Dermatomyositis and malignancy. A multicenter cooperative study

A retrospective analysis of 118 cases of dermatomyositis (DM) collected in 22 departments of dermatology over a 5-year period is presented. 34 cases (28%) were associated with a cancer that was diagnosed more than 1 year prior to (7 cases), concomitantly (22 cases) or more than 1 year after (5 cases) the occurrence of DM. There was a clear correlation between the evolution of DM and a cancer paraneoplastic evolution in 8 cases only (22%). In most of the cases, no extensive diagnostic procedures were necessary for identifying the tumors. 58% of the patients with cancer-associated DM died 20 (8-29) months after the diagnosis of DM. This multicenter study based on a short period of time confirms and extends previous observations based on retrospective single-center analyses.     

皮肌炎/多发性肌炎伴恶性肿瘤45例分析

目的分析皮肌炎，多发性肌炎伴发恶性肿瘤患者的临床情况。方法回顾性分析我院1991—2005年间45例皮肌炎，多发性肌炎伴发恶性肿瘤患者，分析其临床特点及肿瘤相关因素。结果313例皮肌炎，多发性肌炎患者中45例（14．38％）伴发恶性肿瘤，伴发的肿瘤主要为鼻咽癌、肺癌、乳腺癌，69％患者恶性肿瘤发现于皮肌炎的诊断之时。伴发肿瘤的高危因子包括皮肌炎的发病年龄晚（〉40岁）（P=0．0047，OR=0．186）及病程短（〈1年）（P=0．0033，OR=3．884）。结论皮肌炎，多发性肌炎常伴发恶性肿瘤，所合并肿瘤的类型广泛，但以鼻咽癌、肺癌、乳腺癌等为主。对于年龄〉40岁、病程〈1年的患者，应进行相关检查及监测以排除可能合并的恶性肿瘤。     

Mycetoma: retrospective study of 13 cases in Tunisia

Mycetomas are inflammatory pseudotumors of chronic and progressive evolution, of fungal (Eumycetoma), or bacterial (Actinomycetoma) origin. We conducted a retrospective study of patients who have been consulted for mycetoma over a period of 28 years. Thirteen cases of mycetomas were collected during the 28-year period, 10 patients were of rural origin. The lesions were localized on the foot in all instances. There was no male predominance. Histopathological examination confirmed mycetoma in all 13 cases and the determination of the infectious agent in 9 cases: 4 actinomycotic and 5 fungal. Microbiological examination identified the species in 7 cases. Bone involvement was found in 10 cases. Eumycetomas were treated by ketoconazole. The other cases were treated as actinomycetomas by antibiotics. The antibiotic treatment was associated with surgical excision in 4 cases and with amputation in 1 case. Mycetomas are rare in Tunisia, and only observed sporadically, for this reason the diagnosis is usually late, with severe functional, psychological, and socioeconomic consequences.     

Risk factors for erysipelas of the leg in Tunisia: a multicenter case-control study

BACKGROUND: Risk factors for erysipelas (cellulitis) were rarely evaluated in controlled studies. Regional variations of these risk factors have never be assessed. OBJECTIVE: To assess risk factors for erysipelas of the leg in Tunisia. SUBJECTS AND METHODS: Case-control study in seven hospital centers in Tunisia. Cases were 114 consecutive patients with erysipelas of the leg [sudden onset (<24 h) of a well-demarcated dermo-hypodermatitis with fever or chills]. Two controls were matched to each case for age, sex, and hospital (n = 208). Main outcome measures are local and general suspected risk factors for erysipelas of the leg. RESULTS: In multivariate analysis, disruption of the cutaneous barrier (i.e. traumatic wound, toe-web intertrigo, excoriated leg dermatosis or plantar squamous lesions) and leg edema were independently associated with erysipelas of the leg, with respective odds ratios of 13.6 (95% confidence interval: 6.0-31) and 7.0 (1.3-38). No association was observed with diabetes, alcoholism, or smoking. CONCLUSIONS: We confirmed the major role of local risk factors and the minor role of general risk factors for erysipelas of the leg, in a setting different than the one previously studied. Detecting and treating toe-web intertrigo and traumatic wounds should be considered in the prevention of erysipelas of the leg.     

Polymyositis and dermatomyositis associated with malignancy: a 30-year retrospective study

BACKGROUND: Polymyositis and dermatomyositis in association with malignancy are paraneoplastic syndromes, but the incidence, treatment and factors that predict associated cancer and its prognosis all remain unclear. PATIENTS AND METHOD: During the 30-year period 1969-99, we treated 64 patients who had polymyositis (including two with cancer) and 28 patients who had dermatomyositis (including 10 with cancer). We compared the clinical findings of the patients who had cancer with the findings of those who did not have cancer. RESULTS: The risk of cancer is significantly higher in dermatomyositis and somewhat higher in polymyositis. An increased cancer risk was found in male patients with dermatomyositis who were older than 50 years. Cancer was diagnosed within 4 years before or after the diagnosis of polymyositis or dermatomyositis, and usually within 1 year. An operation was not possible in many of the patients with cancer because of the advanced stage of the disease. CONCLUSION: Our findings suggest that early discovery of malignancy is critical in cases of polymyositis and dermatomyositis.     

Scleroderma in children: a retrospective study of 70 cases

BACKGROUND: Scleroderma is uncommon in childhood. The aim of our study was to analyze the frequency of different clinical forms, their prognostic significance, biological features, and co-morbidities and to assess the pertinence of therapeutic options. PATIENTS AND METHODS: The files of 70 children with primary scleroderma seen from 1980 to 1997 were retrospectively reviewed. RESULTS: Localized scleroderma was observed in 56 children and diffuse lesions in 14. Localized scleroderma (44 girls, 12 boys) began early at a mean age of 7 years 2 months. The lesions presented as isolated bands (39 p. 100), associated with morphea (36 p. 100), or multiple morphea (5 p. 100). Mean duration of the clinical course was longer in cases with more and deeper lesions. Eosinophilia was observed at onset in 38 p. 100 of the cases and antinuclear antibodies were found in 28 p. 100. Local corticosteroid therapy (level I or II) appeared to be useful in the superficial and active lesions (morphea) but did not halt progression to deep scleroderma. General corticosteroid therapy (1 mg/kg/24 h) did not prevent the development of sequelae in cases with bands (16/16). Diffuse scleroderma corresponded to systemic scleroderma (6 cases), dual morbidity (dermatomyositis, mixed connective tissue disease) (6 cases), or scleroderma after eosinophil fasciitis (2 cases). Age at onset was around 9 years with female predominance. A particular gloves and socks form was observed and cardiac involvement was common, but there was no case of renal involvement. The therapeutic problems were similar to those in adults. DISCUSSION: Our findings emphasize that scleroderma occurs readily in childhood, unlike what has been reported 10 years ago. Prognosis depends on functional impairment resulting from major sequelae particularly important in localized forms and the life-threatening situations occurring in systemic forms.     

Childhood cutaneous tuberculosis: a 20-year retrospective study in Tunis

Despite prevention programs, tuberculosis is still endemic in developing countries. We assessed the epidemiologic and clinical profiles of childhood cutaneous tuberculosis in our dermatology department from 1981 to 2000 and compared it to previous Tunisian reports and to the relevant literature. This is a retrospective study over a 20-year period (1981-2000) in a large teaching hospital of the capital. Patients included were below age 15 years. Diagnosis was based upon clinical examination, tuberculin reaction, histopathology and response to antitubercular therapy. There were 26 patients with cutaneous tuberculosis, 0.1 percent of the total number of dermatology outpatients for that time period. Of these 26, seven (27 %) were immunocompetent. There were four boys and three girls and the mean age was 9.5 years. Three patients had lupus vulgaris, three had scrofuloderma, and one child had orificial tuberculosis. Six out of seven children were BCG vaccinated. There was no family history of tuberculosis. The Mantoux reaction was positive in six children. There was no systemic organ involvement in all cases. All patients were treated successfully with triple or quadruple anti-tubercular drugs for 4-11 months. Compared to a previous Tunisian report conducted over an 8-year period in the seventies, the incidence of childhood cutaneous tuberculosis has decreased. In that report, scrofuloderma was the most frequent form. Currently the incidence of lupus vulgaris has reached that of scrofuloderma, demonstrating the increase of the clinical pattern associated with strong immunity. All children had localized disease and responded to antimycobcterial chemotherapy.     

Infantile acne: a retrospective study of 16 cases

Abstract:  Infantile acne is a rare and poorly understood disorder. The objective of this study was to improve our knowledge about the epidemiology and clinical course of infantile acne, and evaluate approaches to treatment. This two-center retrospective study covered the period between 1985 and 2007. Inclusion criteria were: (i) age less than 24 months when lesions appeared; (ii) presence of both inflammatory and noninflammatory lesions; (iii) persistence of lesions for at least 2 months. The data were drawn from clinical and photographic records, followed by administration of a telephone questionnaire to parents. It was proposed that each case be reviewed on the basis of the child's appearance and score on an acne scar clinical grading scale. Sixteen children were included. Nine had a family history of severe adolescent acne. The average duration of disease was 22 months. Two patients had been effectively treated with oral isotretinoin. More than half of the patients exhibited scars. We re-examined five children (average acne scar clinical grading scale score = 12/540). On the basis of the frequency of scarring, and the severity and average duration of lesions, the use of oral retinoids in severe infantile acne warrants evaluation.     

Pemphigoid gestationis: a study of 20 cases

INTRODUCTION: Pemphigoid gestationis is a bullous autoimmune sub-epidermal dermatosis, occurring during pregnancy and/or postpartum. The objective of our study was to define the epidemio-clinical profile, the histopathological and immunopathological features, the treatment and the course of pemphigoid gestationis in Tunisian patients. PATIENTS AND METHODS: This was a retrospective study concerning the cases of pemphigoid gestationis recorded between 1989 and 2003 in the dermatology department in La Rabta Hospital in Tunis. The patients were included according to clinical, histopathological and immunopathological criteria. RESULTS: We retained 20 patients: 15 multiparae and 5 primiparae. The average age at onset was 29. The first clinical signs appeared in the 3rd trimester in 60 p. 100 of all cases. Clinically, the urticarial patches were noticed in 90 p. 100 of all cases and blisters in 65 p. 100 of the cases. The eruption was located mainly on the trunk and the limbs. The face was affected in 7 cases, the mucous membranes in 3 cases, the palms in 2 cases and the soles in 1 case. Cutaneous histologic examination revealed a sub-epidermal blister in 11 cases and a lymphohistiocyte infiltrate in all cases. Direct immunofluorescence showed a linear deposition of the third component of the complement along the basement membrane zone in all cases. Fetal prognosis was assessed in 13 cases: 1 fetal death, 1 still-born, 3 miscarriages, 1 anencephaly and 2 cases of transit bullous affection in the new-born. DISCUSSION: Our results are similar to those in the literature, but with some particular aspects: the late onset of the pemphigoid gestationis in the course of the 3rd trimester of pregnancy, the frequent involvement of the face and the mucous membranes and the absence of the two main fetal risks: prematurity and hypotrophy. Moreover, we underline the efficiency of topical class I corticosteroids in the treatment of pemphigoid gestationis.     

Development of monoclonal gammopathy under biotherapy in psoriasis: a French multicenter retrospective study

Background

Biotherapies or targeted therapies are fairly new treatments indicated for moderate to severe psoriasis. The side effects appear to be mainly infectious or cancerous. The role of biotherapies in the development of a pre-cancerous condition, monoclonal gammopathy of undetermined significance (MGUS), has recently been debated in the literature.

Objectives

To evaluate the incidence of MGUS in psoriasis patients treated with biotherapy.

Materials and Methods

This study was a French multicenter retrospective study carried out through the French multicenter study group RESOPSO. Data on the results of serum protein electrophoreses performed before and within at least six months after the start of the biotherapy were collected. Demographic data, medical history, and psoriasis treatment history were specified.

Results

Four hundred and forty three patients were eligible for inclusion. Of these, three presented with monoclonal gammopathy for which the assessment was in favor of MGUS. The average treatment period was 19.7 months. Six patients presented withMGUSprior to the treatment. These patients’ immunoglobulin levels remained stable, with an average remission of 24 months. Only psoriatic rheumatism appeared to be statistically linked to MGUS.

Conclusion

The incidence and frequency ofMGUSin psoriasis patients treated with biotherapy do not appear to increase relative to the general population.

     

Dermatomyositis and nasopharyngeal carcinoma: 3 cases

BACKGROUND: Nasopharyngeal carcinoma is a common cancer in Tunisia with an estimated incidence of 1.8/100,000. The tumor shows a characteristic association with paraneoplastic syndromes. CASE REPORTS: We report three cases of histologically proven dermatomyositis associated with nasopharyngeal carcinoma in patients aged 40, 24 and 65 years. In all cases, the course of the paraneoplastic syndrome ran parallel to the nasopharyngeal carcinoma. DISCUSSION: Dermatomyositis is one of the numerous paraneoplastic syndromes associated with nasopharyngeal carcinoma. Systematic examination of the nasopharynx is required in case of dermatomyositis.     

483例儿童花斑癣回顾性分析

目的总结及分析儿童花斑癣患者的临床特点,比较与成人花斑癣的异同点.方法就诊于我院皮肤科门诊的花斑癣患者认真填写问卷调查表,内容包括:性别、年龄、发病年龄,病程、皮损部位、诱因、发病季节、家族史.并进行真菌学检查.结果儿童花斑癣患者共483例,其中男286例,占59.2%,女197例,占40.8%.年龄15天至12岁.所有患儿面部发病207例,占42.9%,其中发生于前额的共143例,占面部发病的69.1%.多汗患儿405例,占83.9%.有家族史38例,占7.9%.所有患儿均采用1%联苯苄唑乳膏外用,治疗2周以上,447例患儿通过治疗后得到有效的临床症状改善及病原学疗效,总有效率为92.5%.结论儿童花斑癣同成人花斑癣比较有不同的临床特点,发病因素与成人不尽相同,且在诊断上需与儿童面部色素减退性皮肤病进行鉴别.