[Pemphigoid gestationis: a single model of organ specific auto-immunity].
Explore the source record for details and available documents.
Biomedical subjects
Publications and source records attributed to A L Claudy.
Explore the source record for details and available documents.
We report a case of a 48-year-old woman who presented with chronic essential pruritus and was successfully treated with the transdermal estradiol system because of menopausal discomfort. Suberythemogenic UVB phototherapy was proposed to the patient in order to control her pruritus. She developed hyperpigmentation at the application site of estradiol. To the best of our knowledge, this is the first report suggesting a direct relationship between estrogen and melanin synthesis in humans.
Explore the source record for details and available documents.
The aim of our study was to assess the behaviour of a despecified collagen tissue laid on desepidermized human skin (taking area of skin graft). The collagen tissue was prepared according to Bell's method. The collagen was latticed, contracted by fibroblasts, chemically treated (formaldehyde or glutaraldehyde) to stabilize the fibrils and then despecified by cialit treatment. This tissue laid on superficial, non infected wounds very rapidly took a necrotic appearance and was totally lysed after 10 to 12 days without any modification of the healing course. No bacterial infection was observed. Histological and ultrastructural studies showed desorganization of collagen fibre bundles and tissue invasion by inflammatory cells. Circulating antibodies to collagen were absent at day 30. This model lacks interest as a substitute for superficial tissue replacement, but a healing function could be assigned to its high chemotactic power for polynuclears and macrophages and should allow its use in cases of deep tissue loss.
A case of a cutaneous squamous cell carcinoma (SCC) occurring at the site of a long-standing recurrent HSV infection is described. No deficit of the cell-mediated immunity was recorded. HSV2 was isolated in several viral cultures. HSV DNA was visualized in the SCC by in situ hybridization with biotinylated probes in paraffin-embedded tissue. The samples were negative for HPV2, 5, 16, and 18 probes. A causal relationship between HSV infection and cutaneous SCC is hypothesized.
We report a case of typical Letterer-Siwe disease associated with a subacute monocytic leukemia in a 75-year-old man. The simultaneous presentation of both diseases is rare. It may be speculated that our patient did not present a chance association of two different processes but a disorder of the Langerhans cell-mononuclear phagocytic system lineage.
The effects of cyclosporine were studied in nine patients suffering from pemphigus vulgaris. Of four patients treated with cyclosporine alone, only one cleared. Of four corticosteroid-resistant pemphigus vulgaris patients, all improved after cyclosporine addition to corticosteroids. The last patient treated from the beginning with a combined treatment (cyclosporine-corticosteroids) did not respond. The main advantage of using cyclosporine is to allow a decrease in corticosteroid dosages and to permit treating corticosteroid-resistant pemphigus vulgaris patients. No detectable irreversible side effects were noted. The treatment was discontinued in two patients because of reversible side effects. Cyclosporine alone does not seem to be an adequate treatment of the acute phase of pemphigus vulgaris but could be used in addition to corticosteroids. The most important drawback of cyclosporine treatment is the occurrence of clinically silent renal dysfunction (tubular involvement and interstitial fibrosis), which may occur during long-term treatments. More studies need to be carried out to determine the effects of low doses of cyclosporine on renal function in patients who have normal renal functions.
Explore the source record for details and available documents.
The origin of leukocytoclastic vasculitis (LV) being often difficult to determine, we have undertaken since 1980 a prospective study of factors associated with LV. We selected 53 patients whose LV was clinically predominant, and excluded patients in whom LV was an expected phenomenon in a known autoimmune or infectious disease. Twenty-eight of the 53 patients presented with a typical Gougerot-Ruiter disease, 15 with a bullous or necrotic form of the disease and 10 with urticarial lesions. Detail of the prospective laboratory tests performed is given in table I. Correlations between laboratory values and LV-associated factors were significant with the decrease of complement but not with the presence of circulating immune complexes, rheumatoid factor, cryoglobulin or direct immunofluorescence test positivity. Most of the associated factors in our series were infectious agents (streptococci, hepatitis virus), immunological agents (rheumatoid factor, cryoglobulin) or drugs known to be potential LV-inductors; other factors were less common or quite recently described (enterovirus, Yersiniae, cirrhosis, primary liver cancer, Chlamydiae, refractory anemia with an excess of myeloblasts. We do not feel that a large series of laboratory tests should be performed in every case of LV. The clinical context and simple laboratory tests, such as blood cell count, complement assay, plasma electrophoresis and a search for rheumatoid factor should be enough to guide the clinician and help him decide whether further investigations are needed. However, it should be noted that in some cases without clinical pointers only full virological evaluation enabled us to determine that enteroviruses may be involved in the pathogenesis of LV.
A case of thymoma associated with chronic lupus erythematosus is reported. The course of the lupus was parallel to that of the thymoma, with several successive relapses. Histologically, the thymoma became increasingly undifferentiated. Chemotherapy only resulted in a few months of remission.
Explore the source record for details and available documents.
Three patients with acute generalized exanthematous pustular dermatitis (AGEPD) are presented. This new clinical entity comprises generalized erythema, erythema multiforme, amicrobial pustules, fever and spontaneous healing over a 10-day period. Histologically, there are a mixed neutrophilic and eosinophilic vasculitis and an eosinophilic subcorneal and spongiform pustule. All the patients experienced pharyngitis 1 week before the onset of the rash. Two of them had high-titered IgM-neutralizing antibodies to enteroviruses and 1 had an enterovirus in stool and urine. AGEPD may be related to a viral infection and may occur in patients with a genetic predisposition to psoriasis. The diagnosis of AGEPD should be made early enough so that the patients are not given the often aggressive therapy of pustular psoriasis.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
The authors describe three cases of refractory anaemia with an excess of myeloblasts in the bone marrow (RAEM), associated with pyoderma gangrenosum (PG) and vasculitis. The first patient was an 85-year old man whose RAEM had begun in 1979. In 1985, he developed pyoderma gangrenosum in the popliteal fossa and on the right heel. Histology confirmed the diagnosis. A direct immunofluorescence test on the biopsy specimen was negative. Apart from the haematological syndrome, there were no laboratory abnormalities. The cutaneous lesions disappeared after 7 weeks of treatment with prednisolone 20 mg per day. The blood disease remained unchanged. The second patient was a 71-year old woman with RAEM since 1982. In 1984, she presented with lesions of cutaneous vasculitis located on the anterior aspect of the upper third of her left leg. There was neither arthralgia nor fever, and no history of drug toxicity or infection. Beside RAEM, polyclonal hyperglobulinaemia was present. Histological examination of the skin showed evidence of vasculitis with fibrinoid necrosis of vascular walls and perivascular lympho-histiocytic infiltrate with granulocytes and slight leucocytoclasia. Direct immunofluorescence testing of the skin demonstrated intravascular complement deposits. There were no circulating immune complexes; measurements of complement and complement fractions gave normal values; no cryoglobulin was found. The cutaneous lesions recurred on two occasions in 6 months, although no drug toxicity or infection could be elicited and the haematological syndrome was unaffected. The third patient was a 67-year old man with RAEM since 1982. In 1983, an ulcero-necrotic lesion spontaneously developed on his right leg.(ABSTRACT TRUNCATED AT 250 WORDS)
Explore the source record for details and available documents.