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 impai...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.展开更多
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. Obje...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.展开更多
Background: Microcystic adnexal carcinoma (MAC) is a rare cutaneous neoplasm, with a high rate of local recurrences. Objective: A series of MAC was analyzed and compared to previously published cases. Methods: Seven c...Background: Microcystic adnexal carcinoma (MAC) is a rare cutaneous neoplasm, with a high rate of local recurrences. Objective: A series of MAC was analyzed and compared to previously published cases. Methods: Seven cases of MAC were identified in the register of the institution. Medical and pathological records were reviewed. Results: The primary MAC were located on the face in all patients, and 85% were initially misdiagnosed. The mean follow-up duration was 108 months. The recurrence rate was high: 4 patients developed recurrences. In 3 patients, the course of the disease was severe: one of them developed pathologically proven lung metastasis. Conclusion: The present study and review of the literature confirm the clinically aggressive evolution of MAC and its rare ability to give rise to metastasis. Long-term clinical follow-ups with imaging investigations are mandatory.展开更多
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 patien...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.展开更多
Background. Infliximab (Remicade.) is an anti- TNF alpha indicated in the treatment of chronic inflammatory rheumatism, notably rheumatoid arthritis. Case- report. We report the case of a 56 year- old woman who develo...Background. Infliximab (Remicade.) is an anti- TNF alpha indicated in the treatment of chronic inflammatory rheumatism, notably rheumatoid arthritis. Case- report. We report the case of a 56 year- old woman who developed severe worsening of an SSA- positive subacute lupus erythematosus on initiation of treatment with infliximab for rheumatoid arthritis. Discussion. A review of the literature found 30 cases of drug- induced lupus and listed the autoimmune modifications induced by anti- TNF alpha. This first case of subacute lupus erythematosus, existing before the introduction of treatment and worsening during the latter, emphasizes the risk of developing a severe flare of an autoimmune disease during treatment with anti- TNF alpha. It raises the question of the relative contraindications of anti- TNF alpha in patients with lupus erythematosus.展开更多
Background: EUROPSO (European Federation of Psoriasis Patient Associations) undertook a Europe-wide survey examining quality of life and patients’perspectives on treatment and their disease. Objectives: To explore pa...Background: EUROPSO (European Federation of Psoriasis Patient Associations) undertook a Europe-wide survey examining quality of life and patients’perspectives on treatment and their disease. Objectives: To explore patients’perspectives of psoriasis on their lifestyle and well-being and to gain insight into the effectiveness of and satisfaction with currently available therapies for psoriasis. Methods: Self-administered questionnaires (n = 50 500) were mailed to members of psoriasis patient associations in Belgium, the Czech Republic, Finland, France, Germany, Italy and the Netherlands. Results: Responses were received from 18 386 patients (36%), of whom 17 990 had psoriasis. Mean age at onset of psoriasis was 30.5 years, 59%of respondents had self-reported moderate to severe psoriasis (3%or greater body surface area involvement) and 30%had been diagnosed with psoriatic arthritis. The mean Psoriasis Disability Index score was 12.2 (25%of the maximum score), increasing to 21 (44%) in patients with more than 10%body surface area involvement. The greatest impact was on activities of daily living, especially affecting clothing choice, bathing routine and sporting activities. Overall, 77%replied that psoriasis was a problem or a significant problem. While patients were satisfied with the information and care from their dermatologist (40%highly satisfied), available treatment options were less satisfactory, with over 70%reporting only low tomoderate satisfaction. Conclusions: This is the largest survey of people with psoriasis in Europe and shows that psoriasis has a profound impact on quality of life.展开更多
Eczema craquele, or asteatotic eczema, has been associated with malignant lymphoma although this is rare. Since 1986,we have observed seven patients, six men and one woman,mean age 71.5 years (range 43-86 years), with...Eczema craquele, or asteatotic eczema, has been associated with malignant lymphoma although this is rare. Since 1986,we have observed seven patients, six men and one woman,mean age 71.5 years (range 43-86 years), with systemic lymphoma and concurrent eczema craquele. Five patients had T-cell lymphoma, one had a B-cell lymphoma and one had Hodgkin’s disease. All patients shared several characteristics: (1) a synchronous onset of eczema craquele and lymphoma,(2) generalized eczema, (3)absence of alternative disease or conditions that could favour the onset of eczema craquele, and (4) eczema refractory to topical corticosteroids and emollients, but which resolved upon lymphoma remission and invariably recurred with the lymphoma relapse. All the patients except one died within 1 year, most with active lymphoma. The finding of recalcitrant generalized eczema craquele should prompt a search for lymphoma, particularly in older men. Lymphoma-associated eczema craquele has most characteristics of paraneoplastic syndromes and may be a hallmark of aggressive lymphoma.展开更多
Introduction. The aim of our study was to understand the motivations of outpatients who come to dermatological emergencies in a university hospital. Patients and method. This 6-week prospective study included outpatie...Introduction. The aim of our study was to understand the motivations of outpatients who come to dermatological emergencies in a university hospital. Patients and method. This 6-week prospective study included outpatients who came to the dermatology emergency unit. This consultation is proposed each morning (from 8 to 9), from Mondays to Fridays. A questionnaire was distributed to outpatients. They answered questions on the functioning of this consultation and their own symptoms. The consulting dermatologist answered questions on the referring physician, the really urgent characteristics of the disease and the diagnosis. Results. Patients were satisfied by the functioning of the consultation. Indeed, 59 p. 100 of outpatients thought that the timetable was convenient and 70 p. 100 that the delay before getting a consultation was rapid. 75 p. 100 felt they needed treatment rapidly. Nonetheless, 45 p. 100 did not think they had a serious disease. More than half of the outpatients were referred by their general practitioner; the others came spontaneously, or were referred by other departments or general emergencies. The most frequent diagnoses were cutaneous infections (27.6 p. 100), eczema (21 p. 100), then benign tumors, psoriasis, physical dermatoses, viral eruptions... Discussion. A consultation for dermatological emergencies appears to reply to patients demands. Nonetheless, most of these outpatients do not present with real dermatological emergencies. Criteria for real emergencies needs to be further defined and understood by citizens.展开更多
Background. Agranular CD4+CD56+hematodermic neoplasm (blastic NK-cell lymphoma) has been recently described. The skin is often the first organ involved. Observations. Two old men of respectively 70 and 77 years consul...Background. Agranular CD4+CD56+hematodermic neoplasm (blastic NK-cell lymphoma) has been recently described. The skin is often the first organ involved. Observations. Two old men of respectively 70 and 77 years consulted for infiltrated cutaneous lesions. Preliminary histological examination of cutaneous biopsy taken in both patients showed a malignant proliferation suggesting a cutaneous lymphoma, and the patients were referred. Histological examination of new biopsies showed a very similar proliferation in the 2 cases of monotonous medium-sized mononuclear cells without expression of the common antigens CD3 and CD20 and the expression of receptor gene or the immunoglobulin heavy chain gene were evidenced. No extracutanous involvement was initially detected in the first patient. Thrombocytopenia associated with the abnormal presence of 15 p. 100 of circulating CD4+CD56+cells was initially found in the second patient. The first patient was treated with chemotherapy, with complete remission. A cutaneous relapse promptly occurred,followed by bone and cerebral localizations. The patient died one year after the diagnosis of the disease, in spite of intensification of the treatment. Treatment is still ongoing in the second patient. Comments. The histological presentation of these two patients was very similar withanunusalphenotypeoftumorcellsexpressingCD4,CD56, CD123, but not expressing CD3 and CD20. Some cases have beenpublishedunderthe"termofblasticNKlymphoma"which is the actual term for the disease in the WHO classification. However, the tumor cells derive from the dendritic plasmacytoid cells, also called type 2 dendritic cells, and perhaps from a common precursor to lymphocyte T and dendritic plasmacytoid cells. In spite of complete cutaneous response in the 2 cases presented, as in other reports, extra-cutaneous involvement occurs quickly. Overall survival is usually poor since nearly all the patients died in less than 3 years. This justifies attempting agressive protocols, with bone marrow allograft in the younger patients.展开更多
Introduction. The term “ pustular vasculitis of the hands” described by Strutton for an acute eruption of the hands was recently revisited[1]. “ Neutrophilic dermatosis of the hands” has been preferred. We observe...Introduction. The term “ pustular vasculitis of the hands” described by Strutton for an acute eruption of the hands was recently revisited[1]. “ Neutrophilic dermatosis of the hands” has been preferred. We observed 3 new cases. Observations. Two women and a man, without notable past history, consulted for an acute and painful eruption of both handswith fever. Clinical examination showed erythematous papular or nodular or bullous lesions developed on thenar and hypothenar eminences, and on the finger. Histological findings were predominantly neutrophilic infiltration in the dermis, papillary dermal edema, with or without the presence of leukocytoclastic vasculitis. Diagnosis was Sweet s syndrome. No disease was associated. With indometacine (50- 100 mg per day), all the lesions disappeared rapidly without relapse. Discussion. These 3 cases add to 19 other cases (review of literature) which confirm the existence of a homogenous entity that we think it a localized subset of Sweet s Syndrome, with a fast healing. Our observations are specific by the first localization on thenar and hypothenar eminences and by the excellent response to treatment with a non steroidal antiinflammatory agents such indometacine (50- 100 mg per day). This treatment could be proposed as a first line therapy.展开更多
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 pre...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.展开更多
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 pat...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 basemen tmembrane 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.展开更多
Background. Bullous lesions of acral distribution are an uncommon finding in Bazex’s syndrome (acrokeratosis paraneoplastica). We report here one of these rare cases. Patients and methods. A 65 year-old-man, an alcoh...Background. Bullous lesions of acral distribution are an uncommon finding in Bazex’s syndrome (acrokeratosis paraneoplastica). We report here one of these rare cases. Patients and methods. A 65 year-old-man, an alcoholic and a smoker, presented with characteristic lesions of Bazex’s acrokeratosis paraneoplastica associated with a right cervical lymph node mass. An epidermoid carcinoma of sinus piriformis was then discovered. The acrokeratosis lesions worsened and spread to the knees, elbows, trunk and genitalia, with an erosive aspect, pain in the extremities, and tender, hemorrhagic bullous lesions on the toes and sides of the feet. Histological examination of a biopsy sample showed a subepidermal blister with numerous eosinophilic leucocytes. Direct immunofluorescence showed C3 deposits on dermal capillaries and IgA and IgM on colloid bodies in the papillary dermis. Blood eosinophilia and high levels of IgE were noted. Indirect immunofluorescence was negative for anti-epidermal and anti-basement membrane antibodies. Radiation and chemotherapy for the neoplasia resulted in healing of the bullous lesions and almost complete disappearance of keratotic squamous lesions with residual hyperpigmentation. Discussion. Although bullous lesions are rare in Bazex’s syndrome (acrokeratosis paraneoplastica), they were noted as early as 1968 by Degos et al. These acral bullae mimic autoimmune bullous disease. An immunological reaction is thought to occur with antigens of the dermal-epidermal junction and eosinophils appear to be involved.展开更多
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...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.展开更多
Background.We report a case of group B streptococcal septicemia of digestive origin with secondary bilateral breast dermal-hypodermal localization.Case report.A 71-year-old woman with a past history of bilateral breas...Background.We report a case of group B streptococcal septicemia of digestive origin with secondary bilateral breast dermal-hypodermal localization.Case report.A 71-year-old woman with a past history of bilateral breast cancer treated by conservation therapy was hospitalized because of the sudden occurrence of two clearly delimited,inflammatory,dermal-hypodermal cutaneous plaques located on each breast,associated with fever(39°C),4 days after a colonoscopy.Further investigations eliminated carcinomatous mastitis and blood cultures were positive for group B β-hemolytic streptococcus(Streptococcus agalactiae).Histological examination of a sigmoid polyp revealed a tubular adenocarcinoma.Discussion.We report the first documented case of secondary dermal-hypodermal bacterial skin infection(cellulitis)due to group B β-hemolytic streptococcus.The occurrence after colonoscopy examination,chronology of clinical features,bilaterality and positive blood cultures are arguments in favor of the secondary nature of the skin infection process.展开更多
Introduction. Eruptive pseudoangiomatosis was first described in children in the form of an acute non-pruritic macular or popular rash that fades on application of a glass test and resolves within several days. Viral ...Introduction. Eruptive pseudoangiomatosis was first described in children in the form of an acute non-pruritic macular or popular rash that fades on application of a glass test and resolves within several days. Viral aetiology is suspected but has never been demonstrated to date. Observations. We discuss seven cases of infants presenting this disease: 5 boys and 2 girls aged8 days to 16months. The rash presented typical clinical features in all cases and affected the face and limbs in 6 of the 7 subjects. In one child, involvement of the face and back was observed with sparing of the limbs. The rash occurred after an episode of rhinolaryngeal infection in 3 cases and after gastrointestinal infection in 1 case. Spontaneous resolution was seen within 3 to 10 days in 6 patients although a longer course lasting over 9 months was observed in one infant. In another patient, the rash appeared after surgery for mesoblastic nephroma. In one child, a similar rash was seen in both parents. Screening for infectious agents was negative for the two children from whom samples were obtained. Discussion. This series of paediatric cases of eruptive pseudoangiomatosis is characterised by the very young age of one of the children, coexistence of the condition with a renal tumour in another child, the familial nature of the rash in a third child and unusually long disease duration in the final child. However, this series did not allow identification of the causative infectious agent or agents. Probably, as with other syndromes such as Giannotti-Crosti syndrome or “ gloves and socks"” syndrome, eruptive pseudoangiomatosis forms a clinical picture common to a non-specific viral infection.展开更多
Introduction. Pellagra is an exceptional disorder in France. The classical description of pellagra associates a photoinduced rash with neurological impairment and intestinal dysfunction. Without adapted treatment, the...Introduction. Pellagra is an exceptional disorder in France. The classical description of pellagra associates a photoinduced rash with neurological impairment and intestinal dysfunction. Without adapted treatment, the progression is fatal. Case report. A 62 year-old women developed a photoinduced rash, composed of circular and erythematous elements with pustular edges. She also had panniculitis, peripheral neuropathy, depressive mood and diarrhea. Her medical past was marked by epilepsy treated with sodium valproate and hydantoin. Biological exams revealed lowered plasma levels of vitamins PP, B1, B6 and zinc, secondary to intestinal impairment induced by bacteria proliferating in the small intestine. The rash resolved with vitamin PP and zinc supplementation. The bacterial colonisation was improved by long-term, sequential antibiotics. Discussion. We report a rare clinical form of pellagroid rash. The rash was induced by chronic malabsorption resulting from excessive bacterial proliferation in the diverticules of the small intestine. The antiepileptic treatment could have facilitated vitamin PP et zinc deficiency. Panniculitis was related to the bacterial proliferation. We discuss in this paper the relationship between some vitamin deficiencies, their clinical manifestations and the direct role of intestinal bacterial proliferation in the cutaneous manifestations.展开更多
Congenital afibrinogenaemia is a rare genetic disorder transmitted as an autosomal recessive trait and characterized by the complete absence of fibrinogen in the plasma. We report a 41- year-old woman who suffered fro...Congenital afibrinogenaemia is a rare genetic disorder transmitted as an autosomal recessive trait and characterized by the complete absence of fibrinogen in the plasma. We report a 41- year-old woman who suffered from congenital afibrinogenaemia and hepatitis C viral infection and presented with ischaemic necrosis and livedo of the toes. Laboratory investigations showed the presence of mixed cryoglobulinaemia and anticardiolipin antibodies. Resolution occurred with plasmapheresis. We discuss the pathophysiology of this unusual condition and review the literature for skin manifestations associated with this rare haemostasis disorder.展开更多
Introduction. UVA phototherapy, acitretin and oral corticosteroids are currently the front-line treatment of disseminated cutaneous lichen planus. We studied the efficacy of narrowband UVB therapy in this indication. ...Introduction. UVA phototherapy, acitretin and oral corticosteroids are currently the front-line treatment of disseminated cutaneous lichen planus. We studied the efficacy of narrowband UVB therapy in this indication. Patients and methods. We retrospectively studied the dossiers of patients suffering from disseminated cutaneous lichen planus, treated with narrowband phototherapy in the Phototherapy Unit of the University hospital in Montpellier, from May to November of the year 2001. Disseminated lichen planus was defined as lichen involving at least 20p. 100 of the skin surface. Twenty patients were included. UVB were applied thrice weekly using a Philips TL01 cubicle (311313 nm). The protocol was that used for the treatment of psoriasis. We defined 4 types of response: complete response (disappearance of more than 90p. 100 of the lesions), partial response (disappearance of at least 50p. 100) poor response (improvement in 20 to 50p. 100) and failure (less than 20p. 100 reduction in the lesions). Assessment of relapses in the long term was made using a telephone survey among the patients treated or their physicians. Results. Complete response was obtained in 11 out of the 20 patients (55p. 100) and partial response in 4 (20p. 100), corresponding to 75p. 100 of the responders. Response was obtained with a median delay of 3 months, ranging from 2 to 6 months, following a median of 30 sessions (12 to 50) and acumulated dose of UVB of 36± 4.8 joules/cm2. The phototype, gender, age and duration of evolution before treatment did not influence the response. The relapse rate was and estimated 18p. 100 (2/11) 42 months after treatment had been stopped. Discussion. In our opinion, these results underline the efficacy of narrowband UVB in the treatment of disseminated cutaneous lichen planus. They confirm those of earlier studies and are superimposable with those of oral UVA phototherapy.展开更多
Background. The presence of inorganic foreign bodies in granulomatous cutaneous lesions is not infrequent. In this paper, we describe the first case of cerium-induced cutaneous granulomatous dermatitis. Case report. A...Background. The presence of inorganic foreign bodies in granulomatous cutaneous lesions is not infrequent. In this paper, we describe the first case of cerium-induced cutaneous granulomatous dermatitis. Case report. A 57-year-old woman seriously burned four years ago presented with papulonodular lesions affecting only the burned areas treated with topical cerium nitrate-silver sulfadiazine cream (Flammacé rium ). Biopsies revealed sarcoidal granuloma associated with exogenous particles. Electron probe X-ray microanalysis demonstrated a high cerium content. Screening for systemic sarcoidosis was negative. The patient was treated with hydroxychloroquine. After four months of follow-up, clinical and histological evidence of decreased infiltrate was noted. Discussion. Cerium nitratesilver sulfadiazine cream (Flammacé rium ) is widely used for the topical treatment of burns. The main effect of cerium is to create superficial calcification, which decreases wound colonization and prevents the formation of granulation tissue (no hypertrophic scar formation) in bur-ns. Prior to our case, no cutaneous side-effects of cerium had been encountered. Inoculation of foreign matter may or may not induce granuloma formation or sarcoidosis in different subjects, according to their immunologic status. The favorable outcome in this case could in fact be due to a change in the pattern of cytokinin production (TH1→ TH2) rather than the effects of hydroxychloroquine therapy.展开更多
文摘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.
文摘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.
文摘Background: Microcystic adnexal carcinoma (MAC) is a rare cutaneous neoplasm, with a high rate of local recurrences. Objective: A series of MAC was analyzed and compared to previously published cases. Methods: Seven cases of MAC were identified in the register of the institution. Medical and pathological records were reviewed. Results: The primary MAC were located on the face in all patients, and 85% were initially misdiagnosed. The mean follow-up duration was 108 months. The recurrence rate was high: 4 patients developed recurrences. In 3 patients, the course of the disease was severe: one of them developed pathologically proven lung metastasis. Conclusion: The present study and review of the literature confirm the clinically aggressive evolution of MAC and its rare ability to give rise to metastasis. Long-term clinical follow-ups with imaging investigations are mandatory.
文摘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.
文摘Background. Infliximab (Remicade.) is an anti- TNF alpha indicated in the treatment of chronic inflammatory rheumatism, notably rheumatoid arthritis. Case- report. We report the case of a 56 year- old woman who developed severe worsening of an SSA- positive subacute lupus erythematosus on initiation of treatment with infliximab for rheumatoid arthritis. Discussion. A review of the literature found 30 cases of drug- induced lupus and listed the autoimmune modifications induced by anti- TNF alpha. This first case of subacute lupus erythematosus, existing before the introduction of treatment and worsening during the latter, emphasizes the risk of developing a severe flare of an autoimmune disease during treatment with anti- TNF alpha. It raises the question of the relative contraindications of anti- TNF alpha in patients with lupus erythematosus.
文摘Background: EUROPSO (European Federation of Psoriasis Patient Associations) undertook a Europe-wide survey examining quality of life and patients’perspectives on treatment and their disease. Objectives: To explore patients’perspectives of psoriasis on their lifestyle and well-being and to gain insight into the effectiveness of and satisfaction with currently available therapies for psoriasis. Methods: Self-administered questionnaires (n = 50 500) were mailed to members of psoriasis patient associations in Belgium, the Czech Republic, Finland, France, Germany, Italy and the Netherlands. Results: Responses were received from 18 386 patients (36%), of whom 17 990 had psoriasis. Mean age at onset of psoriasis was 30.5 years, 59%of respondents had self-reported moderate to severe psoriasis (3%or greater body surface area involvement) and 30%had been diagnosed with psoriatic arthritis. The mean Psoriasis Disability Index score was 12.2 (25%of the maximum score), increasing to 21 (44%) in patients with more than 10%body surface area involvement. The greatest impact was on activities of daily living, especially affecting clothing choice, bathing routine and sporting activities. Overall, 77%replied that psoriasis was a problem or a significant problem. While patients were satisfied with the information and care from their dermatologist (40%highly satisfied), available treatment options were less satisfactory, with over 70%reporting only low tomoderate satisfaction. Conclusions: This is the largest survey of people with psoriasis in Europe and shows that psoriasis has a profound impact on quality of life.
文摘Eczema craquele, or asteatotic eczema, has been associated with malignant lymphoma although this is rare. Since 1986,we have observed seven patients, six men and one woman,mean age 71.5 years (range 43-86 years), with systemic lymphoma and concurrent eczema craquele. Five patients had T-cell lymphoma, one had a B-cell lymphoma and one had Hodgkin’s disease. All patients shared several characteristics: (1) a synchronous onset of eczema craquele and lymphoma,(2) generalized eczema, (3)absence of alternative disease or conditions that could favour the onset of eczema craquele, and (4) eczema refractory to topical corticosteroids and emollients, but which resolved upon lymphoma remission and invariably recurred with the lymphoma relapse. All the patients except one died within 1 year, most with active lymphoma. The finding of recalcitrant generalized eczema craquele should prompt a search for lymphoma, particularly in older men. Lymphoma-associated eczema craquele has most characteristics of paraneoplastic syndromes and may be a hallmark of aggressive lymphoma.
文摘Introduction. The aim of our study was to understand the motivations of outpatients who come to dermatological emergencies in a university hospital. Patients and method. This 6-week prospective study included outpatients who came to the dermatology emergency unit. This consultation is proposed each morning (from 8 to 9), from Mondays to Fridays. A questionnaire was distributed to outpatients. They answered questions on the functioning of this consultation and their own symptoms. The consulting dermatologist answered questions on the referring physician, the really urgent characteristics of the disease and the diagnosis. Results. Patients were satisfied by the functioning of the consultation. Indeed, 59 p. 100 of outpatients thought that the timetable was convenient and 70 p. 100 that the delay before getting a consultation was rapid. 75 p. 100 felt they needed treatment rapidly. Nonetheless, 45 p. 100 did not think they had a serious disease. More than half of the outpatients were referred by their general practitioner; the others came spontaneously, or were referred by other departments or general emergencies. The most frequent diagnoses were cutaneous infections (27.6 p. 100), eczema (21 p. 100), then benign tumors, psoriasis, physical dermatoses, viral eruptions... Discussion. A consultation for dermatological emergencies appears to reply to patients demands. Nonetheless, most of these outpatients do not present with real dermatological emergencies. Criteria for real emergencies needs to be further defined and understood by citizens.
文摘Background. Agranular CD4+CD56+hematodermic neoplasm (blastic NK-cell lymphoma) has been recently described. The skin is often the first organ involved. Observations. Two old men of respectively 70 and 77 years consulted for infiltrated cutaneous lesions. Preliminary histological examination of cutaneous biopsy taken in both patients showed a malignant proliferation suggesting a cutaneous lymphoma, and the patients were referred. Histological examination of new biopsies showed a very similar proliferation in the 2 cases of monotonous medium-sized mononuclear cells without expression of the common antigens CD3 and CD20 and the expression of receptor gene or the immunoglobulin heavy chain gene were evidenced. No extracutanous involvement was initially detected in the first patient. Thrombocytopenia associated with the abnormal presence of 15 p. 100 of circulating CD4+CD56+cells was initially found in the second patient. The first patient was treated with chemotherapy, with complete remission. A cutaneous relapse promptly occurred,followed by bone and cerebral localizations. The patient died one year after the diagnosis of the disease, in spite of intensification of the treatment. Treatment is still ongoing in the second patient. Comments. The histological presentation of these two patients was very similar withanunusalphenotypeoftumorcellsexpressingCD4,CD56, CD123, but not expressing CD3 and CD20. Some cases have beenpublishedunderthe"termofblasticNKlymphoma"which is the actual term for the disease in the WHO classification. However, the tumor cells derive from the dendritic plasmacytoid cells, also called type 2 dendritic cells, and perhaps from a common precursor to lymphocyte T and dendritic plasmacytoid cells. In spite of complete cutaneous response in the 2 cases presented, as in other reports, extra-cutaneous involvement occurs quickly. Overall survival is usually poor since nearly all the patients died in less than 3 years. This justifies attempting agressive protocols, with bone marrow allograft in the younger patients.
文摘Introduction. The term “ pustular vasculitis of the hands” described by Strutton for an acute eruption of the hands was recently revisited[1]. “ Neutrophilic dermatosis of the hands” has been preferred. We observed 3 new cases. Observations. Two women and a man, without notable past history, consulted for an acute and painful eruption of both handswith fever. Clinical examination showed erythematous papular or nodular or bullous lesions developed on thenar and hypothenar eminences, and on the finger. Histological findings were predominantly neutrophilic infiltration in the dermis, papillary dermal edema, with or without the presence of leukocytoclastic vasculitis. Diagnosis was Sweet s syndrome. No disease was associated. With indometacine (50- 100 mg per day), all the lesions disappeared rapidly without relapse. Discussion. These 3 cases add to 19 other cases (review of literature) which confirm the existence of a homogenous entity that we think it a localized subset of Sweet s Syndrome, with a fast healing. Our observations are specific by the first localization on thenar and hypothenar eminences and by the excellent response to treatment with a non steroidal antiinflammatory agents such indometacine (50- 100 mg per day). This treatment could be proposed as a first line therapy.
文摘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.
文摘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 basemen tmembrane 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.
文摘Background. Bullous lesions of acral distribution are an uncommon finding in Bazex’s syndrome (acrokeratosis paraneoplastica). We report here one of these rare cases. Patients and methods. A 65 year-old-man, an alcoholic and a smoker, presented with characteristic lesions of Bazex’s acrokeratosis paraneoplastica associated with a right cervical lymph node mass. An epidermoid carcinoma of sinus piriformis was then discovered. The acrokeratosis lesions worsened and spread to the knees, elbows, trunk and genitalia, with an erosive aspect, pain in the extremities, and tender, hemorrhagic bullous lesions on the toes and sides of the feet. Histological examination of a biopsy sample showed a subepidermal blister with numerous eosinophilic leucocytes. Direct immunofluorescence showed C3 deposits on dermal capillaries and IgA and IgM on colloid bodies in the papillary dermis. Blood eosinophilia and high levels of IgE were noted. Indirect immunofluorescence was negative for anti-epidermal and anti-basement membrane antibodies. Radiation and chemotherapy for the neoplasia resulted in healing of the bullous lesions and almost complete disappearance of keratotic squamous lesions with residual hyperpigmentation. Discussion. Although bullous lesions are rare in Bazex’s syndrome (acrokeratosis paraneoplastica), they were noted as early as 1968 by Degos et al. These acral bullae mimic autoimmune bullous disease. An immunological reaction is thought to occur with antigens of the dermal-epidermal junction and eosinophils appear to be involved.
文摘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.
文摘Background.We report a case of group B streptococcal septicemia of digestive origin with secondary bilateral breast dermal-hypodermal localization.Case report.A 71-year-old woman with a past history of bilateral breast cancer treated by conservation therapy was hospitalized because of the sudden occurrence of two clearly delimited,inflammatory,dermal-hypodermal cutaneous plaques located on each breast,associated with fever(39°C),4 days after a colonoscopy.Further investigations eliminated carcinomatous mastitis and blood cultures were positive for group B β-hemolytic streptococcus(Streptococcus agalactiae).Histological examination of a sigmoid polyp revealed a tubular adenocarcinoma.Discussion.We report the first documented case of secondary dermal-hypodermal bacterial skin infection(cellulitis)due to group B β-hemolytic streptococcus.The occurrence after colonoscopy examination,chronology of clinical features,bilaterality and positive blood cultures are arguments in favor of the secondary nature of the skin infection process.
文摘Introduction. Eruptive pseudoangiomatosis was first described in children in the form of an acute non-pruritic macular or popular rash that fades on application of a glass test and resolves within several days. Viral aetiology is suspected but has never been demonstrated to date. Observations. We discuss seven cases of infants presenting this disease: 5 boys and 2 girls aged8 days to 16months. The rash presented typical clinical features in all cases and affected the face and limbs in 6 of the 7 subjects. In one child, involvement of the face and back was observed with sparing of the limbs. The rash occurred after an episode of rhinolaryngeal infection in 3 cases and after gastrointestinal infection in 1 case. Spontaneous resolution was seen within 3 to 10 days in 6 patients although a longer course lasting over 9 months was observed in one infant. In another patient, the rash appeared after surgery for mesoblastic nephroma. In one child, a similar rash was seen in both parents. Screening for infectious agents was negative for the two children from whom samples were obtained. Discussion. This series of paediatric cases of eruptive pseudoangiomatosis is characterised by the very young age of one of the children, coexistence of the condition with a renal tumour in another child, the familial nature of the rash in a third child and unusually long disease duration in the final child. However, this series did not allow identification of the causative infectious agent or agents. Probably, as with other syndromes such as Giannotti-Crosti syndrome or “ gloves and socks"” syndrome, eruptive pseudoangiomatosis forms a clinical picture common to a non-specific viral infection.
文摘Introduction. Pellagra is an exceptional disorder in France. The classical description of pellagra associates a photoinduced rash with neurological impairment and intestinal dysfunction. Without adapted treatment, the progression is fatal. Case report. A 62 year-old women developed a photoinduced rash, composed of circular and erythematous elements with pustular edges. She also had panniculitis, peripheral neuropathy, depressive mood and diarrhea. Her medical past was marked by epilepsy treated with sodium valproate and hydantoin. Biological exams revealed lowered plasma levels of vitamins PP, B1, B6 and zinc, secondary to intestinal impairment induced by bacteria proliferating in the small intestine. The rash resolved with vitamin PP and zinc supplementation. The bacterial colonisation was improved by long-term, sequential antibiotics. Discussion. We report a rare clinical form of pellagroid rash. The rash was induced by chronic malabsorption resulting from excessive bacterial proliferation in the diverticules of the small intestine. The antiepileptic treatment could have facilitated vitamin PP et zinc deficiency. Panniculitis was related to the bacterial proliferation. We discuss in this paper the relationship between some vitamin deficiencies, their clinical manifestations and the direct role of intestinal bacterial proliferation in the cutaneous manifestations.
文摘Congenital afibrinogenaemia is a rare genetic disorder transmitted as an autosomal recessive trait and characterized by the complete absence of fibrinogen in the plasma. We report a 41- year-old woman who suffered from congenital afibrinogenaemia and hepatitis C viral infection and presented with ischaemic necrosis and livedo of the toes. Laboratory investigations showed the presence of mixed cryoglobulinaemia and anticardiolipin antibodies. Resolution occurred with plasmapheresis. We discuss the pathophysiology of this unusual condition and review the literature for skin manifestations associated with this rare haemostasis disorder.
文摘Introduction. UVA phototherapy, acitretin and oral corticosteroids are currently the front-line treatment of disseminated cutaneous lichen planus. We studied the efficacy of narrowband UVB therapy in this indication. Patients and methods. We retrospectively studied the dossiers of patients suffering from disseminated cutaneous lichen planus, treated with narrowband phototherapy in the Phototherapy Unit of the University hospital in Montpellier, from May to November of the year 2001. Disseminated lichen planus was defined as lichen involving at least 20p. 100 of the skin surface. Twenty patients were included. UVB were applied thrice weekly using a Philips TL01 cubicle (311313 nm). The protocol was that used for the treatment of psoriasis. We defined 4 types of response: complete response (disappearance of more than 90p. 100 of the lesions), partial response (disappearance of at least 50p. 100) poor response (improvement in 20 to 50p. 100) and failure (less than 20p. 100 reduction in the lesions). Assessment of relapses in the long term was made using a telephone survey among the patients treated or their physicians. Results. Complete response was obtained in 11 out of the 20 patients (55p. 100) and partial response in 4 (20p. 100), corresponding to 75p. 100 of the responders. Response was obtained with a median delay of 3 months, ranging from 2 to 6 months, following a median of 30 sessions (12 to 50) and acumulated dose of UVB of 36± 4.8 joules/cm2. The phototype, gender, age and duration of evolution before treatment did not influence the response. The relapse rate was and estimated 18p. 100 (2/11) 42 months after treatment had been stopped. Discussion. In our opinion, these results underline the efficacy of narrowband UVB in the treatment of disseminated cutaneous lichen planus. They confirm those of earlier studies and are superimposable with those of oral UVA phototherapy.
文摘Background. The presence of inorganic foreign bodies in granulomatous cutaneous lesions is not infrequent. In this paper, we describe the first case of cerium-induced cutaneous granulomatous dermatitis. Case report. A 57-year-old woman seriously burned four years ago presented with papulonodular lesions affecting only the burned areas treated with topical cerium nitrate-silver sulfadiazine cream (Flammacé rium ). Biopsies revealed sarcoidal granuloma associated with exogenous particles. Electron probe X-ray microanalysis demonstrated a high cerium content. Screening for systemic sarcoidosis was negative. The patient was treated with hydroxychloroquine. After four months of follow-up, clinical and histological evidence of decreased infiltrate was noted. Discussion. Cerium nitratesilver sulfadiazine cream (Flammacé rium ) is widely used for the topical treatment of burns. The main effect of cerium is to create superficial calcification, which decreases wound colonization and prevents the formation of granulation tissue (no hypertrophic scar formation) in bur-ns. Prior to our case, no cutaneous side-effects of cerium had been encountered. Inoculation of foreign matter may or may not induce granuloma formation or sarcoidosis in different subjects, according to their immunologic status. The favorable outcome in this case could in fact be due to a change in the pattern of cytokinin production (TH1→ TH2) rather than the effects of hydroxychloroquine therapy.