[Facial rash in a pregnant woman].
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Biomedical subjects
Publications and source records attributed to C Picard-Dahan.
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BACKGROUND: Pemphigoid is a form of auto-immune bullous dermatosis characterised by the production of antibodies directed against components of hemidesmosomes in the basal membrane. The physiopathological process responsible for unmasking of these antigens is unknown. Pemphigoid is more common in elderly subjects and is most often seen in debilitated subjects. The prevalence of pemphigoid anti-pemphigoid antibodies (anti-PB) is not known in the elderly population presenting no dermatological signs evocative of the disease. We studied the prevalence of anti-PBAg2 antibodies in elderly subjects with no signs of pemphigoid as well as in the correlation between the presence of these antibodies and diagnosis of dementia. PATIENTS AND METHODS: Elderly subjects (aged over 69 years) with no signs of pemphigoid were recruited consecutively in dermatology and geriatrics departments (138 subjects). Details of concomitant medication were recorded for all subjects and clinical examination was performed with calculation of MMS (Mini Mental Score). The subjects were then divided into two groups based on MMS score. The first group comprised subjects without dementia (MMS > 24) while the second comprised subjects with dementia. Serum anti-PBAg2 antibodies were determined by ELISA and indirect immunofluorescence with confirmation by Western blot. Antinuclear antibodies, used as a control for non-specific immune response, were assayed in all serum samples. The prevalence of these antibodies was compared between the two groups. RESULTS: The two groups were comparable in terms of age, sex and presence of dermatological diseases (ulcers, bedsores, erysipelas). Each group comprised 69 subjects. The overall presence of anti-PBAg2 antibodies in subjects with no signs are suggestive of pemphigoid was 3.6%. Presence of anti-PBAg2 antibodies was associated with diagnosis of dementia (p=0.04; 0% and 7% in groups 1 and 2, respectively). No correlation was seen between the presence of anti-PBAg2 antibodies and concomitant medication or dermatological disease. The overall prevalence of antinuclear antibodies was 14.5% and the figure was similar between the two groups. DISCUSSION: The presence of anti-PBAg2 could be associated with the diagnosis of dementia in elderly subjects.
INTRODUCTION: Bullous pemphigoid usually affects elderly people. Only a few isolated cases among people younger than 65 years have been reported. OBJECTIVES: Describe the clinical and biological characteristics of patients younger than 60 years suffering from bullous pemphigoid, compare them with the usual characteristics known among elderly people and search for potential pathological associations. PATIENTS AND METHODS: Retrospective, national, multicenter study. Clinical, biological and histological characteristics were recorded with a standardised questionnaire as well as treatments and associated pathologies. RESULTS: Seventy-four cases of bullous pemphigoid diagnosed between June 1970 and March 2002 were analyzed. Mean age at the beginning of the disease was 46 +/- 11.6 years. Further explorations by indirect immunofluorescence of separated skin and/or immuno-electron microscopy and/or immunoblotting were performed for 42 patients (56.8 p. 100). Clinical characteristics among this restricted population were comparable to those found among the 32 other cases. Compared to usual data on bullous pemphigoid in elderly people, we observed a greater proportion of extensive form of disease (75 p. 100), a more frequent head and neck involvement (39.2 p. 100) and an overexpression of anti-BP180 autoantibodies (48 p. 100). Neoplasm was notified for 7 patients (9.5 p. 100), 18 (24.3 p. 100) suffered from a pathology of the basement membrane zone (6 psoriasis, 6 atopic dermatitis and 6 lichen) and 13 from neurological disease, among which 4 were bedridden. Fourty-six patients (62.2 p. 100) received drugs for the long term (mean 2.12 +/- 2.43), 4 patients were treated by PUVAtherapy and 2 by radiotherapy. DISCUSSION: Our results suggest that bullous pemphigoid among young people is more severe and more active than the usual form in the elderly. This particular form could be the result of a higher expression of anti-BP180 autoantibodies, which are considered as a marker of poor prognosis in this disease. We also found a high frequency of pathological associations and physical treatment, all responsible for damage to the basement membrane zone, which can involve auto-immunization against hemidesmosome components.
BACKGROUND: Kaposi's sarcoma (KS) is a potentially life-threatening multifocal neoplasm. Despite the significant decline in the incidence of acquired immune deficiency syndrome (AIDS)-related KS with the use of highly active antiretroviral therapy (HAART), some patients, even those with a good immune restoration, still have aggressive disease. Liposomal anthracyclines or combination chemotherapy are widely used but adverse effects limit their utilization. OBJECTIVES: We studied the efficacy and tolerance of docetaxel in the treatment of AIDS-related KS after pretreatment with anthracycline. PATIENTS/METHODS AND MAIN OUTCOME MEASURE: A retrospective cohort study was done. Nine human immunodeficiency virus (HIV)-infected patients were treated from 1997 to 2002 with docetaxel. Tumour response was evaluated using the AIDS Clinical Trial Group (ACTG) staging criteria. Clinical and biological toxicity was evaluated. AIDS status with HIV viral load and CD4 T-cell count were measured at the beginning and at the end of the treatment. RESULTS: A major (complete or partial) response and a stabilization of the disease were demonstrated in seven and two patients, respectively. Grade 4 neutropenia and thrombocytopenia were observed in four of nine and one of nine patients, respectively. One patient died after sepsis. CONCLUSIONS: Docetaxel has a good and rapid efficacy in anthracycline-pretreated patients with severe AIDS-related KS. Phase II/III trials should be done to compare docetaxel with liposomal anthracyclines as a first-line treatment.
INTRODUCTION: Xanthoma disseminatum is a non-Langerhans histiocyte proliferation, described by Montgomery in 1938. This rare entity is characterized by skin and mucous membrane xanthomatosis, associated with diabetes insipidus and normal lipid metabolism. In this case report, vinblastine produced the regression of the lesions. OBSERVATION: A 51 year-old man presented in 1999 with a four-year history of progressive xanthomatous papulonodular lesions of his trunk, axillary and inguinal folds, neck and face (leonin facies). Treatment with thalidomide for 6 months was ineffective. A rapid extension of the lesions to the pharynx, larynx and trachea with dyspnea occurred and required a tracheotomy. Histopathological study showed a dense histiocytic infiltrate within the upper and mid dermis with Touton giant cells and inflammatory cells. The histiocyte cells were positive for CD68. Neither lungs nor hypophysis were involved. A treatment with 6 cures of cyclophosphamide was insufficient. Vinblastine therapy (32 cycles: 0.1 mg/kg/cycle) produced a spectacular regression of the mucous-cutaneous papulonodular lesions leaving cheloid scars. The patient required a transitory tracheotomy and a bilateral commissuroplasty. DISCUSSION: Prognosis of xanthoma disseminatum is in related to the mucous membrane manifestations (50 p. 100 of cases) and involvement of the upper respiratory tract. The response to any form of therapy in xanthoma disseminatum is unsatisfactory. Surgical excision or laser therapy can improve physical and functional aspects but the evolution is characterized by very frequent relapses. Treatment with antimitotic drugs seems to be ineffective in many cases. In our patient, vinblastine induced a spectacular regression of mucocutaneous lesions without neurologic toxicity. To our knowledge, this is the first report of vinblastine efficacy in this rare and severe disorder.
INTRODUCTION: The interest of intravenous immunoglobulins (Igi.v.) in the treatment of pemphigus is discussed. Pemphigus is not a recognized indication for this treatment by the CEDIT (French Committee for the assessment and diffusion of technological innovations). The aim of this study was to assess the efficacy of Igi.v. in the treatment of severe corticosteroid-dependent or resistant pemphigus. MATERIAL AND METHODS: A retrospective study using a standardized questionnaire was conducted in the various departments of dermatology among the "Groupe Bulles" of the French Society of Dermatology. The study collected the following information from 12 patients suffering from pemphigus and treated with Igi.v.: 1) general demographical data; 2) characteristics of the pemphigus; 3) different treatments applied, and 4) efficacy and side effects of treatments. RESULTS: Among the 12 patients studied at the end of treatment with Igi.v., 8 were in complete remission (1 fleetingly for 2 months and 1 preceding initiation of Igi.v.), and 2 were improved (1 temporarily for 4 months). A reduction in corticosteroid therapy was possible in 75 p. 100 of cases (9 patients). During treatment with Igi.v., immunosuppressors were combined with oral corticosteroids in 3 cases. It was possible to reduce their dose 1 one case and to stop them in another case. No major side effect related to treatment with Igi.v. was observed. Six months and one year after the treatment, complete remission rates were respectively 6/10 and 5/8. One patient relapsed more than one year after the end of Igi.v. treatment. CONCLUSIONS: Although very expensive, treatment with Igi.v. appears of interest in the treatment of severe corticosteroid-dependent or resistant pemphigus. Moreover tolerance is excellent. The results of our study warrant confirmation in a prospective study.
INTRODUCTION: Interferon alpha has many side effects. Among them the risk of occurrence of seizures is not well known by dermatologists. We report three cases of seizures that occurred in patients treated with interferon alpha in two dermatological diseases: mycosis fungoides and melanoma. OBSERVATIONS: A 68 year-old man, treated for mycosis fungoides, and two men aged 47 and 52 years, treated for melanoma, were under interferon alpha. After 11 months, 3 weeks and 9.5 months, respectively, the three patients had seizures without any past history of epilepsy. Anamnesis and assessment of each patient (brain CT, biological results) suggested the responsibility of interferon alpha. After withdrawal of the treatment, no relapse was observed after 3 months, 6 months and 1 year later, respectively. DISCUSSION: Seizures during treatment with interferon alpha have already been reported. According to the series their prevalence would be of 1 to 4 p. 100. Their pathophysiology is not well known, but apparently interferon alpha lowers the epileptogenic threshold by affecting the central nervous system either directly or through cytokines or neuromediators. The risk of occurrence of seizures must be known by the prescribing physician who must systematically search for past history of epilepsy or risk factors for seizures. This rare but existing side effect raises the problem of information to be supplied to the patient by the prescribing physician.
INTRODUCTION: Adult dermatomyositis is associated with cancer in 15 p. 100 to 50 p. 100 of cases and, hence, investigations should be systematically performed to search for cancer. A number of predictive factors have been reported. The aim of our study was to search for predictive factors of cancer, among adults with dermatomyositis. METHODS: We prospectively assessed 26 adults presenting with dermatomyositis, hospitalised in our department of dermatology from January 1993 to June 2000. The parameters assessed were: association with a cancer, age, gender, cutaneous necrosis, muscular weakness, electromyographic abnormalities, erythrocyte sedimentation rate, and muscular enzyme levels. RESULTS: Mean age was of 52 years and sex ratio (M/F) was of 0.53. Cancers were diagnosed in eight cases (31 p. 100) (mean age: 59.5 years; sex ratio=1; cancer localization: lung (2), breast (2), ovary, endometrium, bladder, and melanoma). Five patients in the cancer group had cutaneous necrosis and only 2 in the without cancer (p=0.01; PPV=71.4 p.100). Elevation of muscular enzyme was also associated with cancer. CONCLUSION: Our report demonstrates that cutaneous necrosis is closely associated with cancer and it suggests that in selected patients with dermatomyositis and cutaneous necrosis, more exhaustive and repeated investigations should be performed to search for cancer. The interest of elevation in muscular enzyme as a predictive factor of cancer is discussed.
INTRODUCTION: Diagnosis of retroperitoneal fibrosis is generally delayed and revealed by various non-specific signs. We report the case of an isolated lymphedema of the lower limb revealing retroperitoneal fibrosis complicating a metastatic squamous cell carcinoma. CASE REPORT: In an 83-year-old women, a lymphedema appeared that remained isolated for several months before being associated with alteration in general health. Morphological examinations showed bilateral compression of the urinary excretory tracts and led to the diagnosis of retroperitoneal fibrosis. Histological examination of a sub-clavicular adenopathy that had evolved over 9 months, confirmed the diagnosis of a metastatic squamous cell carcinoma of pulmonary cancer. DISCUSSION: Retroperitoneal fibrosis is an exceptional etiology that must be recognized in isolated lymphadomas of the lower limbs. In view of the possible tumoral origin of retroperitoneal fibrosis, any evocative sign accompanying the lymphedema must be searched for.
BACKGROUND: Trichosporon beigelii, causal agent of white piedra can cause disseminated infection in immunodepressed subjects. Systemic infections due to this pathogen have been reported mainly in neutropenic patients and rarely in AIDS patients. CASE REPORT: A 36-year-old HIV+ man from Senegal was hospitalized for fever and meningoencephalitis associated with skin lesions. T. beigelii was isolated from skin biopsies and cerebrospinal fluid cultures. The patients was treated with amphotericin B with regression of the skin lesions. The diagnosis of disseminated T. beigelii infection was retained. DISCUSSION: Disseminated T. beigelii infections are known to occur in immunodepressed subjects, especially in case of neutropenia. In our patient, the presence of two proven localizations (meninges and skin) and the favorable outcome with amphotericin B favored disseminated infection. The good response to treatment can probably be explained by the absence of neutropenia. Skin lesions are frequent, usually occurring as disseminated papulae or purpural nodules. Pathology examination and skin biopsy culture can provide rapid diagnosis allowing appropriate treatment.
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UNLABELLED: Bullous pemphigoid is the most frequent autoimmune blistering disease. It occurs in the elderly. The cause of this disease is unknown, but cases of bullous pemphigoid have been occasionally attributed to drug therapy. We conducted a multicenter prospective case-control study looking at the drugs used on a long-term basis before the onset of the disease in 116 incident cases of bullous pemphigoid and 216 control patients with malignant or benign skin tumors. RESULTS: Case patients and control patients received many drugs on a long-term basis (mean +/-SD, 4.4+/-3.2 and 4.4+/-2.7 years respectively). Two classes of drugs, neuroleptics and diuretics, were used more frequently by case patients than control patients. Neuroleptics were used by 15.5% of case patients and 8.3% of control patients (adjusted odds ratios; 1.9; 95% confidence interval, 0.95 to 3.8). Diuretics were used by 36.2% of case patients and 24.5% of control patients (adjusted odds ratios, 1.8; 95% confidence interval, 1.1 to 2.9). Among neuroleptics, no specific drug emerged. The association with diuretics was only linked to aldosterone antagonists, which were taken by 12.9% of case patients and 4.6% of control patients (adjusted odds ratios, 3.1; 95% confidence interval, 1.4 to 7.1). CONCLUSIONS: These results suggest that some drug therapies may be a risk factor for bullous pemphigoid. The cause of this association should be further investigated.
INTRODUCTION: We report five cases of pigmented erythroderma occurring during AIDS, noteworthy for its unusual hyperpigmented feature, its advent at the terminal stages of AIDS, and an CD8 cells dermal infiltrate. PATIENTS AND METHODS: It is a retrospective study of five patients infected with HIV: a woman infected by transfusion and four homosexual men, average 55 years old. No one was intravenous drug user. They were all severely immunocompromised; HTLV I/II serology was negative. Skin biopsies were studied with light microscopy (Hematoxylin-eosin) and immunohistochemical studies were performed on frozen sections. RESULTS: The patients had an erythroderma of particular interest because of the associated hyperpigmentation, the severe repercussion (pruritus, weight loss), and the difficulty in treating (except systemic corticosteroids). The histology demonstrated a mononuclear dermal lymphocytic infiltrate, without epidermotropism and atypical cytonuclear feature. The phenotype of the infiltrate was uniformly of the suppressor-cytotoxic subset (CD8+, CD4-). COMMENTS: Our cases are like those previously described as "Pseudo-Sezary", mimicking a lymphoma during AIDS. Numerous factors are probably the cause of this hyperpigmented erythroderma: HIV, CD8 cells... CONCLUSIONS: This severe skin disease, complicating AIDS, seems very particular, but not yet clearly defined. In practice, the problem remains the treatment of this severe erythroderma, because only the systemic corticosteroids are effective, but this is debatable during the treatment of AIDS.
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Bacillary angiomatosis (BA) is a recently described infection usually found in patients with human immunodeficiency virus disease. BA is caused by a Gram-negative coccobacillus. This organism is primarily responsible for skin lesions of the pseudo-botryomycoma type or inflammatory nodules, but it also produces fever, degradation of the general condition and visceral lesions involving the lymph nodes, the liver, the spleen and the bones. Histology shows vascular proliferation with turgid endothelial cells and mostly neutrophilic inflammatory infiltrates. BA is susceptible to many antibiotics. The authors describe the history of the disease and its clinical and histological features, discuss its differential diagnosis and principally deal with the relationship between BA and cat-scratch disease and between BA and verruca peruana. They also present the molecular biology technique which enables a genotypic diagnosis of the disease to be made, replacing a deficient phenotype.
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