[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.

[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.

[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.

[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.

[Bullous mastocytosis in infancy: a rare presentation]. / Mastocytose bulleuse diffuse du nourrisson : une forme clinique rare.

Mastocytosis is a rare condition related to an abnormal proliferation of mast cells and their accumulation in tissues. Cutaneous mastocytosis is the most common form and mainly affects newborns and infants. The symptoms are caused by the release of mediators contained in mast cells, including histamine. Mastocytosis may be associated with a mutation in the gene encoding the c-kit receptor. Clinically, there are different dermatological findings, which combine acute cutaneous, digestive, or even hemodynamic manifestations in varying degrees. The diagnosis is confirmed by the histological study of a skin sample. We report here the case of a 4-month-old infant suffering from diffuse cutaneous bullous mastocytosis, a very rare variety of mastocytosis. This infant had an erosive and bullous manifestation of dermatosis, initially confused with impetigo. The proliferation of bullous lesions led to her hospitalization. Codeine intake for pain was responsible for a large and extensive bullous reaction associated with anaphylactic shock. This context of bullous spread occurring after taking codeine led to the suspicion of bullous diffuse cutaneous mastocytosis, a diagnosis that was confirmed histologically. This observation demonstrates the difficulty of mastocytosis diagnosis, mostly due to its rarity, especially in its diffuse bullous forms. The rapid deterioration of this patient, after the codeine prescription, emphasizes the importance of the eviction of histamine-releaser compounds in the management of this disease.

[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.

[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.

[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.

Timolol may inhibit aqueous humor secretion by cAMP-independent action on ciliary epithelial cells.

The beta-adrenergic antagonist timolol reduces ciliary epithelial secretion in glaucomatous patients. Whether inhibition is mediated by reducing cAMP is unknown. Elemental composition of rabbit ciliary epithelium was studied by electron probe X-ray microanalysis. Volume of cultured bovine pigmented ciliary epithelial (PE) cells was measured by electronic cell sizing; Ca(2+) activity and pH were monitored with fura 2 and 2',7'-bis(2-carboxyethyl)-5(6)-carboxyfluorescein, respectively. Timolol (10 microM) produced similar K and Cl losses from ciliary epithelia in HCO/CO(2) solution but had no effect in HCO/CO(2)-free solution or in HCO/CO(2) solution containing the carbonic anhydrase inhibitor acetazolamide. Inhibition of Na(+)/H(+) exchange by dimethylamiloride in HCO/CO(2) solution reduced Cl and K comparably to timolol. cAMP did not reverse timolol's effects. Timolol (100 nM, 10 microM) and levobunolol (10 microM) produced cAMP-independent inhibition of the regulatory volume increase (RVI) in PE cells and increased intracellular Ca(2+) and pH. Increasing Ca(2+) with ionomycin also blocked the RVI. The results document a previously unrecognized cAMP-independent transport effect of timolol. Inhibition of Cl(-)/HCO exchange may mediate timolol's inhibition of aqueous humor formation.

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.

[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.

Origin of germ cells, sex determination, and sex inversion in medusae of the genus Clytia (Hydrozoa, leptomedusae): the influence of temperature.

In Cnidaria, a separation between soma and germline remains unclear. In this work, we studied the origin of germinal cells and determination of the sexual phenotype in Clytia hemisphaerica and Clytia sp. Colonies of C. Hemisphaerica were cultivated and the medusae liberated by each colony raised until maturity. Two hermaphrodite colonies were obtained, liberating male and female medusae. These two colonies and their medusae were raised at 15 degrees C, 21 degrees C, or 24 degrees C. The medusae budded and cultured at 24 degrees C were mainly female (80%). In contrast, if the medusae were released at 15 degrees C, at whatever temperature they were raised later, they were mainly male (85%). The same occurred if, after release at 24 degrees C but before the formation of the gametes, they were kept at 15 degrees C for at least 24 hr. We suggest that there are two subpopulations of germ cells. The female line will be dominant at 24 degrees C but temperature sensitive, with inhibition of this line by a temperature drop to 15 degrees C, this inverting the population sex-ratio. The irreversible action of a temperature drop to 15 degrees C supports the view that the germ cells are isolated very early. In C. hemisphaerica, hermaphrodite medusae were never observed. On the contrary, in Clytia sp., probably a new species, we have found male, female, but also hermaphrodite specimens. This is the second definite example of hermaphroditism described in any hydromedusan. The transformation of female into hermaphrodite then into male specimens occurs at 13 degrees C. These results demonstrate the unstable character of genetic sex determination in cnidarians, at least in certain species.

Similarity of A(3)-adenosine and swelling-activated Cl(-) channels in nonpigmented ciliary epithelial cells.

Chloride release from nonpigmented ciliary epithelial (NPE) cells is a final step in forming aqueous humor, and adenosine stimulates Cl(-) transport by these cells. Whole cell patch clamping of cultured human NPE cells indicated that the A(3)-selective agonist 1-deoxy-1-(6-[([3-iodophenyl]methyl)amino]-9H-purin-9-yl)-N-methyl-be ta-D-ribofuranuronamide (IB-MECA) stimulated currents (I(IB-MECA)) by approximately 90% at +80 mV. Partial replacement of external Cl(-) with aspartate reduced outward currents and shifted the reversal potential (V(rev)) from -23 +/- 2 mV to -0.0 +/- 0.7 mV. Nitrate substitution had little effect. Perfusion with the Cl(-) channel blockers 5-nitro-2-(3-phenylpropylamino)benzoic acid (NPPB) and niflumic acid inhibited the currents. Partial Cl(-) replacement with aspartate and NO(3)(-), and perfusion with NPPB, had similar effects on the swelling-activated whole cell currents (I(Swell)). Partial cyclamate substitution for external Cl(-) inhibited inward and outward currents of both I(IB-MECA) and I(Swell). Both sets of currents also showed outward rectification and inactivation at large depolarizing potentials. The results are consistent with the concept that A(3)-subtype adenosine agonists and swelling activate a common population of Cl(-) channels.

Formation of the aqueous humor.

1. Glaucoma is a worldwide disease affecting approximately 1-2% of the population aged over 35 years in industrial countries and is a major cause of blindness. 2. Glaucoma is usually associated with an increased intraocular pressure reflecting an imbalance between the rate of production of fluid (the aqueous humor) by the ciliary epithelial cells and its drainage from the eye. Therefore, it is important to understand how this secretion is produced. This requires a knowledge of ciliary epithelial cell composition, which has, in the past, proved difficult to obtain in mammalian preparations. 3. We have recently used the technique of electron-probe X-ray microanalysis to determine this composition under a variety of in vitro conditions. 4. Our results have led to a new model for this secretion that emphasizes the potential secretory role of the Na+/K+/2Cl- cotransporter.