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Biomedical subjects

F Piette

Publications and source records attributed to F Piette.

At least 73 records · Page 4Linked to original sources
PubMed1997

[Perforating milia-like idiopathic calcinosis of the extremities in Down syndrome].

INTRODUCTION: Several skin diseases can be seen in patients with trisomy 21. We report a case of miliary calcinosis of the extremities. CASE REPORT: A 15-year old adolescent with Down's syndrome presented small papular miliary lesions which had developed over 18 months and tended to discharge a chalk-like substance via the epidermis. Approximately 15 lesions were present on the hands and feet. Histologically, there was a well-delimited calcium deposit in the superficial dermis. There was no alteration in phosphorus/calcium metabolism. Brain CT-scan and cardiac echography did not reveal any calcifications. DISCUSSION: Miliary calcinosis cutis may not be exceptional in Down's syndrome, although only 9 observations have been reported. Preferential localizations include the hands, wrists and feet. Association with syringoma has been noted but would appear to be fortuitous. Transepidermal elimination of the calcium deposits is frequent. Pathogenic hypotheses include precipitation of calcium salts in sudation products and/or increased synthesis by fibroblasts. The association with trisomy 21 appears to be significant since only three cases have been reported in patients with normal karyotypes. This entity should be individualized as perforating milia-like idiopathic calcinosis cutis of the extremities.

Adolescent↗
PubMed1997

[Ofuji's eosinophilic pustular folliculitis. Efficacy of acitretin].

BACKGROUND: We report the first case of eosinophilic pustular folliculitis (Ofuji's disease) which was successfully treated with acitretin. CASE REPORT: A 50-year old women (HIV negative) had developed over 3 months an erythematopapulous plaque under the left orbit. The clinical and histological diagnosis was eosinophilic pustular folliculitis. Successive treatment with cetirizine then indomethacin was ineffective. Acitretin (0.5 mg/kg/d) was then started and led to cure within 1 month. Six weeks after the patient spontaneously stopped the treatment, the lesion recurred at the same localization. Further treatment with isotretinoin (0.5 mg/kg/d) was then given but did not alter progression of the lesion. Acitretin was then reintroduced at the same dose and again produced rapid cure. Acitretin was then tapered off to 10 mg/d then maintained at this dose as lesions reappeared with further dose reduction. DISCUSSION: It is difficult to treat eosinophilic pustular folliculitis because of the random nature of response to different drugs. General corticosteroids, dapsone and indomethacin are classically proposed but with variable success. Isotretinoin is proposed on the hypothesis of a link with sebaceous secretion, but results have been contradictory. This drug was ineffective in our case. Acitretin did however provide very rapid improvement with an efficacy confirmed at reintroduction. This retinoid which does not have the specific action of isotretinoin could affect follicular keratinocytes which have been shown to be activated in this chronic skin disease.

Acitretin↗
PubMed1996

[Initial manifestations and late diagnosis in chronic atrophic polychondritis: apropos of a series of 15 patients].

An auricular or nasal chondritis or a saddle nose deformity are the initial manifestation in half of cases of relapsing polychondritis; the other initial manifestations are various and less evocative; polyarthritis, laryngo-tracheal symptoms, episcleritis which delay the diagnosis. From 15 cases of relapsing polychondritis, the diagnosis time from the first symptom are studied; this one is long, about 3 years and 6 months (from 3 months to 17 years) in 13/15 of the cases even if the first manifestation is typical (external chondritis). In two cases only, the diagnosis was established after the first attack. This late of diagnosis had socioeconomical impact and exposed to severe complications like tracheal chondritis.

Adult↗
PubMed1996

Growth hormone therapy in elderly people: an age-delaying drug?

The aims of this review are to present a brief overview of growth hormone (GH) physiology and to summarize the studies of GH treatment in adults. Special attention has been paid to randomized controlled trials. Studies have revealed a partial deficiency of GH secretion in the elderly. GH secretion on the average declines by 14% with each decade in normal adults after 20 years of age. Aging has a central effect on the GH secretion and peripheric effect on insulin-like growth factor 1 (IGF-1) through changes in the body composition. GH administration may attenuate several important decrements in body composition and in function associated with aging. GH may also have very potent anabolic effects in surgical situations. Short-term side-effects of GH therapy include edema, carpal tunnel syndrome and arthralgia. A number of agents such as oral GH-releasing peptides (GHRPs) increase GH secretion; they may be an alternative to GH treatment in the future. Further studies of GH replacement are needed, examining issues such as dosage, tolerance and efficacy before the widespread use of GH in the elderly is advocated.

Adult↗
PubMed1996

Linear and whorled nevoid hypermelanosis versus incontinentia pigmenti: is pigmentary incontinence really a distinctive feature?

This report describes a 3-year-old boy who presented with skin lesions characterized by multiple streaks of hyperpigmentation following Blaschko's lines since 6 weeks of age. The clinical features are consistent with the diagnosis of linear and whorled nevoid hypermelanosis (LWNH), but differ substantially from this entity by the pigmentary incontinence observed on both the paraffin and electron microscopy sections. Despite the presence of pigmentary incontinence, the diagnosis of incontinentia pigmenti (IP) can be ruled out on the basis that the patient is a male, with none of the characteristic anomalies present in this syndrome, and that he never presented with inflammatory or eruptive skin lesions. This observation suggests that pigmentary incontinence may not differentiate IP from LWNH as conclusively as previously reported in the literature.

Age of Onset↗
PubMed1996

[Alzheimer's disease lesions: from morphology to cell biology].

Four different approaches to Alzheimer disease changes have been successively applied, and allowed a permanent feed forward-feed back enrichment of knowledge: morphologists described neurofibrillary tangles, senile plaques, amyloid angiopathy; with the help of immunohistochemical and biochemical techniques, they recognised A beta- and tau-associated pathologies; this, in turn, allowed more precise analysis of the lesions, and permitted recognising new ones such as neuropil threads; molecular genetics and molecular biology provided new insights, allowing the discovery of additional pathologic proteins, the relevance of which to physiology and pathology of the nervous system has now to be settled down. The increasingly intricate complex of lesions of Alzheimer syndrome is reviewed. A more comprehensive understanding is urgently needed for initiating efficient therapeutic researches. It will require together continuing a multidisciplinary approach, and a renewal of research in neuropathology.

Alzheimer Disease↗
PubMed1996

[Is the topography of Alzheimer's disease lesions a clue to their pathogenesis?].

Neurofibrillary changes, labelled by antitau antibodies and deposits, labelled by anti-A beta antibodies, were counted in 6 cortical areas in 29 prospectively studied cases (Charles Foix Longitudinal Study). The intellectual status had been assessed by the Blessed test score; 10% of the cases were found to be normal (score > 27), 10 other percents were deeply demented (score < 2) and the other cases were regularly distributed over the intermediate values. Tau positive neurofibrillary changes were present in the hippocampus and in the parahippocampal gyrus even in intellectually normal cases. They were found in a primary sensory cortex (the visual cortex) only in the most severely affected cases. Associative cortices were spared in the normal cases and in the least demented patients. They were involved only at a critical value of the Blessed Test Score. A beta deposits involved more areas than the neurofibrillary pathology and their distribution was less systematically organized. Their density was poorly correlated with the intellectual status. Neuritic plaques, made of an amyloid core and of a crown of tau positive neurites, were present only in those areas that also contained neurofibrillary tangles. Our findings support the contention that neurofibrillary pathology, involving a set of short range, "feed-backward", cortico-cortical connections, is a close correlate of dementia. The role of A beta deposits remains unclear. Although poorly connected with dementia, they could be the remote initiator of the pathological cascade that leads to the neurofibrillary pathology, immediate cause of the cortical dysfunction.

Aged↗
PubMed1995

[Atrial natriuretic factor and brain natriuretic peptide. Variations in elderly subjects with heart failure].

Atrial natriuretic factor (ANF) is a peptide hormone secreted by the atria in response to increased transmural pressure. This peptide is the first of a series of natriuretic hormones which also includes brain natriuretic peptide (BNP). It is destroyed mainly by an ubiquitous enzyme, neutral endopeptidase (NEP). Its main actions are vasodilatation and natriuresis. It is the main physiological agonist of the renin/angiotensin/aldosterone system. In elderly subjects free of cardiovascular disease, baseline concentrations are higher than in younger subjects. In patients with congestive heart disease (CHD), the level of ANF rises due to permanent increased filling pressures. Both atrial and ventricular secretion increase ANF levels which loose their day/night rhythm. ANF is a risk factor independent of mortality, rhythm disorders and acute heart failure in patients with heart failure. BNP is also raised in CHD. There is an inverse correlation between concentration and severity of left ventricule dysfunction. There has been little work on ANF in elderly subjects with CHD. ANF is elevated in these patients and is an independent risk factor for cardiac decompensation. In addition, in very elderly subjects where the diagnosis of CHD is difficult and echocardiography not always possible, assay of BNP could be an interesting diagnostic tool. Currently work on therapeutic possibilities (administration of exogenous ANF, combinations with NEP inhibitor/conversion enzyme inhibitor, ANF/diuretics) have revealed certain problems (short half life of ANF, transient effects, non-specific activity of NEP). The usefulness of ANF and BNP in heart failure in elderly subjects will undoubtedly lie in its capacity to mark disease severity and as a diagnostic tool, particularly in case of acute dyspnoea.

Age Factors↗
PubMed1995

[Mycoplasma infection with Stevens-Johnson syndrome and antiphospholipid antibodies: apropos of 2 cases].

We report two cases of Mycoplasma pneumoniae infections associated with Stevens-Johnson syndrome and antiphospholipid antibodies. Such an association has been noted once in the literature. The relationship between antiphospholipid antibodies and Stevens-Johnson syndrome and others cutaneous manifestations of infections diseases is discussed. Though mainly described in systemic lupus erythematosus and autoimmune diseases, anticardiolipin antibodies and lupus anticoagulant have been found in many infectious disorders. But in the latter conditions, they have been considered by many authors as "non pathogenic" or "non prothrombotic" on epidemiologic and immunologic data. We suggest that antiphospholipid antibodies could possibly play a role in their pathogenesis especially as the mechanisms are not to date clearly understood.

Antibodies, Antiphospholipid↗
PubMed1995

[Familial sarcoidosis: 3 cases in the same family].

Familial sarcoidosis is uncommon, representing less than 5% of the cases. We reported three cases of sarcoidosis in the same family (two sisters and a brother) with different clinical presentation in spite of the presence in each patient of HLA B8 DR3. These HLA phenotype may be related with favourable sarcoidosis. Until the two sisters had articular and benign lymph nodes diseases, the brother had multivisceral sarcoidosis involving the liver, the lung and the mediastinum. Corticosteroid were used during 6 month with favourable evolution. There is 16 years between the three sarcoidosis history but the disease appeared till the third decade. The two sisters and the brother did not live in the same city. These suggest than genetic predisposition is more effective than environmental basis.

Adult↗
PubMed1995

[Hamartomatous dysplasias with hemihypertrophy. 2 cases including one with Proteus syndrome].

BACKGROUND: Hamartomatous dysplasias with hemihypertrophy are a part of several congenital diseases, sometimes difficult to classify when their symptomatology is relatively poor. CASE REPORTS: Case 1. A girl, 11 year-old, suffered from gigantism of the feet, left hemihypertrophy of the limbs, scoliosis and kyphosis. X-rays showed frontal hyperostose, bony and soft tissues overgrowth involving the left limbs and pelvis with hip dislocation and coxa valga. The vertebrae were hypertrophic and dysplastic. No tumor could be found. Case 2. A girl, 18 year-old, suffered from left hemihypertrophy of limbs and face associated with homolateral cutaneous hemangioma. X-rays showed enlargement of bone and soft tissue. No tumor or venous abnormalities were observed. CONCLUSION: A diagnosis of Proteus syndrome was made in the first patient while the second patient was considered to have Klippel-Trenaunay syndrome even though hemangiomatous involvement has been seen in Proteus syndrome.

Adolescent↗
PubMed1995

[Obstruction: a rare complication of congenital giant blue nevus].

BACKGROUND: The blue nevus is an acquired or congenital dermal melanosis, usually small in size. It is sometimes giant and responsible for complications. CASE REPORT: A newborn had a large infiltrated blue news located on buttocks, lombosacral and perianal areas. He was admitted at the age of 3 weeks for an intestinal obstruction which was due to an impassable anal stenosis secondary to the infiltrated nevus. The tumor was excised twice, completed by plastic surgery at the age of 10 months. CONCLUSIONS: This rare congenital giant blue nevus was responsible for an unreported intestinal obstruction. The risk of malignant transformation was another reason for complete excision.

Anal Canal↗
PubMed1995

Keratoderma with scleroatrophy of the extremities or sclerotylosis (Huriez syndrome): a reappraisal.

Keratoderma with scleroatrophy of the extremities, also referred to as Huriez syndrome, is a rare, autosomal dominant condition, first described in 42 of 132 members of two families from northern France. The term sclerotylosis was proposed because of the pseudosclerodermatous appearance of the hands and digits. The distinctive feature of this syndrome is the risk of the development of squamous cell carcinoma on affected skin. Since the initial description of this disease, three other families, and possibly a fourth, have been reported. In the present study, we reassessed the clinical, pathological and genetic data in 114 members of one of the two original families, of whom 27 were affected by this syndrome.

Acitretin↗
PubMed1995

Parallel interleukin 5 synthesis by eosinophils in duodenal and skin lesions of a patient with dermatitis herpetiformis.

A 59 year old man is presented with a diagnosis of dermatitis herpetiformis. Duodenal and skin biopsy specimens from blisters of both recent and late onset were collected before treatment. Electron microscopy, immunohistochemistry, and in situ hybridisation were performed to analyse the presence of activated eosinophils and the local synthesis of interleukin 5 (IL5). Parallel state of eosinophil activation and IL5 synthesis was found in the duodenal mucosa with total flat mucosa and in skin vesicles of recent onset. It is suggested that duodenal and cutaneous eosinophils can synthesise IL5 and then participate in small bowel and skin lesions.

Dermatitis Herpetiformis↗