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Biomedical subjects

A Cosnes

Publications and source records attributed to A Cosnes.

At least 19 recordsLinked to original sources

Oral Crohn disease: clinical characteristics and long-term follow-up of 9 cases.

BACKGROUND: Oral localization of Crohn disease is uncommon and must be differentiated from nonspecific lesions. Its natural course and its long-term prognosis are unknown. OBSERVATIONS: We studied 9 patients (8 male, 1 female; age range, 7-52 years; median age, 16 years) with Crohn disease and specific oral lesions, including deep linear ulcers, pseudopolyps, and/or labial or buccal swelling and induration. The prevalence of such lesions was 0.5%. The median follow-up was 11 years. Oral localization developed before (n = 2), at the same time as (n = 2), or after (n = 5) the onset of the digestive disease. Noticeable associated localizations were observed in the anoperineum (n = 8) and the esophagus (n = 3). The median duration of the oral lesions was 4 years (range, 1-13 years), without necessary parallelism with the digestive localization. Five patients had complete healing after a median delay of 2 years. CONCLUSIONS: Oral localization of Crohn disease is characterized by a marked male predominance, a young age at onset of Crohn disease, and a very protracted course. The high prevalence of associated anal and esophageal involvement suggests that Crohn lesions have a particular trophicity for squamous cell epithelium.

Adolescent↗

The treatment of mild pemphigus vulgaris and pemphigus foliaceus with a topical corticosteroid.

Seven patients with mild pemphigus vulgaris (n = 3) or pemphigus foliaceus (n = 4) were treated with a very potent topical corticosteroid alone. Clobetasol propionate 0.05% cream was applied to mucosal lesions and involved skin twice a day for at least 15 days, then progressively tapered. Pemphigus was considered to be controlled if healing of lesions was obtained, with a 75% decrease in the number of new lesions per week without addition of any systemic treatment. In all seven patients, the disease was controlled initially with healing of cutaneous lesions within 15 days, while healing of mucosal lesions took at least 1 month. In four patients, remission was maintained with topical corticosteroid alone for a mean 19-month follow-up. In three patients, relapse occurred after 2-11 months, requiring a systemic treatment.

Administration, Topical↗

Increased fibroblast elastase activity in acquired cutis laxa.

BACKGROUND: Acquired cutis laxa is a rare disease characterized by sagging skin, premature wrinkling and reduced skin elasticity. OBSERVATION: We report a 21-year-old woman, who presented with acquired cutis laxa on the face and the ear lobes. Urticarial papules had preceded for 6 years. There was no systemic involvement. Skin specimens were obtained from lax skin and urticarial papules, and from healthy controls. Histology showed only few perivascular lymphocytes in lax ear skin and a dense inflammatory infiltrate in urticarial skin. In both biopsies elastic fibres were decreased as demonstrated by computerized morphometric analyses. Elastase activities of fibroblasts in culture were evaluated. There was a 2- to 3-fold increase in elastase activity in urticarial skin fibroblasts, contrasting with a normal elastase activity in lax ear skin. CONCLUSION: Our findings suggest that the inflammatory cells could play a significant role in the destruction of elastic fibres.

Adult↗

Chilblains and Raynaud phenomenon are usually not a sign of hereditary protein C and S deficiencies.

Hereditary protein C and S deficiencies are risk factors for thrombosis. They are associated with purpura fulminans and coumarin-induced skin necrosis. Recently, necrotic livedo of the extremities, severe chilblains and severe frostbite have been observed in protein C or S deficient patients. Our study was designed to evaluate the prevalence of cold-induced acral manifestations in patients with protein C or S deficiency. One-hundred-and-six patients with protein C or S deficiency and controls matched for sex and age were studied by questionnaire. Data included any history of acral manifestation possibly related to cold exposure, i.e. chilblains, Raynaud phenomenon, acrocyanosis and possible associated factors. Assessment of the diagnosis by a dermatologist was recorded. No difference was found in the prevalence of acral manifestations between patients and controls. This study suggests that protein C and S deficiencies are not risk factors for cold-induced acral manifestations.

Adult↗

[Acral erythema and HIV infection].

BACKGROUND: Acral erythema is a non specific skin condition observed in connective tissue diseases, viral infections and toxic skin reactions. More recently, this symptom has been reported in HIV-infected patients. CASE REPORT: We observed two cases of acral erythema in HIV seropositive patients. Both patients were drug abusers. Erythema was limited to the limbs with no clinical manifestation of connective tissue disease. Both consumed approximately 40 tablets of codeine daily and had hepatitis C without cirrhosis. DISCUSSION: Our 2 cases of acral erythema in HIV-infected patients can be added to 32 cases of digital erythema in HIV patients reported in the literature. The common features in most of these patients was intravenous opiate abuse and hepatitis C without cirrhosis. The degree of immunodeficiency, the nature of opportunistic infections and antiretroviral treatments do not appear to affect the development of skin signs. HIV infection, simultaneous HIV-HCV infection and codeine suggest they might have a pathogenic role in the development of distal vascularization disorders.

Acrodermatitis↗

[Acute mast cell leukemia disclosed by vasomotor flushing].

INTRODUCTION: Acute mast cell leukemia is a rare and severe disease. We report herein a case associated with a flush syndrome. CASE REPORT: A 44-year-old man, presented with a flush of face and trunk. Bone marrow was infiltrated with immature mast cells. In spite of chemotherapy and bone marrow transplantation the patient deceased. DISCUSSION: Pheochromocytoma, carcinoid tumor, and mastocytosis are associated with a flush syndrome. In our patient the diagnosis was an acute mast cell leukemia. Acute mast cell leukemia can follow systemic mastocytosis or occur de novo. This disease is of poor prognosis. No treatment is available.

Adult↗

[Resistance to activated protein C disclosed by postphlebitic disease. Two cases].

INTRODUCTION: Recently, resistance to activated protein C has been discovered. Resistance to activated protein C appears to be the main cause of familial thrombosis. CASE REPORT: Two consecutive patients have been studied. Both have a venous insufficiency associated with eczema in one patient and venous ulceration in the second patient. The two patients had a personal and familial history of venous thrombosis. In both patients, a resistance to activated protein C was found associated with a mutation in the factor V gene in residue 506. DISCUSSION: When a personal or familial history of the venous thrombosis is associated with symptoms of venous insufficiency, resistance to activated protein C must be added to the search for proteins C, S and anti-thrombin III deficiency.

Adult↗

Dermatomyositis without muscle weakness. Long-term follow-up of 12 patients without systemic corticosteroids.

BACKGROUND AND DESIGN: We retrospectively studied 12 patients with dermatomyositis (DM) with at least one laboratory criterion of muscle involvement but without muscle weakness. We reported their outcome without systemic corticosteroid treatment and searched for distinctive baseline data and associated neoplasia among a group of 29 patients with classic DM. RESULTS: After a mean follow-up of 5.3 years, complete or partial skin improvement occurred in nine patients with DM without muscle weakness, and muscle enzyme levels were normalized in all. When compared with the classic DM group, no statistical differences were found for associated neoplasia or for any baseline data studied except, as expected, for muscle parameters. CONCLUSIONS: The lack of distinctive baseline data between DM without muscle weakness and classic DM suggests that there is a continuum from amyopathic to classic DM. However, DM without muscle weakness is a clinical entity with respect to favorable long-term prognosis. Unless frank muscle weakness develops, treatment with systemic corticosteroids can be avoided.

Adolescent↗

[Cosmetic dermopigmentation. The pigment stays ... as do regrets].

INTRODUCTION: Cosmetic dermopigmentation designates tattooing of the superficial derma with pigments in order to obtain an aesthetic effect. We observed two cases illustrating the inconveniences of this technique. CASE REPORTS: Two women underwent dermopigmentation on the face in a beauty institution to create pseudo-freckles. Definite macular hyperpigmentation occurred with dyschromia in both subjects. COMMENTS: There is a major risk in subjects who undertake dermopigmentation of freckles and/or naevus, a practice which should be discouraged by dermatologists.

Adult↗

Internal involvement in localized scleroderma.

We studied 76 consecutive patients with localized scleroderma (morphea with or without linear scleroderma) and analyzed the frequency, prognosis, and predictors of internal involvement in a subset of 53 patients systematically investigated for the presence of such involvement. Internal involvement was found by systematic examination in 16 patients. Only 2 of them, including 1 patient who developed systemic scleroderma, had symptomatic and severe visceral disease. The other 14 patients had asymptomatic and minor abnormalities consisting of abnormal lower sphincter pressure, and/or peristaltic failure in the esophagus and slightly impaired carbon monoxide diffusion in the lung. Frequent seroimmunologic abnormalities had no predictive value. Three parameters were found to be associated with internal involvement: male gender (p < 0.05), increasing number of plaque-like lesions (p = 0.02) and hypergamma-globulinemia at 1st examination (p < 0.005). Mild esophageal and pulmonary involvement are not rare in morphea but are usually silent. In our series, after a median follow-up of 48 months, such involvement did not impair the prognosis. The mildness of these visceral abnormalities suggests that they do not justify routine detection in asymptomatic patients. Morphea and systemic scleroderma behave as 2 different diseases.

Female↗