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Biomedical subjects

P Combemale

Publications and source records attributed to P Combemale.

At least 19 recordsLinked to original sources

[Auto-inflammatory disease with hyperimmunoglobulinemia D of late onset].

BACKGROUND: The term "auto-inflammatory diseases" encompasses a group of disorders characterised by febrile episodes of sudden onset associated with joint, abdominal, lymph node and cutaneous signs, each presenting a genetic and/or laboratory specificity allowing their identification. Polyclonal elevation of serum IgD is highly suggestive of hyper-IgD syndrome, a disease with autosomal recessive transmission that usually begins before the age of one year. CASE REPORT: We report the case of a 46-year-old woman presenting a disease since the age of 30 years clinically very similar to hyper-IgD syndrome except for ocular and pulmonary involvement. However, tests revealed neither mevalonate kinase gene mutation nor elevation of urinary mevalonic acid. The near-monthly attacks were controlled with mycophenolate mofetil. DISCUSSION: Three cases with identical clinical and laboratory profiles have been reported in the literature and diagnosed as late-onset hyper-IgD syndrome. Our case is the only one involving indirect screening for mutation of the mevalonate kinase gene, which proved negative. This finding rules out the diagnosis of classic hyper-IgD syndrome in our case and raises the possibility of auto-inflammatory disease with reactional hyper-IgD of a different cause, either acquired or secondary to an as yet unidentified mutation.

Age of Onset↗

Cannabis arteritis.

The main causes of arteriopathy in young patients include drugs, metabolic diseases, pseudoxanthoma elasticum and Buerger's disease. Arteritis due to Cannabis indica was first reported in 1960, and the role of this drug as a risk factor for arteritis was confirmed in several subsequent publications. A 38-year-old smoker with no previous contributory medical history except for long-standing cannabis abuse developed a dry necrotic lesion of the left big toe. Imaging examinations revealed proximal arteriopathy of the lower limbs that predominated on the left side. He had no atherogenic or thrombogenic risk factors, and no signs of pseudoxanthoma elasticum were found. Remarkably, the development of arteritis paralleled cannabis abuse. The course was slowly favourable after weaning from the drug, vasodilator treatment and hyperbaric oxygen therapy. Despite some subtle clinical differences (more proximal than distal involvement), cannabis arteritis may be considered as a particular form of Buerger's disease, where cannabis, along with tobacco, seems to cause arterial lesions. Along with the noxious effects of cannabis on vessels, a role for contaminating arsenic is also possible. Cannabis arteritis is not widely known, but may prove not to be so rare if one considers consumption of cannabis besides that of tobacco.

Adult↗

Evaluation of a lipidocolloid wound dressing in the local management of leg ulcers.

OBJECTIVE: To evaluate the efficacy, tolerance and acceptability of Urgotul and DuoDERM E dressings in the local management of venous or mixed-aetiology leg ulcers. METHOD: This was a prospective multicentre randomised phase IV clinical trial conducted open-label in parallel groups. It involved 20 investigating centres, including hospital dermatology and vascular medicine departments, and private practices. Dermatologists and angiologists/phlebologists took part. Subjects were adult, non-immunosuppressed patients presenting with a non-infected, non-malignant leg ulcer of predominantly venous origin (ABPI > 0.8). Ulcers were between 4cm2 and 40cm2 in size, with granulation tissue covering more than 50% of their surface area. Ulcer duration ranged from three to 18 months. Patients were followed-up by the investigating physician for eight weeks on a weekly basis; this included clinical examination, wound area tracings and photographs. Nurses (hospital or visiting) assessed exudate volume and clinical appearance at dressing changes. RESULTS: Ninety-one patients were included: 47 in the Urgotul group and 44 in the DuoDERM E group. Baseline patient demographic data and wound characteristics were comparable in the two groups. After eight weeks of treatment wound surface area had reduced by a mean of 61.3% in the Urgotul group and 52.1% in the DuoDERM E group (NS); dressings were changed more frequently in the DuoDERM E group (2.54 +/- 0.57 times per week versus 2.31 +/- 0.45 in the Urgotul group, p = 0.047). Thirty-three local adverse events were recorded in 27 patients: 10 in the Urgotul group and 23 in the DuoDERM E group (p = 0.039). Nurses reported better acceptability for the Urgotul dressing, based on pain on removal, maceration and odour (p < 0.0001). CONCLUSION: Both dressings showed similar efficacy for the local treatment of venous leg ulcers. Nevertheless, medical and nursing staff reported better tolerance and acceptability for the Urgotul dressing.

Adult↗

Capillary leak syndrome induced by acitretin.

The mucocutaneous side-effects of systemic retinoids are well known. We report a patient with diffuse oedema, which is a very rare side-effect of retinoids. The pathophysiology of this condition is poorly known because of the rarity of relevant observations, but apparently corresponds to capillary leak syndrome. The outcome is invariably favourable following withdrawal of the drug, which should be definitively contraindicated in the patient.

Acitretin↗

[Neurofibromatosis 1: recommendations for management].

Twenty experts, members of a French medical network devoted to neurofibromatosis 1 have elaborated recommendations for the management of the disease. Bibliography was obtained through a Medline of articles from 1966 to 1999 for the terms neurofibromatosis, NF1, neurofibroma and from textbooks. A consensual document was written taking into account extracted data. An annual careful clinical examination is recommended except in cases with complications. Screening investigations are not recommended due to the rarity of complications, generally symptomatic and easily detected during the clinical follow-up. The only controversial exception might be magnetic resonance imaging for early detection of optic pathway gliomas in young children. A co-ordinated follow-up in specialised multidisciplinary centres, providing patients with a rational management, is recommended.

Adolescent↗

Functional melanonychia due to involvement of the nail matrix in systemic lupus erythematosus.

Longitudinal melanonychia is an unusual finding in systemic lupus erythematosus. We report the case of a patient of Arabic extraction with cutaneous lesions of lupus erythematosus on the cheek and chest and longitudinal melanonychia affecting several fingers. Results of histologic examination of the skin and direct immunofluorescence analysis confirmed the diagnosis of systemic lupus erythematosus. The distal matrix revealed changes suggestive of systemic lupus erythematosus accompanied by increased activity of melanocytes but without melanocytic hyperplasia. In this case, longitudinal melanonychia associated with systemic lupus erythematosus was of functional origin.

Adult↗

[Neuritic leprosy disclosed by reversal reaction].

We report a case of neuritic borderline tuberculoid leprosy with lingering insidious growth disclosed by a brutal reversal reaction. Inflammatory polyarthralgia and sensory and motor loss in the median and ulnar territories, without skin lesions, suggested vasculitis. A few weeks later, inflammatory skin lesions developed leading to the diagnosis of biopsy proven leprosy. Leprosy should be considered as a possible diagnosis in patients with multineuritis, especially when associated with rheumatic or cutaneous manifestations. Early skin biopsy in neuritic leprosy is discussed.

Adult↗

[Primary infection with West-Nile virus].

BACKGROUND: The recent outbreak of an epidemic of West-Nile fever in New-York revealed this virus' responsibility in some cases of fatal encephalitis. However the clinical manifestations of the infection may be mild as in the case reported herein. CASE-REPORT: A 41-year-old woman without previous medical history presented an acute febrile rash after a trip to Senegal. The course was spontaneously favorable after 5 days, with a biphasic fever at day 3. Reverse passive hemagglutination test confirmed the diagnosis of acute West-Nile fever. DISCUSSION: West-Nile virus, a mosquito-borne flavivirus is usually responsible for a flue-like disease presenting a maculopapular rash in half of the cases. The biphasic evolution of fever is suggestive of the diagnosis. The diagnosis relies on serological tests but cross-reactivities with heterologous flaviviruses may render the interpretation difficult. A rapid recovery is usual but fatal meningoencephalitis can occur especially in the elderly. Treatment is supportive and prevention is limitation of human exposure. West-Nile virus is widely distributed (Africa, Asia, Middle East), but is also endemic in european areas and responsible for seasonal infections.

Adult↗

[The prescription of repellents].

Repellents are chemical products designed to ward off insects. They are effective against a wide range of arthropods including mosquitoes and flies but not against bees and wasps. Choosing the proper repellent can be difficult because no single product repels all pests. This article compares the performance and toxicity of various repellents based on reports describing systemic, topical, or physical repellents made from natural or synthetic ingredients. Essential oils are the most common natural ingredient. Synthetic ingredients range from dimethyphtalate, ethylexanediol, and diethyltoluamide (DEET) to new molecules such as 35/35, piperidine, bayerepel, and KBR 3023. Our findings indicate that DEET, used as the gold standard in most studies, is still the most effective repellent. There is no synergy between any of these products but combinations (e.g. ethylhexanediol and DEET) can optimize protection. In conclusion we propose guidelines for prescribing repellents to achieve the required level of protection in function of the duration of exposure and benefit-to-risk ratio. Several recommendations are made on the safe use repellents for adults and children.

Animals↗

Neutrophilic eccrine hidradenitis secondary to infection with Serratia marcescens.

Neutrophilic eccrine hidradenitis (NEH) is a rare dermatosis which usually develops after administration of chemotherapeutic treatments. An infective origin is exceptional. We report a patient, previously operated on for ependymoma, who presented with an eruption typical of NEH even though he had not received chemotherapy. Culture of a skin biopsy revealed Serratia marcescens. The dermatosis improved after antibiotic therapy but recurred twice and culture again isolated S. marcescens; electron microscopy revealed cytoplasmic inclusions within neutrophils, suggestive of bacteria. The disease improved every time with appropriate antibiotic therapy. An infective aetiology for NEH is rare: three such cases have been reported, of which one was due to S. marcescens. The originality of our case is the recurrence of the disease on three occasions with the same bacterium isolated on each occasion, with disease remission after antibiotic therapy. This case confirms that infections may be a possible cause of NEH and underlines the necessity to search for infective agents, especially in patients immunocompromised by haematopoietic malignancies and/or chemotherapeutic treatments.

Adult↗

The effect of addition of calcipotriol ointment (50 micrograms/g) to acitretin therapy in psoriasis.

Our purpose was to find out whether the addition of calcipotriol ointment (50 micrograms/g) to systemic treatment with acitretin produces additional therapeutic effects and thereby an acitretin-sparing effect, and further to investigate the safety and tolerability of this combination. A multicentre, randomized, double-blind placebo-controlled study was designed. Patients were randomized to receive calcipotriol or placebo. All patients were treated with a starting dose of 20 mg acitretin per day and doses were adjusted at 2-weekly intervals with increments of 10 mg per day up to a maximum of 70 mg per day. The dose requirement for acitretin, clinical signs and adverse events were recorded. Seventy-six patients were randomized to treatment with calcipotriol 50 micrograms/g ointment twice daily and 59 patients to treatment with the vehicle only twice daily. Clearance or marked improvement was achieved by 67% of the patients in the calcipotriol group and by 41% of the patients in the placebo group (P = 0.006). Calcipotriol treatment proved to have a statistically significant additional effect to acitretin on the Psoriasis Area and Severity Index, redness, thickness and scaliness as compared with placebo. Clearance or marked improvement was achieved with a statistically significantly lower cumulative dose of acitretin by the patients in the calcipotriol group as compared with the placebo group. The number of patients reporting adverse events was pronounced and largely related to acitretin. No significant differences were observed between the two treatment groups with respect to adverse events. Laboratory assessments were essentially normal. The addition of calcipotriol ointment to acitretin treatment contributes to the efficacy, reduces the cumulative dose of acitretin to reach marked improvement or clearance, and is well-tolerated and safe.

Acitretin↗

Erythema dyschromicum perstans: report of a new case and critical review of the literature.

Erythema dyschromicum perstans (EDP), described by Convit et al. in 1961, is a rare dermatosis. Its relationship with ashy dermatosis (AD), described by Ramirez in 1957, is still a matter of debate. We report a typical case of EDP. The patient, of North African origin, had a dyschromic (hypo- and hyperpigmented) eruption on the chest and limbs for 2 years. The lesions were occasionally surrounded by a papular border which spread slowly and centrifugally. Histological examination showed a lichenoid infiltrate. A carcinoma of the lung was simultaneously discovered. No treatment was given, EDP is infrequent and often considered identical to ashy dermatosis in the literature. However, the clinical aspects of the two diseases differ. The main features of these two diseases are reviewed and compared on the basis of a literature review. We conclude that EDP and AD are distinct clinical entities.

Aged↗