PubMed Health⌕ Search

Biomedical subjects

D Lipsker

Publications and source records attributed to D Lipsker.

At least 19 recordsLinked to original sources

Which complement assays and typings are necessary for the diagnosis of complement deficiency in patients with lupus erythematosus? A study of 25 patients.

INTRODUCTION: Deficiencies in components of the classical pathway of complement activation are strong risk factors for lupus erythematosus (LE).Yet, it has not been addressed whether the conventional measurements of the serum hemolytic CH50 activity and antigenic concentrations of C3 and C4 are sufficient to asses a deficiency in C4A, C4B or C2 components, the most common deficiencies associated with LE. PATIENTS AND METHODS: In a retrospective series, we performed complement analyses in 35 patients with LE who were systematically screened for a complement deficiency. The majority of patients had cutaneous LE with mild systemic involvement and no complement consumption. Of 25 patients (72%) with complement deficiency we found 13 with a partial C4A deficiency, 2 with a complete C4A deficiency, 6 with a partial C4B deficiency, 2 with a complete C4B deficiency and 2 with a combined partial C2 and C4A deficiency. RESULTS: The total complement activity (CH50) was decreased in only one out of two patients with complete C4B deficiency. CH50 level was found to be low-normal (35-38 U/ml(-1)) in one patient with partial C4B deficiency, one patient with complete C4B deficiency and both patients with combined partial C4A and C2 deficiency. Total C4 levels were normal in 9 out of 13 the patients with a partial C4A deficiency and in 2 out of 6 patients with a complete C4B deficiency. The antigenic concentration of C3 was low in only 1 patients with a complete C4B deficiency and within the normal range in all the others patients. Overall, 50% of the patients had normal or elevated C3, C4, and CH50 levels. DISCUSSION: This study emphasizes that the usual measurements of CH50, C3 and C4 levels are not adequate to detect a C4 and/or C2 deficiency in patients with LE. In epidemiologic or investigative studies addressing the prevalence of complement deficiency, more elaborated diagnostic tests, such as C4 protein allotyping, C2 level measurement and genetic screening for type I C2 deficiency should also be performed.

Adult↗

Pyoderma gangrenosum as initial manifestation of Graves' disease.

Pyoderma gangrenosum (PG) is an uncommon ulcerative condition. It is most commonly associated with inflammatory bowel disease, haematological malignancies and autoimmune disease. We report on two patients have developed PG during the initial course of Graves' disease (GD), prior to treatment of this condition. The lesions completely cleared with treatment by colchicine in the first case and with oral ciclosporin in the second case. The report emphasizes that a diagnostics of PG should be considered in patients with GD and skin ulcers.

Administration, Oral↗

The combination of complement deficiency and cigarette smoking as risk factor for cutaneous lupus erythematosus in men; a focus on combined C2/C4 deficiency.

BACKGROUND: Although deficiencies in the early components of the complement system were among the first identified genetic risk factors for systemic lupus erythematosus (SLE), only a few studies addressed their significance in patients with cutaneous LE (CLE). Among environmental factors, it was postulated that cigarette smoking might intervene in the pathogenesis of LE. OBJECTIVES: To describe the clinical and biological features of patients with CLE and a complement deficiency. A secondary objective was to assess cigarette smoking in patients with CLE. PATIENTS AND METHODS: A retrospective study including all patients diagnosed as having LE between 1995 and 2003 in the Dermatology Department of Strasbourg University Hospital. Patient charts were reviewed and those patients in whom a C4 and/or C2 deficiency was diagnosed were included. Two patients with a combined C2/C4 deficiency were analysed in detail. RESULTS: There were 48 females and 37 males (F/M ratio = 1.3), with a mean age of 41 years at diagnosis; 73% of the patients had chronic LE and 27% subacute CLE. Among 32 screened patients, 24 patients with a mean age of 36 years had a complement deficiency; 17 had a C4A deficiency, five a C4B deficiency and two a combined C4A/C2 deficiency. A high proportion (58%) of these patients was male; 82% of the patients were smokers. This was especially true in males: 94% were smokers compared with 69% of females. CONCLUSIONS: Partial deficiency of C4, C2 or C4 and C2 is a common finding in patients with CLE. Most male patients with CLE are smokers. It is thus suggested that the combination of cigarette smoking and complement deficiency could be a risk factor for LE in men.

Adolescent↗

[Primary cutaneous non T non B CD4+ CD56+ leukemia (2 cases): an original anatomoclinical syndrome].

INTRODUCTION: Non T non B CD4+ CD56+ leukemia is often revealed by cutaneous lesions. We report 2 patients with this disorder who had characteristic anatomoclinical findings. CASE REPORTS: An 81 year-old female and a 75 year-old man presented with erythematous macules which increased in number and progressed to infiltrated plaques and nodules. The lesions became ecchymotic, but the patients remained in good general condition and blood count as well as bone marrow examination were unremarkable. A cutaneous biopsy revealed a lymphomatous mononuclear cell infiltrate. The cells expressed CD4 and CD56, but not CD3. The cutaneous lesions preceded for 10 and 9 months respectively the appearance of overt leukemia and medullar involvement. At this stage, the patients deceased rapidly from their leukemia. DISCUSSION: This is an original anatomoclinical syndrome. The histopathologist must pay attention to the unusual CD4+ and CD3- immunophenotype and search for CD56 expression. The malignant cell responsible for this type of leukemia is now individualized and corresponds to a type II dendritic cell precursor.

Aged↗

[Generalized granuloma annulare and drug-induced immunodeficiency].

BACKGROUND: Granuloma have already been described in the context of immunosuppression. We report six cases of widespread granuloma annulare occurring in patients with drug-induced immunosuppression. CASE REPORTS: There were four women and two men, with mean age of 61 years (35-76). Three patients were treated with chemotherapy for breast (2 cases) and liver cancer. One woman was given chemotherapy for Hodgkin's disease. One had undergone liver transplantation and was given cyclosporine, another was treated with systemic steroids for polyarthritis. All had generalized non-photoexposed granuloma annulare, composed of multiple erythematous papules, sometimes with annular pattern. The lesions were localized on the trunk, legs and arms. Histopathology revealed granuloma annulare in each patient. DISCUSSION: These six cases suggest a relationship between drug-induced immunodeficiency and generalized granuloma annulare. The immune dysregulation induced by the drugs may have been responsible for the formation of granuloma annulare.

Adult↗

[The level of knowledge of general practitioners regarding the early phase of Lyme borreliosis. Survey conducted among 106 general practitioners].

OBJECTIVE: Determine the level of knowledge of the general practitioners on the early phase of Lyme borreliosis in an endemic area and notably to define the attention paid to the use and interpretation of serologic investigations for the treatment of patients with erythema migrans. METHOD: This was a prospective study conducted from May 15 to June 31, 2001 among 106 randomly selected general practitioners installed in Strasbourg, France. The practitioners were all interviewed in their private practice and by the same person. Three standardized questions were asked, evaluating their level of specific medical training on Lyme borreliosis as well as their practice regarding treatment and diagnosis of erythema migrans. RESULTS: One third of the general practitioners had already accomplished a continued medical education course on Lyme borreliosis. Half of them considered that the diagnosis of erythema migrans was clinical, while the other half believed that serological confirmation was mandatory. However, the answers of those having received specific education on borreliosis were right (p=0.0079) since in this sub-group 72% considered that the diagnosis was exclusively based on clinical examination, versus 41% of the untrained physicians. Eighty-eight percent used the recommended antibiotic regimens to treat erythema migrans. Three practitioners proposed an inefficient treatment that had exposed the patients to the risk of extra-cutaneous complications of the disease. CONCLUSION: Fifty percent of the general practitioners working in endemic areas for Lyme borreliosis still believe that seropositivity against Borrelia burgdorferi is required to diagnose erythema migrans, which is untrue. However, this study shows that a specific education on Lyme borreliosis would significantly improve this score.

Adult↗

Hypereosinophilia-induced digital necrosis in a smoking patient.

Raynaud's phenomenon and digital necrosis of the fingers are rare complications of hypereosinophilia. We report a case of a smoking male who developed Raynaud's phenomenon and digital necrosis of the fingers associated with idiopathic hypereosinophilia with angiographically documented occlusion of small and medium arteries of the extremities. The eosinophils may play a thrombotic role in vascular pathology and eosinophil blood count should be checked when investigating patients with digital gangrene.

Adult↗

[Allergic vasculitis following ingestion of celecoxib?].

A 76-year-old patient developed cutaneous vasculitis on the lower legs on the 8th day of treatment with the selective cox-2-inhibitor celecoxib (Celebrex((R))) and the proton-pump inhibitor omeprazole. The patient had no history of allergic reactions. The patient had already been treated previously with omeprazole without any side effects. A cutaneous biopsy confirmed the diagnosis of leucocytoclastic vasculitis. The purpuric skin lesions regressed within 3 weeks after withdrawing the two newly introduced drugs. We discuss the potential role of Celecoxib in triggering this vasculitis.

Administration, Oral↗

Long-term prognosis of patients treated for erythema migrans in France.

BACKGROUND: The long-term prognosis of patients treated for erythema migrans has only rarely been assessed. OBJECTIVES: To evaluate the clinical characteristics and long-term prognosis of patients treated for erythema migrans in the region of Alsace, France. METHODS: In a prospective study, 56 consecutive patients presenting with erythema migrans at the Strasbourg University Hospital between 1995 and 1999 were examined and a Borrelia burgdorferi enzyme immunoassay was performed. Patients were treated with tetracyclines or amoxycillin. Patients were re-examined 6 weeks later and a telephone interview was performed in summer 2000 to evaluate the long-term outcome. RESULTS: There were 25 women and 31 men of mean age 49 years presenting with single (n = 54) or multiple (n = 2) erythema migrans lesions. At the time of diagnosis, 30% of the patients had systemic signs, myalgias or arthralgias and only 36% of 50 patients were seroreactive against B. burgdorferi. None of the 51 patients evaluated at 6 weeks and none of the 37 patients interviewed after a median delay of 3 years had developed complications attributable to Lyme borreliosis. CONCLUSIONS: The prognosis of patients treated for Lyme borreliosis in this part of France is excellent. Therefore, a complete clinical examination is sufficient as an initial evaluation and long-term follow-up is not necessary.

Adolescent↗