[Autoimmune bullous diseases and HHV6: retrospective evaluation of active viral replication].
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Biomedical subjects
Publications and source records attributed to J Chevrant-Breton.
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INTRODUCTION: Spontaneous skin necrosis revealed acquired protein S deficiency due to isotype G autoantibodies. CASE: This 31-year-old male renal transplant recipient, receiving immunosuppressive treatment, was hospitalized for necrotic purpural lesions. We were not able to detect any triggering factor. Sustained anticoagulant therapy remained essential to prevent new skin lesions and perhaps more thrombotic events. COMMENTS: This condition is rare in adulthood, but is well described in children's purpura fulminans, especially the post-varicella form. Its mechanism remains unclear.
UNLABELLED: In children, chronic cervicofacial ulceration related to dental infection is rare. Thus the diagnosis is often late and the treatment is consequently delayed. We report 2 new cases. CASES REPORT: A 13-year-old boy presented with a 1-year history of chronic and suppurative ulceration on the right cheek. Culture was positive for actinomycetes. In spite of a prolonged and miscellaneous antibiotherapy, the lesion recured. The ulceration healed after the eradication of infection on a right superior molar. A 12-year-old girl presented with a right sub-mandibular ulceration, which appeared 3 months before. This lesion did not respond to penicillinotherapy given during 3 months. An infection on a right inferior molar was diagnosed on a tomodensitometry. 3 months after the tooth extraction, the ulceration healed without recurrence. CONCLUSION: These cases emphasize the interest to look for a dental infection at the origin of chronic cervicofacial lesion.
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BACKGROUND: Reticular erythematous mucinosis (REM) syndrome is a rare disorder. Its clinical course is cyclic with remissions and exacerbations. In this disease, photosensitivity has previously been noticed but rarely demonstrated. We report three new cases with positive photobiological investigation. CASE REPORTS: Three patients (two males, one female) with a mean age 47 years were seen with reticular erythematous papules on the upper chest and or back. After sun exposure, the lesions were exacerbated. Skin biopsies showed dermal lymphocytic perivascular infiltration with mucin deposition between collagen bundles. Direct immunofluorescence was negative. Antinuclear antibodies were absent. In cabin, ultraviolet (UV)A exposure reproduced clinically and histologically REM lesions in our cases. UVA and UVB provocating phototests were negative. In all patients treatment with oral antimalarials and external photoprotection was effective. CONCLUSIONS: In our patients, we confirm the photosensitive feature of REM syndrome by provocative irradiation in UVA cabin. The mechanism of triggering is actually unclear. It is supposed that UV radiation, heat, and perspiration are necessary to reveal this affection.
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BACKGROUND: Jessner's lymphocytic infiltration of the skin is a rare and benign disorder. Its clinical course is cyclic with remissions and exacerbations. In this disease, photosensitivity was previously noticed by authors and recently demonstrated. We report four new cases with positive photobiological investigation. CASE REPORTS: Four patients (2 males, 2 females), with a mean age 36 years were seen with erythematous papules or discoid plaques on face, arms and upper trunk. The onset of disease occurred after sun exposure in summer. Skin biopsies showed perivascular lymphocytic infiltration in the dermis. All patients relapsed cyclically with incomplete healing during winter. Photobiological investigation elicited skin lesions in a broad spectrum of UV: UVB (2 cases), UVA (1 case), UVA and UVB (1 case). In all patients treatment with oral antimalarials and external photoprotection was effective. DISCUSSION: Our data suggest that photosensitivity history in patients with Jessner's lymphocytic infiltration of the skin should be searched for, and confirmed by provocative phototesting. This relevant event could guide the therapeutic strategy because antimalarials were effective for the Jessner's lymphocytic infiltration cases with photosensitivity.
A prospective survey was conducted to assess physician responsibility in melanoma prognosis. Consecutive patients with primary melanoma were interviewed and examined using a standardized questionnaire. Main outcome measures were medical components of the delay before tumor resection and tumor thickness. Of 590 melanomas, 29.1% were coincidentally detected by physicians and their tumor depth was lower than in melanomas detected by patients (p < 0.001). Physician sensitivity for melanoma diagnosis was evaluated at 86%. Median time intervals to propose resection and to perform removal of melanoma were short: 0 (mean 103) and 7 (mean 68) days, respectively. Melanomas were managed in an inappropriate way in 14.2% of cases. Location on acral areas and absence of pigmentation were associated with longer medical delays and more frequent inappropriate medical attitudes. Melanomas located on hardly visible areas were less frequently detected by physicians than those on visible areas. Medical delays were shorter, doctor's attitude was more frequently appropriate, and melanoma thickness was lower (p < 0.001) when the patient visited a dermatologist (54.7%) than when he or she visited a general practitioner (33.4%). Our study shows that doctor responsibility accounts for only a small part of the total delay before melanoma removal. However, systematic total examination and better training of doctors, especially about unusual forms of melanoma, could still improve melanoma detection.
A prospective survey was conducted to assess the role of patients in the melanoma prognosis. Consecutive patients with primary melanoma were interviewed and examined using a comprehensive questionnaire including a psychological instrument. Main outcome measures were the delay before medical intervention and the tumor thickness. Of 590 melanomas, 70.8% were detected by patients and this proportion was higher in females. Relatives were involved in the detection of half of the cases. Median delays before the patient realized he had a suspicious lesion, before this lesion was seen by a doctor, and before the melanoma was removed were 4 months, 2 months, and 1 week, respectively. Delays up to several years were observed in some cases. The rate of self-detection tended to be lower, the delays before seeking medical advice to be longer, and the tumor thickness to be higher in old people, in males, in lower-educated individuals, in those living out of towns, and in people with a low awareness about melanocytic tumors than in other cases. Conversely, individuals with a high number of atypical nevi, those who were aware to be at risk, and those who regularly visited a dermatologist tended to detect their melanoma more rapidly. No specific psychological traits were associated with a late reaction, although negligence and anxiety tended to prolong the delays. Knowledge about melanoma was poor in many patients, especially in males, and wrong beliefs were widespread. This study provides the targets of future education programs.
OBJECTIVE: We searched for a correlation between serum S100B protein and cutaneous malignant melanoma stage, according to the American Joint Committee on Cancer staging system, and between elevation of serum S100B protein and development of metastasis. PATIENTS AND METHODS: We conducted a prospective bicentric study for 20 months in 122 patients with malignant melanoma. LIA-mat(R) assay was used to determine serum S100B at each examination. The optimal cut-off value was determined from the ROC curve. RESULTS: The optimal cut-off value to discriminate patients with metastases from patients in remission was 0.09 microg/l. Sensitivity was 46 p. 100 in patients with stage III and 86 p. 100 in patients with stage IV disease. The positive predictive value for stage III/IV was 77 p. 100 and the negative predictive value was 89 p. 100. Serial measurements were made in 56 patients. The serum S100B protein level was elevated in 69 p. 100 of the patients disclosing disease progression (9/13). In 44 p. 100 of the patients (4/9), serum S100B protein level rose within a delay of 3 months before new metastases were detected. DISCUSSION: Our study confirms that serum S100B protein is a tumor marker for melanoma staging and follow-up. A rise in S100B protein precedes or reveals metastasis.
OBJECTIVE: The incidence of cutaneous melanoma has rapidly increased in the white population over the last decades. It has been estimated that the incidence doubles world-wide every 10 years. Different risk factors have been identified, including immunosuppression. The aim of our study-was to determine the relative risk of developing melanoma in the organ transplant population and the clinical and histological features of their melanomas. PATIENTS AND METHODS: This retrospective study was conducted with the collaboration of 9 University Hospital Centers: Besançon, Brest, Caen, Dijon, Lille, Lyon, Nantes, Paris (Pitié-Salpétrière) and Rennes. A questionnaire was sent to the different departments of dermatology of these hospitals to obtain information on patients who had presented a melanoma after a transplantation between 1971 and 1997. During this period, there were 12,477 organ transplant recipients in the transplantation units of these 9 hospitals. Average follow-up for these patients was about 5 years and the average duration of immunosuppressive therapy was about 4.5 years. RESULTS: Among 12,477 organ transplant recipients, we found 17 cases of melanoma but no data could be obtain on one case: 14 occurred in renal transplant recipients and 3 in cardiac transplant recipients. Clinical and histological data were only available in 16 patients. The average time between transplantation and diagnosis of melanoma was 63 months, but it was 5 times shorter for 2 patients who had a past history of melanoma before transplantation. Two patients had a mucosal melanoma; for the cutaneous melanomas, 2 appeared on Dubreuilh melanosis, 2 were in situ melanomas, 7 were superficial spreading melanomas and 3 were nodular melanomas. The histological review of 11 cutaneous melanomas revealed a precursor nevus in 6 cases and a weak or no stroma reaction in 7/7 cases. Complete excision of the melanoma was performed in all patients except one with anorectal melanoma. Four patients died of visceral metastasis within a mean 15 months. The other 12 patients are still alive with a mean 3 year course since tumor treatment. We tried to determine the relative risk of developing melanoma in the renal transplant population (14 cases). The number of expected cases of melanoma was 5.54, giving a relative risk of 2.5. DISCUSSION: Only 4 studies have shown an increase in the incidence of melanoma in the renal transplant population: approximately 2 to 5-fold. In our study, the 2.5-fold increase in melanoma was estimated with an average 5 year follow-up and an average 5 year immunosuppressive therapy. This is probably an underestimation of risk because we were unable to make an exhaustive collection of cases of melanomas even though transplant recipients undergo more physical examinations than a reference population. The mean latency period from transplantation to melanoma diagnosis was 63 months, as in other studies. Histological examination showed that a precursor nevus is frequent with weak host cellular response to the tumor. The prognosis of these melanomas remains difficult to predict, but in our study, it would not appear to be as poor as expected. Discontinuation of immunosuppressive therapy would not appear to be necessary except in the presence of metastasis. Finally, our study demonstrates the importance of good patient follow-up, even after graft rejection due to the persistent risk of melanoma.
BACKGROUND: Orf is a disease of sheep due to a parapoxvirus. Transmission in man is possible, and is generally benign, self-limited condition. Although, rare complications have been mentioned. We report a case of Orf with erythema multiforme in a young girl. CASE REPORT: A 13-year-old girl presented to our department with an erythema multiform. The cutaneous examination revealed painless erythematous nodule of her right index finger developed 15 days after a contact with a lamb. The diagnosis of Orf complicated by erythema multiforme was made on the basis of her typical history. Lesions heal with symptomatic therapy in 15 days. DISCUSSION: Orf presents in sheeps as a pustular dermatitis or a vesicular oral mucosal lesions, and in man as a single lesion on the site of infection. This lesion generally heals without complications, although secondary infection is not uncommon. Twenty one cases of erythema multiforme have been recorded following infection with the Orf virus. In typical cases, the diagnosis is established by a history of contact with infected animals and the appearance of the lesion. Electron microscopic examination can be helpful sometimes. Lesions regress in 4 to 5 weeks without specific treatment.
OBJECTIVE: To test the basic assumption of campaigns for early diagnosis of melanoma, ie, prognosis is correlated with the delay in the diagnosis. DESIGN: Prospective study of the correlation between delays to diagnosis, assessed using a questionnaire, and the Breslow thickness as a prognosis marker. SETTING: Dermatology departments in France. PATIENTS: Five hundred ninety consecutive patients, referred within 12 weeks after resection of cutaneous melanoma. MAIN OUTCOME MEASURES: Assessment of 5 successive time intervals from the first time the patients realized that they had a lesion until the resection of the melanoma, and results of the correlation between each time interval and tumor thickness (Breslow). RESULTS: There is a positive but weak correlation between tumor thickness and the delay to identify a lesion as suspicious (r = 0.17; P = .009). However, this delay tends to be short for the thickest tumors. There is a negative correlation between tumor thickness and the delay to seek medical attention (r = -0.20; P<.001). This delay was shorter for nodular melanoma. No correlation is found between melanoma thickness and physicians' delays. CONCLUSIONS: Poor prognosis can be accounted for by aggressive rapidly growing tumors rather than by delays. In well-informed populations, campaigns for early diagnosis of melanoma may thus no longer have a major impact on prognosis, unless they are focused on subgroups less accessible to information and medical care.
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