成人Still病中持续存在的色素性斑块(法国)

Introduction. Adult- onset Still’ s disease (AOSD) is a systemic affection characterized by intermittent fever, evanescent rash, polyarthralgia or arthritis and neutrophilic leucokytosis. We report a new case of AOSD with persistent pigmented plaques in a Vietnamese woman. Case report. A23 year- old woman had presented within several months: inflammatory polyarthralgia, intermittent fever, asthenia, myalgia, weigh loss, polyadenopathy and an evanescent cutaneous rash. In addition, she presented pigmented skin plaques on the trunk. The investigations noted: neutrophilia (10000/mm3) and a high level of serum ferritin (42 000 μ g/l). The erythrocyte sedimentation rate was 55mm in the first hour. C reactive protein was normal. HIV and syphilis serologies, antinuclear antibodies and rheumatoid factor were negative. No growth of micro- organisms was observed in repeated blood and urine cultures. The histology of the persistent plaque and lymph node were not specific. It required a high dose of corticosteroid to obtain remission. Discussion. The existence of persistent plaques in AOSD is uncommon. Nevertheless, the exact relationship between the fixed cutaneous lesions and AOSD remains unclear. But their synchronous evolution with systemic symptoms and response to therapy suggest that they represent a specific manifestation of the disease.

伊维菌素成功治疗1例HIV感染患者的螨皮炎

Introduction. Demodex is a saprophyte parasite in mammals. In Man, it is associated with differing clinical profiles (rosacealike dermatitis, folliculitis and blepharitis). We report a case of demodecidosis in an HIV-infected patient that was successfully treated with ivermectin. Case report. A man from Laos, infected by HIV and treated for glandular tuberculosis, presented with a prurigenous eruption on the face and the pre-sternal and interscapular areas. Direct examination of scraped product and histopathological examinations confirmed the diagnosis of demodecidosis. Clinical cure was obtained after 2 single cures of ivermectin a one month’s distance. Discussion. The features of demodecidosis are often similar to those of rosacea. In immunodeficient patients, the semiology remains the same but the eruption is more abundant. During HIV-infection, demodecidosis occurs at the AIDS stage or with a CD4 count lower than 200/mm3. Many anti-dust mite molecules are used to treat the disease but frequently lead to irritation. Administration of a single cure of ivermectin, repeated if necessary, appears to be an interesting alternative to contact anti-dust mite agents.

下肢局部溃疡治疗的知识和问题:对Indre-er-Loire地区全科医师的调查(法语)

Introduction. The quantity of topical treatments for leg ulcers has increased over the last 15 years. Objective. To determine the prescriptions for topical tr eatments and the problems in the management of leg ulcers using a questionnaire sent to general practitioners. Material and methods. A questionnaire in 3 parts was sent to 95 general practitioners in the area working in 36 different distric ts with an explanatory letter. The first part was composed of 29 closed question s regarding prescription of topical treatment for leg ulcers. The second part, a lso closed, included 3 pictures showing a budding, a necrotic and a fibrinous ul cer. The physicians had to choose which treatmenttheywouldhaveprescribedandthefr equencyofdressings changes. The third part was composed of two open questions re garding the problems encountered. The survey started on May 1st and was closed o n June 30th 2002. No reminders were sent. Results. We received 52 replies, 49 of which were exploitable. Forty-five percent (43/95) did not reply. Occlusive dr essings were widely prescribed (mainly hydrocolloid 38/49 and hydrocellular 28/4 9). Vaseline impregnated gauzewere less prescribed (14/49). Dressings with balsa m of Peru were prescribed often by 10 and occasionally by 27 general practitione rs. Alginate or charcoal dressings were not always used appropriately. Antisepti cs were prescribed by 10 physicians. Mechanical debridement of fibrinous or necr otic wounds was rarely used even for necrotic ulcers. Topical anesthetics were p rescribed in 21 out of 33 cases of mechanical debridement. Some physicians refer red care (n=4), others complained about lack of compliance (n=15), cost (n=14), local intolerance (n=10) and the excessively wide variety of dressings (n=10). W e drew-up a synopsis with guidelines for treatment, which was sent to all the g eneral practitioners who had participated in the survey. Conclusion. Forty-thre e of the 95 physicians approached did not reply. This poor response rate, which is common in this type of survey, may include physicians who are not at ease in the management of ulcers and this may bias the results. The physicians who repli ed to the survey knew the subject well but complained of the costs and variety o f dressings. They were all eager to receive further information and guidelines.

播散性组织细胞质菌病:HIV感染患者的不典型溃疡型(法语)

Introduction. Histoplasma capsulatum var capsulatum is a dimorphic fungi predominating on the American continent. It is responsible for disseminated histoplasmosis associated with AIDS. The presentation in the form of cutaneous ulceration is uncommon and misleading. Observation. A 25 year-old man presented with 3 ulcerations, of 2 to 4 cm in diameter, localized on the lower lip and knees. The patient exhibited fever, alteration in his general status of health and a pulmonary interstitial syndrome. He was seropositive for the human immunodeficiency syndrome (HIV). His lymphocyte CD4+ level was of 1/mm 3. Diagnosis of histoplasmosis was established by direct examination and culture of the cutaneous ulcerations and bronchoalveolar washing fluid. Discussion. The clinical aspect of cutaneous localizations of disseminated histoplasmosis is usuallymultiple,disseminated,papularornodular-typelesions. Ulcerations represent less than 20% ofthe cases described. In our patient, the aspect ofthe lesions at first evoked cutaneous leishmaniosis. Direct mycological examination followed by culture confirmed the final diagnosis.

原发于皮肤的骨外Ewing肉瘤(法语)

Background. Cutaneous extraskeletal Ewing s sarcoma is rare, being seen principally in children. We report a case of cutaneous sarcoma in the sole of the foot in a child. Case report. A 9-yearold child with no medical history of note was presenting a skin tumor for 3months on the heel of the right foot. This tumor was burgeoning and painful and measured 3.5cm in diameter; it was ulcerative at the surface and covered with a crust. Histological and immunohistochemical examinations confirmed the diagnosis of Ewing s sarcoma. Staging examinations proved negative and the patient underwent polychemotherapy, resulting in complete regression of the tumor. Comments. Until 1998, 37 cases of cutaneous and subcutaneous Ewing s sarcoma were reported, being seen in 21 girls and 16 boys. Mean age at diagnosis was 15 years and mean tumor size was 3 cm (range: 1 to 12 cm). The tumors were observed throughout the body, being seen in the sole of the foot in 2 cases. Confirmation of the diagnosis was made by histological examination (malignant proliferation of small round cells in the dermis), immunohistochemical examination (CD99+ ) and cytogenetic analysis (translocation between chromosomes 22 and 11). The prognosis for cutaneous Ewing s sarcoma appears more favorable than that of Ewing s sarcoma in bone. Of the 37 patients treated, 7 had metastases and 2 presented relapse. Treatment for cutaneous Ewing s sarcoma, though not codified, consists of polychemotherapy associated with surgery and/or radiotherapy.

在法属圭亚那对人类免疫缺陷病毒(HIV)感染和非HIV感染患者的皮肤利什曼病的对比性研究

Background:Few data are available on cutaneous leishmaniasis caused by dermot ropic species in human immunodeficiency virus (HIV)-infected patients. Objectiv es:To describe nine cases of cutaneous leishmaniasis in HIV+patients and to co mpare their clinical features and their response to treatment with those of HIV -patients with the forms of leishmaniasis commonly found in French Guiana. Meth ods:A case-control study was carried out between July 1994 and December 2000 i n French Guiana. We compared the following variables in nine HIV-infected patients with leishmaniasis and 27 matched controls:clinical type of leishmaniasis, number of lesions, presence of lymphangitis and adenopathy, the rate of recovery after treatment, and recur rence or reinfection. Results:Eight of the HIV-infected patientshad localized cutaneous leishmaniasis and one had mucocutaneous leishmaniasis. All of the cont rols had localized cutaneous leishmaniasis. Leishmania guyanensis was the only s pecies isolated from HIV-infected subjects. HIV-Leishmania coinfected patients had a higher rate of recurrence or reinfection (P < 0.02) and a lower rate of r ecovery after one treatment cycle with pentamidine (P < 0.02) than did HIV-subj ects. The CD4+lymphocyte counts exceeded 200 mm-3 in all HIV+patients at the time of the diagnosis with leishmaniasis. Conclusions:In French Guiana, cutaneo us leishmaniasis in moderately immunosuppressed HIV-infected subjects (>200 CD4 +T cells mm-3) is characterized by a higher rate of recurrence or reinfection and is more difficult to treat than that in HIV-subjects.

Kaposi肉瘤及器官移植22例

Background. The discovery of the Human Herpes virus 8 (HHV8) improved our knowledge of the pathogenesis of Kaposi s sarcoma. After organ transplantation, Kaposi s sarcoma exhibits distinctive features compared with other forms of the disease. Patients and methods. We report 22 cases of post-transplant Kaposi s sarcoma (12 kidneys, 2 kidney-pancreas, 6 livers and 2 hearts). The aim of this retrospective study was to analyze clinical and virological characteristics in these transplant patients and to specify the frequency of HHV8 seroconversions in this population. Results. Twenty-one patients showed cutaneous lesions and 9 had visceral involvement. HHV8 serology was positive in 16/20 patients at transplantation and in 21/22 cases at the time of Kaposi s sarcoma diagnosis. Most cases corresponded to viral reactivations whereas seroconversions occurred in 2 cases and may have been linked to viral transmission by the graft. Treatment led to recovery in 68p. 100 of the cases. Two heart-transplant patients died from their disease. We included in our series two cases of re-transplanted patients without recurrence of Kaposi s sarcoma and one case of familial Kaposi s sarcoma. Discussion. Seroconversions after transplantation emphasize the interest of systematic screening of HHV8 serology in transplant recipients and their donors.

Ito型色素镶嵌现象揭示20三倍体镶嵌性

Introduction. Ito hypomelanosis-type pigmentary mosaicism is characterized by congenital pigmentation disorders along Blaschko’ s lines. We report a case of Ito-type pigmentary mosaicism associated with a congenital growth hormone deficiency having revealed trisomy 20 mosaicism. Observation. A 4 year-old boy presented with congenital pigmentation disorders. His history was marked by: inter-uterine delayed growth of unknown etiology, a dysmorphic syndrome, psychomotor retardation with speech problems, right cryptorchidia and an isolated, idiopathic, congenital growth hormone deficiency that had been treated with recombinant somatropine since the age of three. The clinical examination revealed alternating hypo and hyper-pigmented maculae with linear distribution on the limbs and in “ twirls" on the trunk following Blaschko’ s lines. The blood karyotypewas normal, the karyotype on fibroblasts of hypopigmented skin revealed trisomy 20 mosaicism. Discussion. The occurrence of pigmentary mosaicism related to trisomy 20 mosaicism is exceptional. The combination of Ito hypomelanosis-type pigmentary mosaicism and delayed growth due to growth hormone deficiency has never been reported before. Our observation, unusual because of such an association, raises the question of the eventual existence of associated genes located on the chromosome 20 implied in the secretion of growth hormone and/or melanogenesis. It also underlines the interest of conducting cytogenic explorations on fibroblasts of damaged skin in the case of Ito-type pigmentary mosaicism, even if the blood karyotype is normal or in the absence of a patent phenotype abnormality.

隐性营养不良型大疱性表皮松解伴IgA肾病:4例报道(法语)

Background. The Hallopeau-Siemens type of recessive dystrophic epidermolysis bullosa (HS-RDEB) is a severe hereditary dermatosis, associated with a collagen VII deficiency. A chronic inflammatory syndrome, secondary to recurrent cutaneous infections, may be the cause of AA amyloidosis, with chronic renal failure, involving life prognosis. Less frequently, an IgA glomerulonephritis may occur and induce renal failure. Only two cases have been previously described. We report herein four new cases. Case report. We report four cases of HS-RDEB associated with IgA glomerulonephritis. A renal biopsy confirmed the diagnosis in all four cases. Later on, two patients had a second renal biopsy, indicated for deterioration of renal function. One of these patients showed AA type renal amyloidosis on the second biopsy. None of these six biopsies, conducted in our four patients led to local cutaneous complications. Subsequently three patients presented with terminal renal failure. Hemodialysis was set up, with good tolerance and improvement in quality of life. Discussion. IgA glomerulonephritis should be suspected if a patient with HS-RDEB presents with hematuria. Renal biopsy is not contraindicated, confirms the diagnosis and helps to specify the prognosis. Hemodialysis is possible and well tolerated in the terminal stage of renal failure. There is not enough evidence for a genetic link between HS-RDEB and IgA glomerulonephritis, but repeated skin infections may be involved in the pathophysiology of the renal disease.

在HIV感染的患者中使用可生物降解的物质改善面部皮下脂肪萎缩(法国)

Introduction. Facial lipoatrophy in HIV-infected patients undertri-therapy occurs frequently and alters their quality of life. No systemic treatment is capable of curing this problem. Patients and methods. Eighty-one patients were included in an open, prospective, compassionate study. A strict intradermal injection of a biodegradable, polyacrylamide cationic copolymer was administered in several sessions. Results. In all the patients, the correction of the facial lipoatrophy was considered satisfactory and stable over a period of 6 months using comparative photographic cliches. The thickness of the dermal skin, assessed by sonography, was doubled. No local or systemic side effect was reported. Conclusion. Our study shows that is it possible to durably correct the facial lipoatrophy of HIV-infected patients under tri-therapy by using a biodegradable implant.

CO_2激光汽化疗法治疗Hailey-Hailey病(法国)

Introduction. Only ablative methods lead to long termremission of areas affected by Hailey- Hailey disease: excision/skin graft, cryosurgery, dermabrasion... The method using the CO2 laser is a recent addition in the management of this dermatitis. We report our experience with this technique in 4 patients. Patients and methods. Carbon dioxide laser vaporization was proposed to 4 patients exhibiting Hailey- Hailey disease resistant to classical treatments. A test under local anesthesia was performed beforehand in all the patients. A 60 year- old man had an immediate reaction and refused to continue treatment. In the other 3 cases, the result of the test at 6 months was considered satisfactory. These patients were treated under general anesthesia in a single area of 50 to70 cm2, and a half- body for comparison. The CO2 laser was used in pulse mode, with successive irradiations, until a homogenous, whitish- yellow aspect with first retraction was obtained. Results. Although the healing delays were long (a mean of 1 month) and required major analgesics over the first few days, the cosmetic results were satisfactory and no abnormal scarring was observed. After a median follow- up of 27 months, no relapse of the disease other than punctiform elements was noted. All the patients wanted treatment of the other remaining affected areas be continued. In 2 patients, CO2 laser vaporization permitted treatment of areas not easily accessible to other ablative methods (around the mouth, the anus and the vulva) with anatomy and normal function spared. Discussion. These results are globally good. Although the time to healing was long, the cosmetic and functional results were always satisfactory, without abnormal scarring. Moreover, in 2 of the patients, CO2 laser was able to treat areas inaccessible to other methods. The reason for the efficacy of ablative methods is debated. Re- epidermization with keratinocytes of appendices and not expressing the molecular defect, and the constitution of dermal cicatricial tissue, are two currently proposed hypotheses.

疱疹样脓疱病合并Ondinecurse(法国)

Introduction. Impetigo herpetiformis is a rare dermatitis that occurs during pregnancy and may be life threatening for both mother and child. In this case report, we present an Ondine curse involving the baby, and the good response to isotretinoine. Case report. A first pregnancy, 26 year-old woman developed at 8 months a widespread skin lesion involving the medial side of the thighs, abdomen and intertriginous areas, with a severe systemic toxic condition and fever. Diagnosis of impetigo herpetiformis was made and corticosteroids, methotrexate and cyclosporinewere unsuccessful. Isotretinoine rapidly improved the patient with good control of the disease. The full term baby had an Ondine curse. Discussion. Our case is typical of impetigo herpetiformis. Maternal and infant complication may be life threatening and we report a real Ondine curse the etiology of which remains unknown. Moreover, this observation is unusual because the lesions did not clear despite delivery and good treatment. In our opinion, the great improvement with isotretinoine would suggest it could be used as first line treatment.

评估Colemanlipostructure治疗HIV感染患者的面部脂肪萎缩及与该技术疗效相关的参数

Objective: To evaluate the efficiency of Coleman lipostructure in patients infected with human immunodeficiency virus (HIV). Design: Open-label study and survey. Setting: Ambulatory dermatosurgery department of a university hospital. Patients: Thirty-three consecutive HIV-infected patients undergoing Coleman lipostructure between 2000 and 2001. Interventions: Clinical examination,blood tests, and standardized photographs at baseline and 1 year after the lipostructure. Me an Outcome Measures: Efficiency was assessed by the agreement of 3 independent m edical specialists on facial lipodystrophy improvement after surgery and by patient satisfaction. Results: Facial lipoatrophy was improved in 12 patients (36%; 95%confidence interval, 20%-52%) as judged by all 3 evaluators. Quantity of fat injected (P=.01) and a low serum triglyceride level before surgery (P=.03) were significantly associated with improvement of facial lipoatrophy. Of the 33 patients, 14 (43%) were very satisfied, 17 (50%) were partly satisfied, and 27 (81%) had a better quality of life. The most common comment was that the patie nt looked better and appeared less ill. Conclusion: Our 1-year evaluation of Coleman lipostructure for correction of facial lipoatrophy in HIV-infected patien ts proved the efficiency of this treatment when measured conservatively by agree ment on improvement by 3 independent specialists and demonstrated a patient satisfaction rate of 93%.

单纯用钙泊三醇后再用钙泊三醇/倍他米松二丙酸盐与Tacalcitol治疗寻常银屑病疗效比较:随机双盲试验

Background: A two-compound product containing calcipotriol 50 μg/g and betamethasone dipropionate 0.5 mg/g (Daivobet., Dovobet.)hasbeendemonstratedtobeaneffective,oncedaily, treatment for psoriasis vulgaris. Objective: To compare the efficacy and safety of treatment with the two-compound product for 4 weeks followed by calcipotriol for 4 weeks, with that of tacalcitol for 8 weeks in patients with stable psoriasis vulgaris. Methods: 501 patients were randomised to double-blind treatment with the two-compound product followed by calcipotriol 50 μg/g once daily, or to tacalcitol 4 μg/g once daily. Results: Treatment with the two-compound product/calcipotriol was significantly more effective than tacalcitol in terms of mean percentage PASI reduction (65.0 vs. 33.3%at week 4 and 59.0 vs. 38.4%at week 8; P < 0.001 for both). Conclusion: A treatment regimen comprising calcipotriol/betamethasone ointment (Daivobet) for 4 weeks followed by calcipotriol for 4 weeks is superior to tacalcitol ointment for 8 weeks in patients with psoriasis vulgaris.

婴儿出现弥散性粟粒疹提示Bazex-Dupré-Christol综合征(法语)

Background. We report two cases of Bazex-Dupré-Christol syndrome in a father and daughter with divergent clinical pictures at two different ages. Patients and methods. A 6-month-old infant was seen with her parents for profuse milia which had appeared when she was 2 months old and persisted ever since. The remainder of the dermatological examination and the psychomotor development tests were normal. Dermatological examination of the father showed atrophic cutaneous lesions with follicular punctuated depressions (like “ice-pick marks”) on the back of the hands and the forearms. He also presented diffuse hypotrichosis and hypohydrosis. In addition, he had a history of basal cell carcinoma with surgery before the age of 35 years. Finally, questioning revealed the existence of numerous similar cases in the family. In view of all these factors, a diagnosis of Bazex-Dupré-Christol syndrome was made. Discussion. Bazex-Dupré-Christol syndrome is a genodermatosis with X-linked dominant inheritance. Diagnosis is based on association of follicular atrophoderma, congenital hypotrichosis, hypohydrosis and early basal cell carcinoma. Other than fragile skin and cosmetic blemishes, these tumors are the only complication of the disease and require regular dermatological surveillance and solar protection. Common initial signs of the disease are abnormally profuse milia in neonates that tend to persist throughout childhood. Several differential diagnoses may be evoked.

Kimura病伴随毛细血管外肾小球肾炎(法语)

Background. Kimura’s disease is a rare and benign chronic in-flammatory soft-tissue disorder of unknown origin. Most cases involve young Asian males. The typical presentation consists of painless papules or nodules with a predilection for the head and neck region, associated with lymphadenopathy, parotid gland involvement, peripheral blood eosinophilia and raised immunoglobulin E. Patients and methods. A 49-year-old Mauritian man was hospitalized for nephrotic syndrome. Examination revealed a painless 3 cm nodule in the left pre-auricular region and multiple enlarged left cervical lymph nodes. Histopathologic examination of a nodule confirmed the diagnosis of Kimura’s disease, which was associated with necrotic extracapillary glomerulonephritis. Corticosteroids were initiated, resulting in rapid improvement of renal function and partial regression of the patient’s cutaneous nodules and lymph node disorder. Discussion. Renal manifestations are the only visceral localization of Kimura’s disease, and proteinuria is seen in 12%of cases, of which 50%are accompanied by nephrotic syndrome. Many different histologic types of renal involvementmay be observed with potentially severe lesions. To our knowledge, this is the first case of Kimura’s disease associated with extracapillary glomerulonephritis.

Acute lung injury in mechanically ventilated patients with epidermal necrolysis:an exposed-unexposed retrospective cohort study

To the Editor,During the acute phase of epidermal necrolysis(EN,including Stevens-Johnson syndrome and toxic epidermal necrolysis),mucosal lesions involving the tracheo-bronchial tract have been reported[1,2]and approximately one in four EN patients develop acute respiratory failure and require endotracheal intubation and mechanical ventilation(MV)support[3].Asymptomatic respiratory sequelae may be detected on pulmonary function tests after the acute phase in as much as 50%of EN patients,possibly related to pulmonary lesions that occurred at the acute phase[4].

慢性荨麻疹、银屑病和异位性皮炎对生活质量影响的比较研究

Background: A better management of chronic skin disorders (CSDs) requires a knowledge of their impact from the patient’ s point of view. Objectives: To determine which aspects of the patient’ s life are mainly impaired in the different CSDs,andprovide comparative references to estimate better the real impact of the different CSDs. Patients and methods: A prospective cross-sectional and matched study of 1356 adult outpatients to compare the health-related quality of life (HRQL) profile in chronic urticaria (466 CU), psoriasis (464 PSO) and atopic dermatitis (426 AD), using the VQ-Dermato, a multidimensional instrument in French validated for CSDs. Results: After adjustment for confounders, HRQL dimensions were differently affected in the three CSDs. The ’ physical discomfort’ dimension was more degraded in AD and CU than in PSO (P < 0.001), and ’ leisure activities’ more in PSO than in CU (P < 0.001). ‘ Self-perception’ and ‘ reatment-induced restrictions’ dimensions were much less affected in CU than in PSO and AD (P < 0.001). In PSO, the ‘ daily living activities’ dimension was much less impaired than in CU and AD (P < 0.001). No aspect of HRQL was really spared in AD. Conclusions. The comparison shows that CU, PSO and AD are characterized by completely different qualitative profiles of impact on HRQL, which are influenced by their clinical characteristics and usual treatment options. It underlines the severe impairment of CU which is often underestimated.

转移性恶性黑素瘤患者对达卡巴嗪发生超敏反应(法语)

Background.Dacarbazine(DTIC)is the first-line chemotherapy for metastatic malignant melanoma without cerebral metastasis.Its clinical and hematological safety is usually good.Hypersensitivity in hepatic failure patients is the most serious side effect described.Patients and methods.This was a retrospective study of the prevalence of hypersensitivity in patients treated with DTIC for metastatic melanoma between 11/01/2002 and 10/31/2003.Hypersensitivity was diagnosed in the event of fever,hypereosinophilia(>500/mm3)with or without liver dysfunction(> twice pre-therapeutic values).Clinical data,DTIC administration modalities,number of courses and clinical and laboratory safety data were recorded.Results.Twenty patients were included,11 women and 9 men of median age 58.6 years(22-82 years)with multiple metastases in all cases.DTIC was the first-line treatment for 19 patients,being administered for 4 days to 10 patients and for 1 day to the other 10 patients,depending on their overall health status.Five hypersensitivity-like manifestations were observed,all in the 4-day treatment group.In 3 patients,fever and hypereosinophilia were seen without liver dysfunction at D3 of the second course of treatment.In 2 patients,treatment was stopped after the second course because of disease progression.In the third patient,4 courses were given with recurrence of symptoms,although the latter were controlled during the fifth course with corticosteroids and antihistamines given 15 minutes before the start of treatment.Two patients experienced severe forms of hypersensitivity with fever,hypereosinophilia,liver dysfunction(cytolysis and cholestasis)and delayed medullar aplasia,after the first and second course respectively.In one patient,bone marrow examination showed a block at the promyelocytic stage consistent with a toxic etiology.Treatment with DTIC was stopped,and all signs regressed with symptomatic treatment.Discussion.Hypersensitivity with DTIC seems to be frequent,being observed in 20%of our patients,with early onset(after the first or second course)and absence of dose-dependence.We describe for the first time two cases of medullar aplasia occurring in association with DTIC hypersensitivity.During phase I studies,the hematologic toxicity of DTIC was moderate,rarely affecting red cells,and was observed with higher doses than those used in metastatic malignant melanoma.We suggest that this aplasia forms part of the signs of hypersensitivity because of the bone marrow morphology,the existence of anemia and concomitant resolution with all the others signs of hypersensitivity.Conclusion.Laboratory monitoring(NFS,liver enzymes)is thus justified,particularly after the first and second courses of DTIC.In case of fever and hypereosinophilia without liver dysfunction,DTIC may be continued together with symptomatic treatment.In the event of hepatic dysfunction,and of course severe hematological disorders,potentially fatal complications can occur and treatment must be stopped.

转移性恶性黑色素瘤患者检测到高水平血清蛋白酶体

Background: Proteasomes, nonlysosomal proteolytic structures, axe implicated in cell growth and differentiation. An abnormal expression has been described in haematopoietic malignancies and in some solid tumours. Objectives: To study the plasma proteasome levels in patients with malignant melanoma (MM) using an enzyme- linked immunosorbent assay (ELBA)technique, and to compare them with the values obtained in a normal population and in patients with severe psoriasis or chronic idiopathic urticaria (CIU). Methods: Plasma proteasome level was measured using a sand wich ELBA test in normal donors (n=14), and in patients with stage I/II (n=13), stage III (n=6) and stage IV (n=10) MM, severe psoriasis (n=13) and CIU (n=6). Tissue proteasome expression was also detected by immunohistology using a monoclonal antibody in paraffin- embedded samples of normal tissue, psoriasis skin and MM. Results: In normal donors, mean ± SEM plasma proteasome concentration was 2138 ± 221 ng mL - 1. Patients with stages III and IV MM exhibited a significantly higher value (3373 ± .470 ng mL- 1 and 8931 ± 1232 ng mL- 1, respectively). Values in patients with stage I/II MM and CIU were not significantly different from those in normal volunteers. Patients with severe psoriasis also exhibited increased values (3398 ± 374 ng mL - 1) but to a lesser extent than in patients with stage FV MM. There was a significant correlation of proteasome levels with serum lactate dehydrogenase in the MM group. Tissue expression as demonstrated by immunohistoche mistry paralleled the se findings. The strongest expression was seen on MM slides and to a lesser extent in psoriasis samples, the weakest expression being observed in normal skin. Conclusions: Proteasomes are strongly expressed in cutaneous MM; high levels of circulating proteasomes are detected in patients with metastatic MM with a high melanoma burden, and at a lesser extent in psoriatic patients, which suggests proteasomes represent a marker more of nonspecific inflammation than of early cancer.