problem. In developing countries it remains a major cause infant mortality despite therapeutic progress, especially with regard to the efficacy of oral rehydration, during the last two decades. Mortality in industrialized countries is much lower and generally stable. In most cases, acute diarrhea is a self-limiting illness that resolves with or without symptomatic treatment in a few days. In view of the high frequency and generally benign course of diarrheal diseases, management must emphasize a cost-effective approach with selective use of available diagnostic methods. The initial approach should be based on the patient's history, physical examination, and risk factors to identify infections that require more specific measures. The purpose of this report is to review currently available drug therapies and to describe treatment guidelines for adolescents and adults with uncompromised immune systems. Early rehydration is still the mainstay of treatment. Use of "etiological" treatment using antimicrobial or antiparasitic agents depends on the severity of disease and risk factors.

[Chronic tropical pancreatitis: a case report]. / La pancréatite chronique tropicale: à propos d'un cas.

The purpose of this report is to describe a case of tropical calcific pancreatitis (TCP). This disease is specific to tropical regions and constitutes the main cause of chronic pancreatitis in children worldwide. It can also be observed in young adults (2nd and 3rd decade). Shortage of dietary lipids during childhood has been implicated in the development of TCP and mutation of the SPINK1 gene has been cited as a predisposing genetic factor. The underlying pathophysiology of TCP is the same as chronic calcific pancreatitis (CCP) due to alcohol abuse. The main features are a sex ratio of 1, absence of alcohol consumption, occurrence of childhood diabetes in one third of cases, low incidence of acidoketosis, and presence of macro-calcifications especially in ducts. In 10% of cases TCP is complicated by pancreatic carcinoma occurring at an early age, located mainly in the body and tail of the pancreas, and having a less favorable prognosis than primary cancer. Treatment of patients with TCP is the same as for patients with CCP due to alcohol abuse. Prevention depends on improvement of nutritional status of the population.

[Hepatitis C in tropical areas]. / L'hépatite C sous les tropiques.

The tropical features of hepatitis C have not yet been fully elucidated due to the scarcity of data. However it has been estimated that two-thirds of the infected population lives in tropics. The most heavily affected regions are Africa, China, and southeast Asia with prevalence rates of 5.3, 3.0, and 2.4 p. 100 respectively. In several countries mostly in Africa, prevalence rates range from 5 to 10 p. 100 or higher. Age is a major risk factor for infection. The classic transmission modes, i.e., blood transfusion and intravenous drug use, do not account for these high prevalence rates. Another mechanism could be parenteral injection under unsafe conditions. The most widespread genotypes in tropical areas are genotypes 1, 2, and 3. Other genotypes can be encountered locally including genotype 4 in black Africa and Egypt, genotype 6 in southeast Asia, and genotypes 1 and 3 in India. The association of hepatitis C with chronic liver disease has been the focus of several studies, mainly in Africa. The seroprevalence of virus C ranges from 2 to 55 p. 100 in cases of chronic hepatitis or cirrhosis and from 0 to 47 p. 100 in cases of hepatocellular cancer. Hepatitis C could be the underlying cause of 33 to 50 p. 100 of chronic liver diseases not linked to virus B. It is observed more often in patients with chronic hepatitis or cirrhosis than cancer in which virus B is dominant. Infection by both virus is rare without liver disease but is more frequent in patients with cancer than chronic non-tumoral liver disease.