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C Lok

Publications and source records attributed to C Lok.

At least 19 recordsLinked to original sources
PubMed2006

Lhermitte-Duclos disease and Cowden's syndrome. Report of two cases.

OBJECTIVE AND IMPORTANCE: Lhermitte-Duclos disease, or dysplastic cerebellar gangliocytoma is a rare entity characterized by a hamartomatous lesion in the posterior fossa. Cowden's syndrome, or hamartoma-neoplasia syndrome is a rare underdiagnosed autosomal dominant genodermatosis with high incidence of malignant tumors. Several recent reports suggest that Lhermitte-Duclos disease may be a component of Cowden's syndrome. CLINICAL PRESENTATION: We report two cases of Lhermitte-Duclos and Cowden disease occurring in adult patients. A 40-year-old woman had symptoms of raised intracranial pressure and macrocephaly. She displayed the stigmata of fibrocystic breast disease, thyroid goitre. Clinical examination showed mucocutaneous lesions. Her mother, brother and uncle had manifestations of Cowden's disease. An asymptomatic 38-year-old male had bilateral optic nerve drusen related to a cerebellar neoplasm. He exhibited manifestations of Cowden's syndrome and his familial history confirmed this hypothesis. INTERVENTION: The first patient was operated on for Lhermitte-Duclos disease. A conservative strategy was performed for the second patient and the clinical and imaging follow-ups were uneventful over 5 years. CONCLUSION: We stress the possibility that Lhermitte-Duclos and Cowden disease might be a peculiar form of phakomatosis. A conservative strategy can be chosen without neurological signs because of slow tumor growth. However, these patients should be carefully examined and followed up because of the risk of future malignancy.

Adult↗
PubMed2006

[Myxofibrosarcoma associated with anetoderma].

BACKGROUND: Association of malignant cutaneous tumor and secondary anetoderma is rare. Secondary anetoderma in myxofibrosarcoma has not been described to date. We report a case below. CASE REPORT: A 80-year-old woman presented with a 40 x 40 mm, round, flesh-colored lesion on her left buttock. Physical examination showed a soft, protuberant lesion, without firm underlying subcutaneous mass. Pathologic examination of the surgical specimen revealed a myxofibrosarcoma, with focal loss of elastic fibers in the overlying dermis. There was no evidence of systemic involvement. One year later, she developed a recurrent tumor at the same site, with similar clinical presentation, which was treated by broad excision. DISCUSSION: Secondary anetoderma is usually seen in association with cutaneous infections and benign skin tumors. An anetodermic presentation of myxofibrosarcoma has not been reported to our knowledge. Myxofibrosarcoma (formerly referred to as myxoid malignant fibrous histiocytoma) is characterized by an abundant myxoid background in at least one half of the tumor. The tumor recurs in almost two-thirds of cases and metastasizes in one-fourth. Our case confirms that a unique, acquired anetodermic lesion can reveal a malignant tumor. A large deep biopsy should be performed systematically when this variety of anetoderma is observed.

Aged, 80 and over↗
PubMed2005

Post-transplant cutaneous T-cell lymphomas.

Post-transplant cutaneous lymphomas are rare. Cutaneous T-cell lymphomas account for 30% of these lymphomas. The clinical appearance of the skin lesions is identical to cutaneous lymphomas observed in non-immunosuppressed patients, with infiltrated plaques, nodular and ulcerated tumors, but with an increased frequency of erythroderma. Standard histology and immunohistochemistry are also consistent with the features of mycosis fungoides and CD30+ cutaneous lymphomas observed in the general population. However, the pronostic differs from the usually favourable outcome of cutaneous T-cell lymphomas, as 8 out of the 13 patients of our series died, in less than 1 year for 6 of them. This unfavourable course appears to be the same as that observed for systemic T-cell lymphoma in transplant recipients. In contrast to post-transplant B-cell lymphomas (systemic and primary cutaneous), the link to a virus has not been demonstrated. The prognosis is also less favourable for post-transplant cutaneous T-cell lymphomas than for post-transplant cutaneous B-cell lymphomas.

Diagnosis, Differential↗
PubMed2005

Venous leg ulcer: a meta-analysis of adjunctive therapy with micronized purified flavonoid fraction.

OBJECTIVE: To assess the effect of oral treatment with micronized purified flavonoid fraction (MPFF) on leg ulcer healing. DESIGN: Meta-analysis of randomised prospective studies using MPFF in addition to conventional treatment. MATERIALS AND METHODS: Five prospective, randomised, controlled studies in which 723 patients with venous ulcers were treated between 1996 and 2001 were identified. Conventional treatment (compression and local care) in addition to MPFF was compared to conventional treatment plus placebo in two studies (N = 309), or with conventional treatment alone in three studies (N = 414). The primary end point was complete ulcer healing at 6 months. RESULTS: At 6 months, the chance of healing ulcer was 32% better in patients treated with adjunctive MPFF than in those managed by conventional therapy alone (RRR: 32%; CI, 3-70%). This difference was present from month 2 (RRR: 44%; CI, 7-94%), and was associated with a shorter time to healing (16 versus 21 weeks; P = 0.0034). The main benefit of MPFF was present in the subgroup of ulcers between 5 and 10 cm2 in area (RRR: 40%; CI, 6-87%), and those present for 6-12 months duration (RRR: 44%; CI, 6-97%). CONCLUSION: These results confirm that venous ulcer healing is accelerated by MPFF treatment. MPFF might be a useful adjunct to conventional therapy in large and long standing ulcers.

Administration, Oral↗
PubMed2005

[Bullous pemphigoid in young patients: a retrospective study of 74 cases].

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 basement membrane 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.

Adult↗
PubMed2005

[Topical silver sulfadiazine-induced acute renal failure].

INTRODUCTION: Prolonged topical application of silver sulfadiazine cream can induce argyria and adverse effects of sulphonamides. We report a case of a woman with acute renal failure following repeated applications of topical silver sulfadiazine on pyoderma gangrenosum wounds. CASE REPORT: A 61 year-old woman suffering from rheumatoid arthritis, Sjogren's syndrome and scleroderma was treated with corticosteroids (1 mg/kg/day) and topical application of silver sulfadiazine cream (200 g/day) for extensive pyoderma gangrenosum wounds on the legs. Three weeks later, the patient was transferred to intensive care because of pulmonary edema, oligoanuria and disrupted consciousness. Laboratory data revealed leukopenia (1100/mm(3)) with neutropenia and renal failure (serum creatinine 316 micromol/l). Proteinuria was moderate and ultrasonography of the kidneys was normal. Silver concentration in blood was 1818 nmol/l (N < 92 nmol/l) and 1381 nmol/l (N < 9 nmol/l) in urine. Sulfadiazine concentration in blood was undetectable. All the signs regressed after withdrawal of silver sulfadiazine and after several sessions of hemodialysis. DISCUSSION: Various causes of renal failure are discussed in our patient. However, direct silver-induced renal toxicity is the most likely and is confirmed by the high concentration of silver in blood and urine and the improvement on withdrawal of the topical cream, without modification in the oral treatment. The absence of red corpuscles and crystals in the urine and undetectable concentrations of sulfadiazine in blood are not in favor of sulphonamide renal toxicity. Furthermore, the autoimmune diseases of our patient were well-controlled. Leukopenia could be secondary to silver sulfadiazine medullar toxicity. This observation confirms that this topical cream should not be used for long periods on extensive wounds.

Acute Kidney Injury↗
PubMed2005

Clinical variants of the preprotuberant stage of dermatofibrosarcoma protuberans.

BACKGROUND: Some cases of dermatofibrosarcoma protuberans (DFSP) do not protrude above the skin. OBJECTIVES: To assess the prevalence of these DFSPs and further to describe their presentation and course. METHODS: One hundred and forty-three patients were retrospectively collected. They were asked to complete a standardized questionnaire indicating the history and appearance of the DFSP from the first skin changes identified to the time of diagnosis. RESULTS: Eighty-one DFSPs were described as protuberant ab initio, and 62 as initially nonprotuberant (npDFSP). The latter remained at this stage for a mean period of 7.6 years. Twenty-nine per cent of npDFSPs were 'morphoea-like', 19% were 'atrophoderma-like' and 42% were 'angioma-like'. Age at diagnosis was similar for both initial presentations. npDFSPs were most often misdiagnosed by physicians. CONCLUSIONS: Nearly half the patients first identified their early DFSP-related skin changes as patches. Both this frequency and the long duration at this preprotuberant stage should prompt dermatologists to consider the diagnosis of DFSP earlier, in order to make surgical treatment easier.

Adolescent↗
PubMed2004

[Angioedema and angiotensin converting enzyme inhibitors: a report of 19 cases].

PURPOSE: Inhibitors of angiotensin-converting enzyme are worldwide used and are a real progress for the treatment of systemic hypertension or cardiac failure and are a real progress. The most common adverse side effect is cough. Angioedema is a sudden and localized edema involving the deeper cutaneous and mucosa tissue lappers. 0.1-0.5% of patients treated by ACE inhibitors could develop angioedema. MATERIALS AND METHODS: We report a series of 19 cases, recruited in the Parmacovigilance Center of the University Hospital of Amiens from 1997 to 2003. RESULTS: All the patients had a facial swelling edema at initial presentation. Intestinal mucosa or preputial are misleading localisations. In 1/3 of cases, angioedema appeared after the first administration, in 1/3 of cases it appeared with a delay of 1-2 years. The oropharynx localisation with glottic involvement may need an airway intervention. One patient died from pulmonary distress at home. CONCLUSION: The pathophysiology is current unknown although there is increasing evidence for bradykinin accumulation involvement. The treatment by ACE inhibitors must be broken off; angiotensin II antagonists may be an alternative treatment, but has to be introduced carefully.

Adult↗
PubMed2004

[The Carney complex].

Explore the source record for details and available documents.

Endocrine System Diseases↗
PubMed2004

[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↗
PubMed2004

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↗
PubMed2003

[Cutaneous T-cell lymphoma following renal transplantation].

INTRODUCTION: Post-transplant cutaneous lymphomas are the second skin cancer after cutaneous carcinoma and are usually of the B-cell type. Post-transplant cutaneous T-cell lymphomas are extremely rare. We described a case of a cutaneous T-cell lymphoma in a renal transplant recipient. CASE REPORT: A 52-year-old woman was hospitalized for an erythematous infiltrated eruption. Seven years earlier, she had undergone kidney transplantation. No palpable lymphadenopathy or hepatosplenomegaly was present. The patient's skin biopsy specimen was histologically suggestive of CD30- fungoid mycosis. The same clonal TCR-rearrangement was identified in the blood and in the skin. No EBV was detected within the cutaneous lesion on immunohistochemical analysis or by PCR in the blood. Chlorambucil (Chloraminophène) was associated with a topical treatment with chlormethine (Caryolysine) and corticosteroids while tacrolimus (Prograf) was reduced and stopped. There was no evidence of recurrence of the lymphoma after 12 months of follow-up. DISCUSSION: The particularity of our observation is the apparition, 7 years after transplantation, of a CD30-, EBV-fungoid mycosis with a blood and cutaneous clonal TCR-rearrangement. Despite this poor prognosis factor, the cutaneous lymphoma regressed after reduction of the immunosuppressive treatment reduction and institution of topical corticosteroids, chlormethine and chlorambucil.

Female↗