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O Enjolras

Publications and source records attributed to O Enjolras.

At least 91 records · Page 5Linked to original sources
PubMed1992

[Hemangiomas and superficial vascular malformations: classification].

Superficial vascular malformations of the face, trunk and limbs are better known today, and they can be divided up into simple and complex vascular malformations. Simple vascular malformations may form five major categories: immature hemangiomas of infants, port-wine stains, capillarovenous angiodysplasias, and arteriovenous fistulae and malformations. Complex angiodysplasias are systematized (Sturge-Weber and Bonnet-Dechaume-Blanc syndromes, Cobb's metameric angiomatosis, Klippel-Trenaunay and Parkes Weber's syndromes) or disseminated (Weber-Osler-Rendu disease and blue rubber-bleb nevus syndrome). Various modalities of treatment may be contemplated, according to the type of malformations, and an interdisciplinary consultation is essential to decide whether a watch-and-wait policy, a physical method (laser), embolization, fibrosing injections, vascular, maxillofacial or plastic surgery, or a successive combination of various techniques should be resorted to.

Angiodysplasia↗
PubMed1992

[Exploration strategy for superficial vascular malformations].

Transillumination of cystic lymphangiomas, MRI of venous vascular malformations, pulsed Doppler and arteriography of arteriovenous malformations, echo-Doppler and radiomeasurement of malformations in the lower limbs are key examinations in the exploration strategy for superficial vascular malformations.

Adult↗
PubMed1992

[Congenital cutaneous Langerhans histiocytosis. Apropos of 7 cases].

Seven cases of congenital Langerhans' cell histiocytosis (LH) are reported, with emphasis on clinical and immunohistochemical features. This is a polymorphic disease at birth. In 4/7 cases, the diffuse, generalized rash could be classified as cutaneous Letterer-Siwe disease (LSD); 3/4 remained purely cutaneous and healed in less than 3 months; whereas the fourth-one persisted, pulmonary lesions appeared, and the infant died on his 40th day. In 3/7 cases, the clinical diagnosis at birth was either a Blueberry Muffin Baby (BMB) or Hashimoto-Pritzker type LH (HPLH); the lesions healed rapidly, although one cas was contradictory: typical BMB at birth, histology mimicking a monoblastic cutaneous leukemia, no T.O.R.C.H. syndrome, normal bone marrow, immunophenotyping of LH, auto-involution; 2/3 were MZ twins, both with few lesions. We would like to stress the fact that the clinical spectrum of LH should include BMB, which, however, in most cases must be considered a differential diagnosis. Regarding cutaneous congenital LH, an eponymic classification (LSD, HPLH) is difficult to follow strictly, because overlapping pictures are observed. There is a wide spectrum of cutaneous congenital LH. The main problem at birth is the lack of prognostic criteria. Neither the presence of the rash at birth, nor its type and extension, is necessarily evidence of risk of systemic disease. Cases of HPLH involute, as also do cases of cutaneous LSD, and the "Blueberry Muffin" type of LH; overlapping clinical aspects exist. Histopathological data, electron microscopy or immunohistochemistry, define LH, but they do not enable the outcome to be predicted.(ABSTRACT TRUNCATED AT 250 WORDS)

Diagnosis, Differential↗
PubMed1991

[Local treatments of cutaneous psoriasis].

Most of psoriatic patients require topical therapy. Dermal application of drugs may be the unique treatment; but it can also combined with oral drugs, or phototherapy. Patients are treated at home or in day-care centers; some require hospitalization. Topical corticosteroids are widely used: abuses are frequently observed, therefore skin and systemic side-effects may occur to varying degrees. Tars are still useful. Short-contact anthralin is active and well tolerated, and compliance is better than with conventional tar therapy, especially in children. Topical mechlorethamine clears the plaques but contact dermatitis may occur. New therapeutic approaches include Vitamin D3 analogues.

Administration, Topical↗
PubMed1991

[Superficial vascular (arterial and venous) malformations: clinical aspects and complementary tests].

A simple and precise classification has been established during a ten-year international cooperation. There are two major groups: hemangiomas, always regressive; and vascular malformations, which never regress; they grow, throughout life, to varying degrees. Management of vascular malformations has been clearly defined. Capillary malformations do not require examination, unless they are associated with other anomalies, such as hypertrophic underlying bone, or the leptomeningeal vascular anomaly of the Sturge-Weber syndrome. Venous type malformations are diffuse, and consist of entirely anomalous channels, with low flow. CT scan and MRI clearly demonstrate the extent of tissue involvement in venous malformations; for these slow-flow malformations the evaluation is performed without resorting to invasive diagnostic techniques: phlebography is rarely performed, and arteriography is unnecessary. Arteriovenous malformations are dangerous high-flow vascular malformations, leading to skin ischemic necrosis, and congestive cardiac failure. Arteriography shows enlarged tortuous arteries, with arteriovenous shunting, and early venous drainage. CT scan and MRI show the deep components of the lesions. Doppler ultrasound evaluation is used to follow the course of the disease.

Arteriovenous Malformations↗
PubMed1990

Management of alarming hemangiomas in infancy: a review of 25 cases.

During the past 10 years, 25 infants with alarming hemangiomas--lesions that impaired important functions and were life threatening, especially when there was visceral involvement--have been treated. A vascular mark was present at birth in 68% of these infants. Visceral hemangiomas were associated with bulky cervicocephalic hemangiomas or with small hemangiomas scattered over the body. Among the 25 infants, 12 had laryngeal hemangiomas, 3 had hepatic hemangiomas, and 1 had gastrointestinal hemangiomatosis. Ocular sequelae, malocclusion, and cutaneous distortion were the most important functional problems. Corticosteroid treatment was used for 23 of 25 infants with alarming hemangiomas. There was a varied treatment response: total failure (30% of the patients); excellent, dramatic, rapid improvement (30% of the patients); and moderate, doubtful response, with the natural course of the disease remaining unaltered (40% of the infants). Arterial embolization, used in 6 infants, gave inconstant results. Cardiac failure, frequently associated with large cutaneous hemangiomas and always seen with hepatic multinodular hemangiomas, required digitalization. In some cases arterial embolization reduced the increased cardiac output. Liver hemangiomas had a high mortality; all 3 infants with hepatic involvement died.

Adrenal Cortex Hormones↗
PubMed1989

A cAMP binding abnormality in psoriasis.

In 34 psoriatic patients with various cutaneous manifestations (psoriasis vulgaris, erythroderma psoriaticum, guttate psoriasis), the ability of the RI regulatory subunit of cAMP-dependent protein kinase (PKA) to bind a cAMP analogue (8-azido [32P] cAMP) in erythrocyte membranes was significantly lower than that in 19 normal subjects (mean [SEM] 565 [35] vs 930 [35] fmol/mg protein). This enzyme defect was not found in patients with other forms of dermatitis that can be confused with psoriasis or with other inflammatory diseases. There was a significant negative correlation between the severity of the disease as expressed by the psoriatic area and severity index score and the binding of the cAMP analogue to PKA. A long-term study showed that oral retinoid treatment of psoriatic patients resulted in a correction of the binding defect. Unaffected members of psoriatic families had significantly lower than normal binding of cAMP to PKA (773 [60] fmol/mg protein). This study shows for the first time that in psoriasis a biochemical defect expressed in erythrocytes correlates with the severity of the disease as well as its clinical evolution. These results will be useful in clinical management of psoriatic disease for the choice and follow-up of retinoid therapy.

Administration, Oral↗