[Lichen striatus in children and "blaschkitis" in adults].
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Biomedical subjects
Publications and source records attributed to F Cambazard.
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Skin basocellular carcinoma is very frequent, and does not usually cause distant metastasis. However, a few cases of distal lymph node, pulmonary, bone metastasis have been described in the literature. In the present case, we report a basocellular mediastinal lymph node metastasis. Some precise histological characteristics described by Lattses must be present to admit authenticity of these metastasis. The treatment of such cases is based on surgery, radiotherapy and chemotherapy containing cisplatinum. However, the overall prognosis of these metastases remains poor.
INTRODUCTION: Human papilloma virus is highly prevalent, but rarely localized in the conjunctiva. CASE REPORT: A 19-year-old man with no past history of skin or mucosal disease consulted for tumefaction of the conjunctiva. Exeresis revealed a viral papilloma. In situ hybridization was positive for human papilloma virus 6/11. DISCUSSION: Conjunctival lesions of human papilloma virus are often located in the caroncula as in our patient. The papillomatous aspect of the tumour may suggest squamous cell carcinoma (sometimes induced by human papillomavirus 16/18). Contamination may be manual or occur at birth via the maternal genital mucosa. Treatment usually is based on wide exeresis with cryo-application although spontaneous regression is possible.
Over the past 8 years, we have followed a child born as a harlequin baby, who survived due to treatment with retinoids. His condition evolved clinically towards the erythrodermic form of lamellar ichthyosis (non-bullous congenital ichthyosiform erythroderma, NBCIE). According to ultrastructural and biochemical criteria, our patient originally presented with type II harlequin ichthyosis. Investigations showed an abnormal keratinosome structure and extrusion, a keratin pattern characteristic for epidermal hyperproliferation, and an absence of conversion of profilaggrin to filaggrin. Persisting keratinocyte hyperproliferation, associated with the presence of a dermal infiltrate, is in agreement with the present clinical picture of severe NBCIE. However, abnormal lamellar body production and defective filaggrin processing, which is not one of the diagnostic criteria of NBCIE, persist in the patient's skin. Further studies of the epidermal lipid composition, and of possible mutations of the keratinocyte transglutaminase gene performed on epidermal cell cultures of harlequin ichthyosis, will be necessary before type II harlequin ichthyosis can be accepted as an extremely severe form of NBCIE.
INTRODUCTION: The objective of this study was to evaluate the efficacy of thalidomide in the treatment of chronic erythema multiforme unresponsive to usual treatments. PATIENTS AND METHODS: Twenty-six patients with chronic erythema multiforme were given thalidomide 100 mg/day after other treatments had failed, particularly acyclovir and prednisone. RESULTS: Twenty patients had recurrent erythema multiforme and were given thalidomide at the beginning of an episode. The duration of the episodes was reduced by 11 days on the average. Six of the patients had subintrant erythema multiforme and were given continuous treatment. Lesions disappeared within 5 to 8 days and remission was maintained with low dose-treatment. DISCUSSION: The spectacular results obtained here should be verified in a controlled study.
INTRODUCTION: Skin cancers are more frequent and more aggressive in immunosuppressed patients. CASE REPORT: A 58-year-old man was seen in January 1994 for squamous cell carcinoma of the right shoulder which had grown to 7 x 7 cm in the last 5 months. The patient had a past history of surgical exeresis of 5 squamous cell carcinomas and 3 basal cell carcinomas. Despite complete exeresis, the disease spread to localized then diffuse skin metastases. Acitretine and alpha-interferon were uneffective. Polychemotherapy stabilized the situation but several bronchopulmonary infections with atypical germs led to death (in February 1995). The patient had CD4 lymphocytopenia with a count < 100/mm3 in January 1994 of unknown origin (viral infection was eliminated). DISCUSSION: Our patient had idiopathic CD4 lymphocytopenia. In 40 p. 100 of the cases CD4 lymphocytopenia is caused by AIDS and in 53 p. 100 by other diseases, especially skin diseases including Kaposi syndrome, mycosis fungoides, squamous cell or basal cell carcinoma. The cutaneous carcinomas which often develop in AIDS patients with low CD4 counts are usually less aggressive than in our patient. Certain neoplasia could be the cause of the lymphopenia. CD4 counts are usually more variable and lymphopenia of shorter duration in other etiologies.
The pathogenesis of venous leg ulcers is based on the leakage of fibrinogen leading to a pericapillary fibrin cuff and plugging of capillaries by white blood cells. On the basis of a previous work, we had assumed that the key event in the pathogenesis of venous leg ulcers is related to inflammation generated by activated white blood cells that accumulate under unrelieved blood pressure, because in ulcer biopsies we had detected the presence of tumor necrosis factor-alpha (TNF-alpha) in intracapillary monocytes, elastase in the polymorphonuclear leukocytes near the vessels, and a pericapillary undegraded fibrin cuff causing a diffusion barrier to oxygen. This concept was developed because TNF-alpha synthesized by activated monocytes is responsible for many deleterious effects. It has a potent mitogenic effect on fibroblasts, leading to new collagen deposition and angiogenesis, it induces an increase in collagenase production, it acts through upregulation of an intracellular adhesion molecule (ICAM-1), leading to leukocyte sequestration and consequently a release of toxic metabolites by the polymorphonuclear cells, an early step in chronic inflammation, it activates the coagulation pathway via a marked increase in monocyte-associated tissue factor (TF) procoagulant activity, and it inhibits fibrinolysis by promoting the release of PAI-1, contributing to undegraded fibrin deposition. Therefore, we were interested in evaluating, in patients with venous leg ulcers, the effect of pentoxifylline administered at 1,200 mg daily (versus placebo) for 2-months, as this drug induces a decrease in TNF-alpha synthesis and also blocks its activity. This pilot assay was performed in blind. Evolution of several parameters in ulcer biopsies are analyzed: TNF-alpha, intact fibrin, fibrin degradation products, ICAM-1, TF, and elastase. Pentoxifylline administration induced a decrease of local elastase and of fibrin deposit. These results support the hypothesis that accumulation of activated leukocytes is the key event in venous leg ulcers.
INTRODUCTION: Secondary myelodysplasia after antimitotic therapy is a rare complication usually observed with alkylating agents. The condition usually progresses to acute leukaemia with very poor short term prognosis. CASE REPORT: We report the cases of 2 women who developed myelodysplasia 2 and 9 months after treatment associating dacarbazine and fotemustine for visceral metastases of a malignant melanoma. DISCUSSION: The frequency of these rare complications is probably underestimated because of the rapid unfavourable outcome of metastatic malignant melanoma. We were unable to determine whether dacarbazine, fotemustine or their combination was incriminated in this complication. Risk could be reduced by carefully determining the cumulative doses of these antimitotics.
INTRODUCTION: We report the apparently first description of bullous Kaposi's sarcoma in a patient with AIDS. CASE REPORT: Kaposi's sarcoma was observed in a HIV positive homosexual who had developed AIDS. The delay between the development of Kaposi's sarcoma and the appearance of bullae was 6 months. Bullae occurred after oedema and necrosis of the subepidermic derma. DISCUSSION: This unusual clinical presentation of Kaposi's syndrome in an AIDS patient led to rapid tumor extention and would thus suggest a rapidly unfavourable prognosis.
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INTRODUCTION: Based on experimental data, HMG CoA reductase inhibitors are theoretically contraindicated in patients with porphyria. These new cholesterol lowering drugs are increasingly being prescribed. We report the first case of drug-related porphyria cutanea tarda due to HMG CoA reductase inhibitors. CASE REPORT: Porphyria cutanea tarda was diagnosed in a patient with chronic alcoholism 18 months after beginning a treatment with simvastatin followed by pravastatin. These drugs were given under different trade names and reintroduced successively leading to acute episodes. Total withdrawal of HMG CoA reductase inhibitors was followed by clinical normalization. COMMENTS: This case confirmed the theoretical contraindication for the use of HMG CoA reductase inhibitors in patients with porphyria. The risk of revealing porphyria with this type of cholesterol lowering drugs is emphasized. Since these drugs are used very frequently, similar cases may well be reported soon.
INTRODUCTION: We report an unusual complications of prolonged contact with white spirit leading to caustic burns. CASE REPORT: A 20-year-old patient was seen for caustic perianal burns several hours after ingesting one-half liter of white spirit in an attempted suicide. Motor diarrhoea also occurred quickly and a prolonged anal evacuation of white spirit. Complementary examinations did not reveal any lesions of the buccal, oesophageal, gastric or rectosigmoidal mucosa. DISCUSSION: Necrosis of the skin due to prolonged contact with petrol products is well known. Usually due to the delapidating action of dissolved petrol products on the superficial hydrolipid film and subjacent cell membranes. The absence of digestive tract lesions could be explained by the rapid onset of motor diarrhoea which may have limited the time of contact and thus protected the digestive tract mucosa which has a high content of mucopoly-saccharides and a low lipid content.
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Histiocytic cells infiltrating the lesions in eosinophilic granuloma of bone as well as in cutaneous histiocytosis X were studied using a murine monoclonal antibody (MA) produced with proliferating cells from an eosinophilic granuloma of bone. This MA reacts with Langerhans cells (LC) of normal human skin or mucous membranes and with proliferating cells of eosinophilic granuloma of bone and skin lesions of Letter-Siwe disease, as shown by immunohistochemistry and immunogold labelling. As other murine MA's obtained after immunization with human cortical thymocytes, this MA immunoprecipitates the 49-kDa CD1a antigen found on human LC and thymic-cell surfaces but not its breakdown product after treatment with trypsin, as demonstrated by analysis of immunoelectron labelling, cytofluorometry and gel electrophoresis. This first production of a CD1a MA from an eosinophilic granuloma supports the concept of Langerhans-cell histiocytosis.