Search PubMed⌕ Search

Biomedical subjects

J L Verret

Publications and source records attributed to J L Verret.

At least 19 recordsLinked to original sources

[Cold-induced urticaria].

Cold urticaria is characterized by the development of urticaria, usually superficial and/or angioedematous reaction after cold contact. It was found predominantly in young women. The diagnosis is based on the history and ice cube test. Patients with a negative ice cube test may have represented systemic cold urticaria (atypical acquired cold urticaria) induced by general body cooling. The pathogenesis is poorly understood. Cold urticaria can be classified into acquired and familial disorders, with an autosomal dominant inheritance. Idiopathic cold urticaria is most common type but the research of a cryopathy is necessary. Therapy is often difficult. It is essential that the patient be warned of the dangers of swimming in cold water because systemic hypotension can occur. H1 antihistamines can be used for treatment of cold urticaria but the clinical responses are highly variable. The combination with an H2 antagonists is more effective. Doxepin may be useful in the treatment. Leukotriene receptor antagonists may be a novel, promising drug entity. In patients who do not respond to previous treatments, induction of cold tolerance may be tried.

Acetates↗

Mutations in CGI-58, the gene encoding a new protein of the esterase/lipase/thioesterase subfamily, in Chanarin-Dorfman syndrome.

Chanarin-Dorfman syndrome (CDS) is a rare autosomal recessive form of nonbullous congenital ichthyosiform erythroderma (NCIE) that is characterized by the presence of intracellular lipid droplets in most tissues. We previously localized a gene for a subset of NCIE to chromosome 3 (designated "the NCIE2 locus"), in six families. Lipid droplets were found in five of these six families, suggesting a diagnosis of CDS. Four additional families selected on the basis of a confirmed diagnosis of CDS also showed linkage to the NCIE2 locus. Linkage-disequilibrium analysis of these families, all from the Mediterranean basin, allowed us to refine the NCIE2 locus to an approximately 1.3-Mb region. Candidate genes from the interval were screened, and eight distinct mutations in the recently identified CGI-58 gene were found in 13 patients from these nine families. The spectrum of gene variants included insertion, deletion, splice-site, and point mutations. The CGI-58 protein belongs to a large family of proteins characterized by an alpha/beta hydrolase fold. CGI-58 contains three sequence motifs that correspond to a catalytic triad found in the esterase/lipase/thioesterase subfamily. Interestingly, CGI-58 differs from other members of the esterase/lipase/thioesterase subfamily in that its putative catalytic triad contains an asparagine in place of the usual serine residue.

1-Acylglycerol-3-Phosphate O-Acyltransferase↗

[Chronic arsenicism].

BACKGROUND: Arsenic is an ubiquitous natural element. Chronic and low level ingestion or inhalation may result in chronic arsenicism first characterized by skin changes. CASE REPORT: A 75 year old man, non-insulin-dependent diabetic, presented a diffuse hyperpigmentation with scattered white spots on the trunk. He complained of asthenia. Clinical diagnosis of chronic arsenicism was confirmed by arsenic determination in urine, plasma and phaneres. Thorough investigations led to discover very high arsenic levels in the own wine of the patient. This was probably the result of a wrong use of sodium arsenite-based fungicide, for cultivating his vine yard. DISCUSSION: Chronic arsenicism has become rare but it should always be kept in mind. Clinical presentation, with particular cutaneous features and routes of exposure are reviewed. Treatment is symptomatic. As arsenic is known to be a strong carcinogenic agent, patients with chronic arsenicism have to be followed up during a long time.

Aged↗

[Effectiveness and dangers of interferon-alpha in the treatment of severe hemangiomas in infants].

UNLABELLED: Alarming hemangiomas, due to their site or repercussions, require pharmacological treatment. Corticosteroid therapy is indicated by first intention. In the event of failure, interferon alpha is proposed. CASE REPORTS: Case 1. A five-week-old infant was admitted to hospital for an extensive hemangioma of the left side of the face and neck with necrosis of the upper lip and ear. Prednisolone (2 mg/kg/day) by intravenous route brought about no improvement. Interferon alpha 2a (3 MU/m2/day of Referon by subcutaneous injection) enabled regression of lesions from the sixth month of treatment. After 11 months of treatment, the hemangioma had all but disappeared and interferon therapy was stopped. Repair surgery was planned at 24 months of age. Case 2. A one-month-old infant suffered from a hemangioma of the right side of the face with orbital invasion and risk of amblyopia. Prednisone (2 mg/kg/day) by oral route was ineffective. Interferon alpha 2a enabled regression of the hemangioma and the eye opened from the third month of treatment. Interferon therapy was stopped after 14 months. Initial repair surgery intervention was possible at two years of age. Spastic paraplegia was diagnosed at 18 months of age. The brain and medullar magnetic resonance imaging was normal. No etiology could explain the neurological attack. The possible toxic effect of interferon alpha is discussed. CONCLUSION: Interferon alpha is an effective treatment for hemangiomas. It significantly reduces spontaneous regression time. The uncertainty of long-term effects in infants with hemangiomas incites its indication to be limited to alarming corticosteroid-resistant forms and necessitates prolonged neurological surveillance.

Amblyopia↗

Allergic contact dermatitis from hydrangea--is it so rare?

8 cases of allergic contact dermatitis from hydrangea seen in Angers, France, during the last 15 years are reported and compared to other cases found in the literature. In this review, allergic contact dermatitis from hydrangeas appears to be an occupational dermatosis among nursery workers, presenting as an eczema involving the hands and especially the first 3 fingers, with chronic features of fissuring and scaling and with a chronic course. Differential diagnosis from irritant contact dermatitis may be difficult. Patch tests with the stem as well as the leaf of hydrangeas gave strong positive reactions in all patients, and hydrangenol, the allergen of hydrangea, when tested, always also gave a positive reaction. Sensitization seems to occur after close and prolonged contact with the plant, which could explain the relative frequency in Angers because it provides almost 90% of hydrangea seedling production in France.

Adult↗

[Papular mucinosis associated with myopathy].

BACKGROUND: Papular mucinosis is an uncommon condition sometimes associated with myopathy as in our case. CASE REPORT: A 54-year-old woman presented several disseminated pruriginous papules that spread progressively. Histopathology of a skin biopsy showed mucine deposits and fibroblasts in the derma. Serum monoclonal lambda IgG was evidenced. The patient complained of weakness of the proximal muscles and dysphagia. Serum creatine phosphokinase was moderately elevated and the electromyography showed a typical myogenic pattern. Histopathology of a muscle biopsy disclosed a polymyositis aspect without mucine deposits. The patient was given prednisone. The skin lesions regressed totally with no modification of the muscle deficit. DISCUSSION: Myopathy associated with papulous mucinosis generally presents as a proximal deficit with dysphagia. Myalgia and amyotrophy are exceptional. Moderate serum myolysis is often evidenced. The electromyogram shows a myogenic pattern. The pathophysiology of this condition remains unclear, but the association with papulous mucinosis does not appear to be fortuitous due to its frequency. No standard treatment has been established. General corticosteroid therapy is generally effective and methotrexate, melphalan, isotretinoine and D-penicillamine have given good results in a few cases.

Biopsy↗

An epidemic of occupational contact dermatitis from an immersion oil for microscopy in laboratory personnel.

Since November 1997, 14 successive cases of occupational contact dermatitis were observed in 13 laboratory technicians and 1 physician, working in a genetics laboratory in Leuven (Belgium) in 3 laboratories of bacteriology in Strasbourg, Montbeliard and Angers, and in the laboratory of hematology in Bordeaux (France). The dermatitis, located on the hands, forearms and face, relapsed after each exposure to an immersion oil for microscopy. Patch tests performed in 10 patients were positive to epoxy resin (ER) in the European standard series (10/10 patients) and to newly formulated Leica immersion oil (7/7), 1 patient testing negatively with the former oil. A breakdown performed in 2 patients with the oil's ingredients confirmed sensitization to liquid modified ER components, contained at >80% concentration in the oil. The presence of DGEBA was demonstrated by HPLC analysis at a +/-30% rate. Although the safety data sheet indicated a revision of the formula, nobody was alerted to the risk of sensitization and the need for skin protection. ERs, as a source of occupational allergy, can provoke epidemics of contact dermatitis in industry. This report of epidemic contact dermatitis from ERs, used for their optical properties in an immersion oil for microscopy, emphasizes the need for perpetual vigilance in occupational medicine and the usefulness of multicentre contacts in dermato-allergology.

Adult↗

Necrobiosis lipoidica: treatment by hyperbaric oxygen and local corticosteroids.

Necrobiosis lipoidica (NL) is closely associated with diabetes mellitus. Two-thirds to three-fourths of patients with NL have diabetes, although NL occurs in only 0.3% of diabetic patients. Typical lesions are found on pretibial skin, usually in young female diabetic patients whose disease is inadequately controlled. The cause of this dermopathy remains unknown. Multiple treatments have been described but have not led to consistent results. We report the case of a 28-year-old insulin-dependent diabetic woman with a disease duration of 23 years who spontaneously developed ulcerated NL on pretibial skin. NL progressively improved during 113 sessions of hyperbaric oxygen therapy and local corticosteroids.

Administration, Topical↗

[Dorfman-Chanarin syndrome].

BACKGROUND: Dorfman-Chanarin syndrome is an uncommon condition characterized by non-bullous congenital ichtyosiform erythrodermia and lipid vacuoles in circulating leukocytes. CASE REPORT: We report an unusual presentation in a child who had a dry congenital ichtyosiform erythroderma. Blood smears revealed lipid vacuoles in granulocyte cytoplasm, leading to the diagnosis of Dorfman-Chanarin syndrome. The child also had liver and ophthalmologic involvement. DISCUSSION: Dorfman-Chanarin syndrome is a rare autosomic recessive hereditary disease (27 cases reported in the literature) related to the accumulation of neutral lipids in organ tissues. Clinical manifestations are dry congenital ichtyosiform erythroderma and lipid vacuoles in circulating granulocytes. The syndrome may be expressed more or less severely in several organs. Diagnosis is confirmed on blood smears. The vacuoles can also be observed in smears of heterozygous subjects and can serve as a screening test. The pathogenesis of Dorfman-Chanarin syndrome is poorly understood but appears to be related to perturbed intracellular triglyceride catabolism. Treatment is symptomatic.

Child, Preschool↗

[Granulomatous mycosis fungoides histologically simulating cutaneous sarcoidosis].

BACKGROUND: Granulomatous mycosis fungoïdes is an uncommon mycosis fungoïdes. We report a misleading case initially thought to be cutaneous sarcoidosis. CASE REPORT: A 34-year-old man developed stationary erythematous plaques on the trunk and members. Pathology reported a sarcoidal aspect. No extracutaneous sarcoidal lesions were found. The lesions resolved with puvatherapy. Five years later, the patient developed a voluminous skin tumor in the scapular area. Pathology reported granulomatous mycosis fungoides. Radiotherapy was given. Other plaques developed on the skin with the same histological aspect as initially and disappeared after local applications of chlormethine. During the clinical course, the patient developed visceral localizations of mycosis fungoides in the abdomen and pelvic nodes which responded to polychemotherapy. DISCUSSION: Histologically, granulomatous mycosis fungoïdes is defined as the association of epidermotropic T-cell lymphoma with epitheloid and giant cell dermal granulomas and no necrosis. Variants may be misleading when the epitheloid and giant cell aspect predominates, as in our observation. An analysis of 28 reported cases showed no disinction between the clinical features. Clinical course or treatment for granulomatous fungoides mycosis or classical fungoid mycosis. Extracutaneous sarcoidosis is rarely associated. Most likely, there is a tissue reaction to the lymphona.

Adult↗

[Pustular candidiasis in heroin addicts].

INTRODUCTION: Pustular candidiasis in heroin addicts is a rare entity in dermatology. We report a case. CASE REPORT: A 29-year-old female heroin addict developed a painful pustular growth on the scalp. There was no fever. Multiple follicular pustulae measuring 2 to 3 mm were associated with hyperesthesia of the scalp and painful cervical nodes. Biopsy showed acute ostiofolliculitis with a few blastospores and mycelial filaments. Candida albicans was isolated from the pustulae and the buccal cavity. Candida serology was positive (indirect immunofluorescence 1/100, coelectrosyneresis: 4 archs). Search for other localizations and HIV serology were negative. The last injection of brown heroin had been taken 15 days earlier; lemon had been added. Treatment with flucanazole (400 mg/d) led to improvement within 48 hours. DISCUSSION: Sudden development of pustulae or nodules in pilous zones in a heroin addict should suggest the diagnosis. Outcome depends on early treatment after diagnosis and search for other localizations. Our case presented two particular aspects: ostiofollicular localization of the pustulae and a long delay (15 days) between the (presumably) last injection and the development of the lesion. Folliculitis develops almost exclusively in addicts who use brown heroin. Contamination by Candida albicans results from the lemon used to improve solubility at injection.

Adult↗

[Unilesional plaque-type mycosis fungoides: 3 cases].

BACKGROUND: The clinical course of unilesional plaques with a histological diagnosis of mycosis fungoides is generally benign. This uncommon situation raises the question of nosology among epidermotropic T-cell proliferations. CASE REPORT: Two women (age 65 and 70 years, cases 1 and 2) and one man (age 66 years, case 3) each had a well-limited unique oval-shaped plaque measuring 1 to 3 cm in diameter for several months. The localization of the erythematous, squamous plaques with minimal infiltration and little or no pruritus remained unchanged in the dorsal area. Histology reported mycosis fungoides: dense superficial dermal infiltration in band attached to the epidermis with a clear lower limit, formed by small and medium-sized atypical lymphocytes, sometimes with cerebriform nuclei. Epidermotropism on isolated cells or theques was noted. There was no spongiosis. Immunolabeling demonstrated predominance of CD4+ cells and tumoral infiltration uptake of anti-V beta 8 monoclonal antibodies in the two cases examined. The lesion totally regressed in case 1 after local application of carmustine with no recurrence after 5 years. Patient 2 declined treatment and no change was observed 5 years later. Surgical exeresis was performed in case 3 without recurrence 3 years later. DISCUSSION: No histological feature or T-cell immunophenotype distinguishes this clinical entity from classical mycosis fungoides. As no other long-term lesions developed, our three cases would argue for a benign course of what is probably a variety of mycosis fungoides. Unilesional plaque mycosis fungoides must be lymphomatoid contact dermatitis. Woringer-Kolopp disease differs by its nearly exclusive epidermotropic distribution.

Aged↗

[Schnitzler syndrome and Waldenström disease. Fatal outcome of the original case].

The association between chronic urticaria, macroglobulinaemia and various other manifestations has been individualized as Schnitzler's syndrome. We report the terminal course of an original case followed up for 20 years, which ended as lymphoplasmocytic lymphoma with multiple sites, whereas no lymphomatous proliferation, meticulously looked for, had never been found hitherto. The lymphoma, associated with a macroglobulinaemia level above 5 milligrams, was diagnosed as Waldenström disease. Although most cases of Schnitzler's syndrome seem to follow a benign course (but the follow-up is not always long) a few cases have been reported showing evolution towards, or association with, lymphoma. This indicates that the follow-up of patients with Schnitzler's syndrome should be prolonged.

Aged↗

["Tufted angioma". A benign vascular tumor to differentiate with Kaposi sarcoma].

Among recently characterized vascular tumors, tufted angioma or angioblastoma is a benign acquired slowly progressive cutaneous tumor, which most commonly arises in the neck and upper trunk in children and young adults. This case report emphasizes the clinical and histological features of tufted angioma. Light microscopic examination reveals numerous lobules of closely packed capillaries scattered throughout the dermis. Vascular lumina are difficult to define. There are no atypical cells. Familiarity with tufted angioma should prevent this lesion from being misdiagnosed as malignant vascular tumor arising in young persons, especially Kaposi's sarcoma.

Adolescent↗