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Biomedical subjects

M Dupin

Publications and source records attributed to M Dupin.

At least 19 recordsLinked to original sources
PubMed2006

[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↗
PubMed2005

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↗
PubMed2004

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↗
PubMed2002

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↗
PubMed2002

[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↗
PubMed2001

[Transformation of mycosis fungoides to pleomorphic T-cell lymphoma and central nervous system involvement].

INTRODUCTION: Although mycosis fungoides is a malignant T-cell lymphoma involving mainly the skin, neurological complications are possible, with a poor prognosis. EXEGESIS: A 59-year-old man, treated for mycosis fungoides with transformation to a pleomorphic T-cell lymphoma for 1 year, was seen for mental status changes with confusion. A brain parenchyma localisation was found. CONCLUSION: This observation emphasizes the exceptional neurological tropism in the patients with mycosis fungoides. A transformation to a more aggressive cutaneous T-cell lymphoma seems necessary to induce a central nervous system involvement.

Brain Neoplasms↗
PubMed2001

[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↗
PubMed2000

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↗
PubMed1998

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↗
PubMed1998

The atrophic variant of dermatofibrosarcoma protuberans in childhood: a report of six cases.

Dermatofibrosarcoma protuberans (DFSP) is typically diagnosed during early adult life at a tumoral stage. It occurs only rarely in children. We report six childhood cases of DFSP which presented initially with the misleading clinical appearance of atrophic plaques, and we review over 140 cases of DFSP in childhood. As compared with adult forms, DFSP in children does not show distinctive features except for a tendency for acral localization. The diagnosis is difficult because of the slow course of the lesions, which present initially as apparently benign atrophic morphoeaor keloid-like plaques. We believe that DFSP in childhood is probably under-estimated, as a significant proportion of patients diagnosed as young adults had an onset several years earlier. Better knowledge of the initial appearance is important for making an early diagnosis and for an easier surgical treatment.

Adolescent↗
PubMed1998

[Infiltrating lipoma].

BACKGROUND: Deep or infiltrating lipoma is an often misdiagnosed clinical entity. We report two typical cases. CASE REPORTS: Two women, aged 92 and 62 years, were seen for a tumefaction on an upper limb with progressively increasing volume. The clinical presentation suggested deep lipoma, confirmed by magnetic resonance imaging and histology which eliminated liposarcoma, the main differential diagnosis. Surgical excision was successful. There has been no recurrence. DISCUSSION: These two observations recall the clinical, diagnostic and therapeutic features of infiltrating lipoma.

Aged↗
PubMed1998

[African tick-bite rickettsiosis: the 1st case of autochthonous infection?].

BACKGROUND: African tick-bite fever is caused by R. africae. All cases reported to date in France have occurred among patients who came from southern Africa, the endemic zone. We report the first case, to our knowledge, of a patient infected in France. CASE REPORT: A 69-year-old white man who had never left France developed fever and 3 "black spots" on the legs with lymphangitis and enlarged nodes. The clinically suspected diagnosis was confirmed by positive serology reactions to R. africae. As cross immunity with R. conorii can occur, the diagnosis was further confirmed by western blot for R. africae and by the persistence of the reaction after adsorption of the R. conorii serum. DISCUSSION: African tick-bite fever was identified as a clinical entity different from Mediterranean spotted fever by Kelly in 1992 who demonstrated the causal role of R. africae. The typical clinical presentation associates fever, several black spots, lymphangitis and multiple node enlargement. The serological diagnosis is difficult owing to cross immunity with R. conorii. Western blot and polymerase chain reaction are required for definitive diagnosis. In our case, the infection was probably due to a R. africae imported into France in the luggage of the patient's daughter who had spent 3 months in Zimbabwe.

Aged↗