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M D'Incan

Publications and source records attributed to M D'Incan.

At least 19 recordsLinked to original sources

[Carcinoma cuniculatum: an uneasy diagnosis to suspect in case of chronic osteomyelitis with unsuccessful outcome despite appropriate antibiotic therapy].

INTRODUCTION: The clinical and histological features of cuniculatum carcinoma (CC) are often misleading. CASE REPORT: We report a case of CC of the foot, which was misdiagnosed as osteomyelitis for a two year period and which relapsed 2 months after complete resection. DISCUSSION: The CC has to be evoked in patients with chronic osteomyelitis and torpid wound. The anatomopathologist needs to be aware of the suspected diagnosis.

Amputation, Surgical↗

[Necrobiotic xanthogranuloma without monoclonal gammopathy and with a rapidly fatal outcome].

BACKGROUND: Necrobiotic xanthogranuloma is an extremely rare form of histiocytosis that presents clinically as yellowish infiltrated plaques or nodules. Ocular involvement is seen in over 80% of cases. Histopathology reveals numerous xanthomous histiocytes and collagen necrobiosis. Benign monoclonal gammopathy associated with myeloma is found in 80% of patients, but the course is normally long, with 100% survival at 10 years. CASE REPORT: A 76-year-old man presented skin lesions that subsequently became ulcerated. The clinical appearance and histopathological examination resulted in diagnosis of necrobiotic xanthogranuloma. No monoclonal gammopathy or myeloma was seen. The disease was marked by sensitivity to corticosteroids with failure of other therapies (cyclophosphamide, alpha interferon), onset ofcorticosteroid dependency, iatrogenic Cushing's syndrome and diabetes, which were in part responsible for the infectious complications and subsequent death of the patient. DISCUSSION: Necrobiotic xanthogranuloma is difficult to treat, even in the absence of myeloma or monoclonal gammopathy. Corticosteroids are probably the most efficacious treatment, but can give rise to multiple complications, resulting in this particular case in death of the patient.

Aged↗

[Erosive pustular dermatosis of the leg: role of zinc deficiency?].

BACKGROUND: Erosive pustular dermatosis of the legs is a rare and recently described condition seen in elderly subjects. It is characterised by symmetric erosions of the legs resulting from a confluence of sterile pustules, usually following minor trauma. Treatment with dermal corticosteroids is rapidly effective but is not codified and relapse is common. CASE-REPORTS: Three patients aged respectively 74, 84 and 92 years presented ulcers of the legs and ankles associated with peripheral pustules following minor injury. These pustules were sterile and exhibited a spongiform appearance on histological examination. None of these patients had a prior history of psoriasis. However, all presented zinc deficiency. Treatment with dermal corticosteroids combined with oral zinc gluconate resulted in complete resolution in two of the patients after several days of therapy and proved effective during relapse in the third patient. DISCUSSION: Erosive pustular dermatosis of the legs is a benign dermatosis that is probably under-reported that should be included in the category of inflammatory neutrophilic dermatoses. The cases we present suggest a triggering or enhancing role of zinc deficiency.

Adrenal Cortex Hormones↗

[Relationship between clinical findings of patients with bullous pemphigoid and antigens recognized by their circulating antibasement membrane zone antibodies].

INTRODUCTION: Clinical features and extent of bullous pemphigoid lesions differed widely among patients. The pathogenic role of anti-BPAG2 antibodies has been recently demonstrated. The aim of this study was to analyze the relationship between clinical features of bullous pemphigoid patients and the antigens recognized by their serum. PATIENTS AND METHODS: One hundred and twelve bullous pemphigoid patients were included in this prospective multicenter study. Inclusion criteria were the following: 1) diagnosis of bullous pemphigoid established on the presence of 3 of the 4 clinical features of bullous pemphigoid, histological picture of bullous pemphigoid and positive direct immunofluorescence; 2) serum available for immunoblotting studies. The clinical and biological findings were prospectively recorded on standard forms. Sera were collected and analyzed using indirect immunofluorescence and immunoblotting on human epidermal extracts. RESULTS: Analysis of patient's clinical features depending on the antigens recognized by their serum showed that patients whose serum contained anti-BPAG1 antibodies had more frequently pruritus, blisters on the lower limbs and a positive indirect immunofluorescence. Patients whose serum contained anti-BPAG2 antibodies had blisters more frequently localized on the head, and a more frequently negative indirect immunofluorescence. Patients whose serum was negative by immunoblotting had less frequently urticarial and/or eczematous lesions, bullae less frequently localized on the lower part of the trunk, abdomen and lower limbs, lower eosinophilia and a more frequently negative indirect immunofluorescence. CONCLUSION: Patients with circulating anti-BPAG1 antibodies exhibited the most typical, clinical and biological features of bullous pemphigoid.

Aged↗

[Phase 2 clinical study of 123I-N-(2-diethylaminoethyl)-2-iodobenzamide in the diagnostic of primary and metastatic ocular melanoma].

PURPOSE: Iodobenzamides are reported to possess an affinity for melanoma. A first selected compound, BZA, was studied in a phase 2 clinical trial on 159 patients as an imaging agent for the detection of primary melanoma and metastases with good results. We report the results of a second phase 2 clinical trial on 40 patients with a new radiopharmaceutical BZA2 (an orthoiodinated BZA analog), which was expected to provide quality images sooner after injection and with better imaging contrast. PATIENTS AND METHODS: Performance was evaluated in 40 patients classified with primary ocular lesions (12), suspicion of metastases of ocular or cutaneous origin (15), or with no known secondary lesion (13), and results were compared with conventional investigation techniques (ophthalmoscopy, ultrasonography, and angiography for ocular melanoma, whole-body CT scan and ultrasonography for metastases). RESULTS: No adverse events were recorded. The overall results on a per patient basis showed a sensitivity of 78% and a specificity of 95%. The four false negatives observed were ocular lesions (three with a thickness<3mm and one achromic), but all the proven secondary lesions were imaged. Moreover, negative BZA2 scintigraphy in cases of suspicious lesions led to the correction of two diagnoses: the prostatic origin of bone metastases and the endocrine tumor origin (APUD system) of an ocular lesion. DISCUSSION: BZA2 scintigraphy is an easy test with good tolerance. In the diagnosis of ocular primary melanoma, the sensitivity of the test is 64%, although limited by the thickness (3mm) and the pigmentation of the lesion. However, the BZA2 scintigraphy is a very useful test for the detection of melanoma metastases, with a sensitivity of 100% and a specificity of 95%. CONCLUSION: BZA2 scintigraphy showed good tolerance in patients and it appears promising for differential diagnosis, staging, and restaging of melanoma.

Benzamides↗

In vivo and in situ modulation of the expression of genes involved in metastasis and angiogenesis in a patient treated with topical imiquimod for melanoma skin metastases.

There is a growing body of evidence to support the efficacy of topical imiquimod in the treatment of primary skin carcinomas. Conflicting data exist concerning the use of imiquimod for the treatment of skin melanoma metastases. To date, only the impact of imiquimod on cytokines involved in immunological processes has been studied extensively. We report a woman successfully treated with imiquimod (once daily for 8 weeks) for skin melanoma metastases in whom we investigated the expression of molecules involved in metastasis and angiogenesis. Before and after treatment, a skin lesion was biopsied and the expression of the following molecules was investigated using real-time reverse transcription-polymerase chain reaction: matrix metalloproteinase (MMP)-1, 2 and 9 and their inhibitors KiSS-1 and tissue inhibitor of metalloproteinase (TIMP)-1, vascular endothelial growth factor (VEGF), fibroblast growth factor-2, and angiogenesis inhibitors (thrombospondin-1 and 2). Interferon (IFN)-alpha was also investigated as an in vivo marker of imiquimod activity. IFN-alpha was upregulated by the treatment. Under imiquimod, the following molecules were upregulated: TIMP-1, KiSS-1 and MMP-1. MMP-2 expression was not modified. MMP-9 expression was dramatically decreased. The expression of angiogenesis inhibitors was slightly increased but VEGF expression remained at a basal level. These results suggest that imiquimod could downregulate metastasis invasion and angiogenesis. However, these data were obtained at a transcriptional level and from a single case, and further investigations should include migration assays and additional cases in order to confirm that imiquimod may be safely used for treatment of melanoma metastases.

Administration, Topical↗

Generalized pustular psoriasis complicated by acute respiratory distress syndrome.

Psoriasis has a chronic and relatively benign course. However, severe complications are possible. One rare complication is acute interstitial pneumonitis. This entity should be suspected when a patient presents with dyspnoea and high fever. Knowledge of this pathology is crucial, for although it is essential to rule out aetiologies requiring specific management such as microbial infection or drug-related syndromes, diagnosis should not be delayed as its severe clinical course is improved by corticosteroids. We report two patients with an acute respiratory distress syndrome arising during the course of pustular psoriasis. Repeated bacteriological testing in lungs and blood remained negative. In both cases lung involvement was severe, requiring artificial ventilation. Dramatic clinical resolution was obtained by using corticosteroids. Besides infectious causes and drug hypersensitivity to methotrexate or acitretin, acute respiratory distress syndrome, sometimes due to a pulmonary capillary leak syndrome, is a rare cause of pneumonitis in the course of psoriasis, and may be fatal. Its pathogenesis is unknown. However, animal models suggest a role for T-helper (Th) 1 lymphocytes, known to be activated in psoriasis, and a role for tumour necrosis factor-alpha, a major Th1 cytokine, in alveolar damage.

Adult↗

Clinical criteria for the diagnosis of bullous pemphigoid: a reevaluation according to immunoblot analysis of patient sera.

BACKGROUND: We previously proposed a set of 4 clinical criteria for the diagnosis of bullous pemphigoid (BP) that consisted of age greater than 70 years, absence of atrophic scars, absence of mucosal involvement and absence of predominant bullous lesions on the neck and head. These results have been challenged because direct immunoelectron microscopy (IEM), which was used as the standard diagnostic criterion in our initial study, does not identify the different antigens of the basement membrane zone. OBJECTIVE: To reassess the validity of these clinical criteria for the diagnosis of BP using immunoblot analysis of patient sera as the main diagnostic criterion, in order to precisely identify the antigens recognized by patient sera. METHODS: One hundred and eighty-nine sera from patients with various subepidermal autoimmune blistering diseases (AIBDs) were tested by immunoblotting using dermal and epidermal extracts. IEM was used as a complementary diagnostic procedure in a few patients whose serum recognized BPAG2 exclusively or was negative in immunoblotting. RESULTS: 142 patients (75%) had at least 3 of the 4 clinical diagnostic criteria. Sera from patients who lacked the set of BP clinical criteria were more frequently immunoblot negative (34%) than sera from patients who had the criteria (18%; p = 0.025). BPAG1 was more frequently recognized by sera from patients with the set of BP clinical criteria (78%) than by sera from patients without the criteria (45%; p = 5.10(-4)). In contrast, BPAG2 was recognized by a great number of sera from patients who lacked the criteria of BP (71%), which was in accordance with the presence of numerous patients with cicatricial pemphigoid in this group. Among patients with various subepidermal AIBDs, the diagnosis of BP could be made with a sensitivity of 86%, a specificity of 90% and an excellent prognostic positive value over 95%, if 3 of these clinical criteria were present. CONCLUSION: These results confirm the interest of this set of clinical criteria for the rapid diagnosis of BP.

Aged↗

Epidemiological survey of melanoma in the Auvergne region (France): is there an increased incidence in Auvergne?

To assess the efficiency of melanoma screening and prevention campaigns in the Auvergne region (France), cases of melanoma have been recorded since 1st June 1998. The epidemiological follow-up of melanoma was carried out using two sentinel networks; one involving the pathologists, and the other, the dermatologists of the region. Incidence was calculated using the capture-recapture method, by cross-matching the data supplied by both dermatologists and pathologists. Between June 1st 1998 and December 31st 2000, 363 cases of melanoma were recorded. The crude incidence rate of melanoma per 100,000 person-years was 17.1 for all melanomas and 14.6 for invasive melanomas. These rates of incidence were higher than the estimated national rate of France, and were close to incidences found in countries of Northern Europe. This might be explained by an increase in screening for melanoma, by more precise estimation of the incidence due to the capture-recapture method, or by geographic factors (mountainous area). An answer may be provided by following the variation in time of incidence and thickness of melanomas; the increase in the number of thin (low Breslow index) melanomas corresponding with increased screening.

Adolescent↗

Cutaneous lymphoma associated with Epstein-Barr virus infection in 2 patients treated with methotrexate.

Whether patients with rheumatoid arthritis (RA) have an increased risk of developing non-Hodgkin lymphoma is controversial, and opinions differ on the possible role of methotrexate in the occurrence of lymphomas in patients with RA. We report 1 T-cell lymphoma and 1 B-cell lymphoma restricted to the skin associated with Epstein-Barr virus infection that healed completely and spontaneously after discontinuation of methotrexate in a man with RA and a woman with dermatomyositis. Cutaneous infiltrating cells were infected by a replicative form of Epstein-Barr virus. After discontinuation of methotrexate, the cutaneous lesions disappeared completely in 15 days without recurrence. Discontinuation of methotrexate is necessary in patients with RA or dermatomyositis who have a lymphoproliferative disorder, and a follow-up period of several weeks should be observed before specific therapy is initiated.

Aged↗

[Value of abdominal-pelvic computed tomography in adult rheumatoid purpura].

PURPOSE: Henoch-Schonlein purpura is a rare eventuality in adulthood. Abdominal involvement can worsen the short-term prognosis and justify medical treatment to avoid surgery. METHODS: Abdominopelvic computed tomography (CT) was performed in six adult patients (four men and two women; age range, 19-74 years) with Henoch-Schonlein purpura before any treatment. Abdominal symptoms were concomitant with the purpuric rash in two cases and followed it in the other cases. RESULTS: The small bowel was always involved with a parietal thickening, and the target sign was observed in three patients. The lesions were located in the duodenum in two patients, the jejunum in two and the ileum in five. Two segments were involved in one patient and three segments in another patient. A peritoneal effusion was always present. No colonic lesion was discovered on CT. Once the results were known, all patients received steroids with initially intravenous methylprednisolone in five patients at doses ranging from 80 to 1,000 mg/day. On follow-up, none of the patients underwent surgery. CONCLUSION: Abdominopelvic CT scan demonstrated signs suggestive of intestinal vasculitis. An underlying neoplasia or an eventual other cause of abdominal pain were explored and a steroid therapy was then began with a good outcome. Abdominal CT can be helpful in the diagnosis of abdominal involvement in Henoch-Schonlein purpura.

Abdominal Pain↗

[Cutaneo-systemic papulosclerotic mucinosis (scleromyxedema): remission after extracorporeal photochemotherapy and corticoid bolus].

INTRODUCTION: Scleromyxedema is the consequence of a dermal infiltration by mucine, commonly associated with a monoclonal gammapathy of unknown significance. CASE REPORT: A 46 year-old woman was hospitalized for a scleromyxedema with a bilateral macular edema and a restricted pulmonary syndrome. A quite complete cutaneous response and a complete ocular and pulmonary response were obtained after 12 extracorporeal photopheresis courses and 4 flashes of prednisolone (17 months follow-up). COMMENTS: There is no consensus on guidelines for the treatment of scleromyxedema. Steroids and melphalan are usually indicated. However these drugs induce severe side-effects. In the absence of controlled studies concerning the efficiency of the different drugs used in scleromyxedema, the main advantage of photopheresis lies in the safety of this procedure.

Combined Modality Therapy↗

[Acquired angioneurotic edema. Clinical and biological characteristics in 9 patients].

OBJECTIVE: Angioneurotic edema (AE) is a rare but severe disease. Hereditary AE is the more well-known form. The acquired form is exceptional: the symptoms are the same but there are some biologic and treatment differences. We investigated the clinical and biochemical features in nine patients with acquired angioneurotic edema (AAE). PATIENTS AND METHODS: Four of the patients with type I AAE presented an accelerated metabolism of C1Inh, associated with a hematology disease. Their C4, C1q and C1Inh plasma levels were low. Four patients had type II AAE associated with an autoantibody to C1Inh. Their C1Inh plasma levels were normal or low but the functional levels were low in all four. One patient had AAE induced by oral contraceptives. The C1Inh plasma level was normal but the functional level was very low; there were no autoantibodies. Symptoms resolved when oral contraceptives were withdrawn and the C1Inh level returned to normal. DISCUSSION: Treatment of AAE is a difficult matter. For type I AAE, it consists in treating the associated disease. For type II AAE, the treatment goal is to lower the autoantibody level. Management of these diseases requires close collaboration between clinicians and biologists.

Adult↗

Prognostic factors in primary cutaneous lymphomas other than mycosis fungoides and the Sézary syndrome. The French Study Group on Cutaneous Lymphomas.

Prognostic studies of primary cutaneous lymphomas (PCL) other than mycosis fungoides (MF) and the Sézary syndrome (SS; non-MF/SS PCL) have been mainly performed on subgroups or on small numbers of patients by using univariate analyses. Our aim was to identify independent prognostic factors in a large series of patients with non-MF/SS PCL. We evaluated 158 patients who were registered in the French Study Group on Cutaneous Lymphomas database from January 1, 1986 to March 1, 1997. Variables analyzed for prognostic value were: age; sex; type of clinical lesions; maximum diameter, location, and number of skin lesions; cutaneous distribution (ie, local, regional, or generalized); prognostic group according to the European Organization for Research and Treatment of Cancer (EORTC) classification for PCL; B- or T-cell phenotype; serum lactate dehydrogenase (LDH) level; and B symptoms. Univariate and multivariate analyses were performed using a model of relative survival. Forty-nine patients (31%) died. The median relative survival time was 81 months. In univariate analysis, EORTC prognostic group, serum LDH level, B symptoms, and variables related to tumor extension (ie, distribution, maximum diameter, and number of skin lesions) were significantly associated with survival. When these variables were considered together in a multivariate analysis, EORTC prognostic group and distribution of skin lesions remained statistically significant, independent prognostic factors. This study confirms the good predictive value of the EORTC classification for PCL and shows that the distribution of skin lesions at initial evaluation is an important prognostic indicator.

Adolescent↗

Acquired angioedema with C1 inhibitor deficiency: is the distinction between type I and type II still relevant?

BACKGROUND: Acquired angioedemas are divided into type I associated with lymphoproliferation and type II caused by anti-C1-inhibitor antibodies. Recent reports have suggested that this distinction is not so clear-cut, mainly because of the presence of antibodies against the C1 inhibitor in some cases belonging to the type I group. We report herein 2 additional cases of acquired angioedema with anti-C1-inhibitor antibody. MATERIAL AND METHODS: One man and 1 woman had had acquired angioedema for several years. In the man, a monoclonal component had been detected several years before the present study. In the second patient, a monoclonal component was detected during the study. The following data were studied on successive blood samples collected during angioedema manifestations: complement component levels, functional activity of the classical pathway, functional and antigenic C1 inhibitor doses, ELISA test to detect autoantibodies to C1 inhibitor and Western blot analysis of the C1 inhibitor. RESULTS: In both patients, CH50 and C4 activities were decreased, and an autoantibody to C1 inhibitor was detected. In 1 case, the antibody appeared after the monoclonal component; in the second case, it appeared before and belonged to a different immunoglobulin class. CONCLUSION: Our data suggest that the distinction between type I and type II acquired angioedema is no longer valid because of overlapping in some cases.

Aged↗