[Dowling-Meara epidermolysis bullosa. Neonatal presentation].
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Biomedical subjects
Publications and source records attributed to C Lok.
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INTRODUCTION: The perforating granuloma annulare is a rare form of granuloma annulare. The clinical diagnosis is difficult and the confirmation is histological. The localisation is unique in less than 10% of all cases. We report a documented case with a histological suspicion of transfollicular perforation. OBSERVATION: A 36 year-old woman, without any particular antecedent, presented on her upper arm a single ulcerated nodular lesion that had evolved for one year. The clinical examination and biological investigations were normal. The histological examination led to the diagnosis of perforating granuloma annulare with a large epidermic ulceration. Treatment with a topical corticosteroid was disappointing and the removal was decided. DISCUSSION: In our observation, the single localization of this lesion raises the problem of differential diagnosis such as cutaneous tuberculosis, atypical mycobacteriosis, skin sarcoidosis, foreign body granuloma, epidermoid carcinoma or perforating dermatitis. The histological examination permitted diagnosis of a perforating granuloma annulare with large epidermic ulceration. The infiltration and destruction of a hair follicle evoked the possible transfollicular elimination of the necrotic material. Other pathologies with the histological aspect of a palisading granuloma were excluded from this context. No associated pathology such as diabetes nor any other favouring factors such as ultraviolet light or insect bites or traumas were identified. The physiopathology of perforating granuloma annulare and the process of perforation remain unknown. Numerous therapies have been proposed with variable results.
BACKGROUND: We recently carried out a study concerning consultations by French dermatologists in private practice. We evaluated consultations at the dermatology departments of 3 university teaching hospitals in France. MATERIALS AND METHODS: This was a 2-month prospective study conducted in 2003 at the dermatology departments of the university teaching hospitals of Amiens, Lille and Rouen. Each consultant completed a questionnaire covering the duration of the study. The following data were recorded: consultation date, function of the consultant, study centre, type of consultation, type of disease, and whether or not the patient was hospitalised after the consultation. RESULTS: 7296 files were examined during the study. 38% of the consultations were performed by part-time hospital consultants, 29% by dermatology interns, 18% by hospital practitioners, 9% by university professors and 6% by clinical heads or assistant heads. The most commonly encountered diseases were allergies (17%), cancer (16%), arteriovenous disease (15%) and infectious disease (11%). Three types of consultation were identified: emergency consultations without an appointment, consultations by appointment for a specific problem and consultations by appointment without a specific problem. The number of resulting hospital admissions ranged from 2 to 10% of consultations, depending on the type of consultation and the role of the consultant in question. DISCUSSION: This study shows that in France, consultations at hospital dermatology departments differ greatly from those of dermatologists in private practice. The main diseases seen (cancer, arteriovenous disease, allergy, infectious dermatosis) accounted for the majority of hospitalisations in these departments. The organisation of hospital consultations is increasingly tending both towards treatment of highly specialised diseases through specifically oriented consultations and also towards the emergency treatment of certain forms of acute dermatosis.
BACKGROUND: Imatinib (Gleevec) is a tyrosine kinase inhibitor used to treat chronic myeloid leukemia. We describe a case of drug reaction with eosinophilia and systemic symptoms (DRESS) after institution of treatment with imatinib. PATIENT AND METHODS: A 78-year-old woman was treated with low-dose imatinib for chronic myeloid leukemia since November 2003. A macular and pruritic eruption appeared on the patient's trunk after 7 weeks of treatment and gradually worsened. After 1 month, she was admitted for generalized skin eruption with fever and diffuse lymphadenopathy. Laboratory data showed hypereosinophilia and blood cultures positive for Staphylococcus aureus. Imatinib was stopped and replaced with hydroxyurea (Hydrea). Improved clinical and laboratory results were seen with antibiotics and topical steroids. DISCUSSION: To our knowledge, this is the first case of DRESS following treatment with imatinib. Cutaneous reactions to imatinib are frequent and are usually mild, comprising maculopapular eruption, pruritus and facial edema. Few cases of serious skin reactions have been reported until now. Several authors suggest that the prevalence and severity of cutaneous manifestations are related to a pharmacologic effect of imatinib. Our observation cannot rule out an underlying immunologic mechanism. Septicemia may also play a part in the development of DRESS.
BACKGROUND: Pigmented bullous pemphigoid has many clinical manifestations. We report a pigmented erythematous form with disseminated bullae. CASE REPORT: A 70-year-old woman with a history of breast adenocarcinoma developed an eruption of pigmented macules on the trunk and members of 72 hour duration. At five days, there was an eruption of tight bullae. The diagnosis of bullous pemphigoid was retained because of the association of infraepidermic bullae, linear deposits of C3 along the basal membrane and the presence of the 180 kDa minor bullous pemphigoid antigen on immunoblotting. DISCUSSION: The initial pigmented aspect of this bullous pemphigoid suggested disseminated pigmented bullous erythema, but histology data with immunotransfer corrected the diagnosis. This very atypical presentation led us to look for a particular etiology. There was no argument in favor of a malignancy in this patient in complete remission after treatment of her breast adenocarcinoma. The fact that the patient was phototype IV would probably explain the pigmented nature of the initial lesions.
INTRODUCTION: Differential infrared thermography, proposed in this paper, is a technique based on direct observations of infrared radiations emitted by the skin. The evolution of cutaneous temperature caused by the application of dermocorticoids on healthy skin demonstrates their pharmaco-dynamic properties. MATERIALS AND METHODS: The cutaneous thermal image was recorded in real time. Image processing using subtraction function readily provided differential infrared thermographic analysis of the effects. Four activity classes of dermocorticoids had been applied on healthy skin. A test immediately carried out after dermocorticoid application on skin without occlusion and the classical skin blanching-test have been performed. RESULTS: Temperature differences between the dermocorticoids were detected within the first three hours after the application on the skin without occlusion. The dermocorticoid class II cream formulation under study induced a decrease in temperature more pronounced than the others dermocorticoids. The skin-blanching effect was more noticeable for dermocorticoids class I and II and it was not detected for class IV. DISCUSSION: Subtraction thermograms provide a means of differential imaging. Evolutive temperature differences subsequent to unique application without dermocorticoid occlusion are evidenced during a short duration (the first three hours). This may correspond to efficacy differences while classical tests of vasoconstriction analyse the cutaneous blanching induced after the 6th hour. Concerning the skin-blanching effect, results of this first investigation are not sufficient for a precise qualitative and quantitative interpretation. The main interest of differential infrared thermography is to be quantitative, without contact, continuous in real time. Differential infrared thermography is more sensitive than classical thermography. It allowed an objective evolution survey for dermocorticoids.
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Between 1981 and 1984, 72 patients (22-75 years) were included in a prospective study for treatment of Polyarteritis Nodosa (PN) which associated therapeutic plasma exchanges (TPE), corticosteroids (CS) 1 mg/kg/day, and a randomized trial of cyclophosphamide (CP) 2 mg/kg/day. We observed the incidence and analysed side effects (SE) occurring during TPE considering the technology of plasma removal. Full data were available for 63 patients. Seven hundred and eighteen TPE were performed. Centrifugation was used in 594 PE (82.7 per cent) with intermittent flow centrifugation (IFC) 320 times (44.5 per cent) and continuous flow centrifugation (CFC) 274 times (38.2 per cent). Filtration (F) was used in 124 sessions (17.3 per cent). Replacement fluid was 4 per cent albumin in 650 TPE and fresh frozen plasma (FFP) in 89 TPE. A total of two hundred and eleven SE were reported in 53 patients (84.1 per cent) during 173 TPE (24.1 per cent). Forty-six TPE were temporarily stopped because of SE (6.4 per cent). The mean volumes of removed plasma (ml/kg/TPE) were: 60.7 +/- 8.7 ml/kg when CFC was used 54.5 +/- 20 ml/kg with F and 52.5 +/- 9.6 ml/kg with IFC (n.s.) Main SE were: technical difficulties in 80 TPE, moderate or severe hypotension in 47, allergy to replacement fluid in 39. Hepatitis B antigen appeared in one patient. No death occurred during TPE and SE were usually minor and transient.(ABSTRACT TRUNCATED AT 250 WORDS)