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Biomedical subjects

E Delaporte

Publications and source records attributed to E Delaporte.

At least 145 records · Page 8Linked to original sources

["Metastatic" Crohn's disease of the penis].

Inflammatory genital cutaneous involvement of Crohn's disease is a rare complication. In a 23 year old man, who had colonic Crohn's disease treated by colectomy 3 years earlier, a phimosis revealed a penile localisation of Crohn's disease. "Metastatic" locations of Crohn's disease are defined by granulomas sitting at a distance from the injured bowel. Their clinical manifestations are misleading and their course is independent of the digestive disease. In our patient, after failure of classical therapy, cyclosporin A permitted a slow regression.

Adult↗

HTLV-I infection among prostitutes and pregnant women in Kinshasa, Zaïre: how important is high-risk sexual behavior?

High-risk sexual behavior as risk factor for human T-cell lymphotropic virus type I (HTLV-I) infection was assessed in cross-sectional studies with 1,183 prostitutes and 1,166 pregnant women in Kinshasa, Zaire. Eighty six (7.3%) prostitutes were positive for HTLV-I. The seroprevalence among prostitutes from the regions along the equator was 12.7%, whereas among prostitutes from the other regions it ranged between 0 and 4.3%. In the prostitutes from the high-prevalence regions, but not in the prostitutes from the low-prevalence regions, HTLV-I infection was associated with increasing age [odds ratio (OR) = 1.1 per year increment], active syphilis (OR = 2.3), and human immunodeficiency virus (HIV) infection (OR = 2.0). Forty three (3.7%) pregnant women were HTLV-I seropositive. Among the women from low-prevalence regions, there was no significant difference in HTLV-I seroprevalence between prostitutes (4.3%) and pregnant women (3.5%). In a group of 409 prostitutes who were observed for a mean duration of 23 months, the incidence of HTLV-I infection was 0.7 per 100 women-years, whereas the incidence of HIV infection was 9.8 per 100 women-years. We conclude that in Kinshasa prostitution per se was not associated with an increased risk of HTLV-I infection.

Adult↗

[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↗

[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↗

[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↗

[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↗

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↗

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↗

Interferon-mediated changes in the expression of CYP1A1 in human B lymphoblastoid (AHH-1 TK +/-) cells.

The expression of constitutive and inducible cytochrome P450s has been shown to be downregulated by interferon through an unknown pretranslational mechanism that depresses the mRNA encoding P450 apoproteins. To establish an association between gene transcription and P450 apoprotein downregulation by interferon, we studied the effect of recombinant interferon (IFN-alpha 2a) on CYP1A1 in human B lymphoblastoid cell lines. The cHoI cell line expresses inducible native CYP1A1, while the genetically engineered derivative h1A1 v2 expresses a noninducible extrachromosomal vector-derived human CYP1A1 cDNA lacking the CYP1A1 promoter region. We characterized CYP1A1 activity, apoprotein, and mRNA by ethoxyresorufin O-deethylase activity, Western immunoblotting, and Northern blot analysis, respectively. In cHoI cells, following induction with dibenz[a,h]anthracene, interferon depressed CYP1A1 apoprotein and mRNA levels by 55 and 76%, respectively, with no detectable changes in enzyme activity. In h1A1 v2, however, interferon increased CYP1A1 activity, apoprotein, and mRNA. The depression of CYP1A1 mRNA and apoprotein levels incHoI cells, in contrast with the increase observed in h1A1 v2 cells, suggests that nuclear mechanisms are essential for interferon-mediated depression of inducible P450s. From our preliminary results we propose that interferon-mediated downregulation of CYP1A1 may result from inhibition of gene transcription.

B-Lymphocytes↗

SAPHO syndrome.

Palmoplantar pustulosis and severe acne are sometimes associated with peculiar aseptic skeletal conditions, but such skeletal lesions can be found without skin lesions. The term SAPHO syndrome has been coined for this cluster of manifestations. (The acronym SAPHO refers to synovitis, acne, palmoplantar pustulosis, hyperostosis, and osteitis.) The most common site of the disease is the upper anterior chest wall, characterized by predominantly osteosclerotic lesions, hyperostosis, and arthritis of the adjacent joints. Osteosclerosis of the vertebral bodies, hyperostosis, and erosions of the vertebral plates can be encountered. Unilateral sacroiliitis is frequently observed. Long bone involvement consists of osteosclerosis or osteolysis with periosteal new bone formation. Peripheral arthritis can be present but is rarely associated with joint destruction. The pathogenesis of this syndrome remains unknown, but a link with seronegative spondyloarthropathies is probable. Radiologists should be aware of this unusual syndrome to avoid misdiagnosis (eg, tumor, infection), unnecessary surgery, and antibiotic therapy.

Acne Vulgaris↗

Dermatomyositis with follicular hyperkeratosis.

We report 2 cases of dermatomyositis with follicular hyperkeratosis (FHK) in children. They occurred in a 10-year-old Vietnamese girl and a 9-year-old Caucasian boy. The girl's FHK disappeared after 2 months of treatment. The boy presented, 15 months after the onset of his dermatomyositis, with a generalised FHK which lasted for 6 months. FHK can appear before, during or after dermatomyositis. It is more often generalized but can be localised. Erector pili myositis and ostial hyperkeratosis may be the explanation. The prognostic value of FHK in dermatomyositis is unknown. This manifestation, initially considered to be more frequent in the Far East, is not as rare in Western countries as the few reported cases suggest.

Child↗