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Biomedical subjects

D Lipsker

Publications and source records attributed to D Lipsker.

At least 37 records · Page 2Linked to original sources
PubMed2000

Schnitzler syndrome: heterogeneous immunopathological findings involving IgM-skin interactions.

The Schnitzler syndrome is the association of chronic urticaria, intermittent fever, osteosclerotic bone lesions and a monoclonal IgM gammopathy. It is not yet firmly established whether the monoclonal immunoglobulin component plays a part in the pathophysiology of the urticarial lesions. Immunoblotting on epidermal and dermal human skin extracts as well as immunoelectron microscopic (IEM) studies on Lowicryl K4M-embedded skin sections were performed in three patients with the Schnitzler syndrome. Western blotting on epidermal extracts showed the presence of IgM-kappa antiskin autoantibodies in two patients. These antibodies displayed the same isotype as the monoclonal components and recognized a 280-290-kDa antigen in one patient and a 200-kDa antigen in the other patient. IEM studies showed sparse IgM deposits in the epidermis, around the keratinocytes, near the desmosomes in one patient and dense deposits below the lamina densa, in the region of the anchoring fibrils, in another patient. Antiskin IgM autoantibodies of the same isotype as their monoclonal gammopathies can be present in the serum of some patients with the Schnitzler syndrome. These IgM antibodies seem to deposit in vivo in the epidermis and at the dermal-epidermal junction, in the region of the anchoring fibrils. These findings suggest that the monoclonal gammopathy plays a part in the pathophysiology of the skin rash. They also suggest patient heterogeneity both in the skin antigens that are recognized as well as in their localization.

Autoantibodies↗
PubMed2000

Direct molecular typing of Borrelia burgdorferi sensu lato species in synovial samples from patients with lyme arthritis.

Since Lyme arthritis was first described in the United States, it has now been reported in many countries of Europe. However, very few strains of the causative bacterium, Borrelia burgdorferi, have been isolated from synovial samples. For this reason, different molecular direct typing methods were developed recently to assess which species could be involved in Lyme arthritis in Europe. We developed a simple oligonucleotide typing method with PCR fragments from the flagellin gene of B. burgdorferi sensu lato, which is able to differentiate seven different Borrelia species. Among 10 consecutive PCR-positive patients with Lyme arthritis from the northeastern France, two species were identified in synovial samples: B. burgdorferi sensu stricto in 9 cases and B. garinii in 1 case. Conversely, all B. burgdorferi sensu lato species detected in 10 consecutive PCR-positive biopsies from a second set of patients with erythema migrans from the same geographical area were identified as either B. afzelii or B. garinii (P < 0.001). These results indicate that B. burgdorferi sensu stricto is the principal but not the only Borrelia species involved in Lyme arthritis in northeastern France.

Arthritis, Infectious↗
PubMed1999

[Cutaneous lymphoma manifesting as pigmented, purpuric capillaries].

INTRODUCTION: A few cases of patients with both purpuric pigmented dermatitis and cutaneous lymphoma have been reported. The aim of this study was to evaluate the prognosis of purpuric pigmented dermatitis. MATERIAL AND METHODS: This is a monocentric retrospective study at the dermatology department of the university hospital of Strasbourg. The records of all patients hospitalized for purpuric pigmented dermatitis between 1967 and 1997 have been reviewed. RESULTS: Eight women and 9 men aged between 17 and 84 years were hospitalized for purpuric pigmented dermatitis during the reference period. Except for one patient, all had had a cutaneous biopsy showing the typical features of purpuric pigmented dermatitis. On the basis of clinical signs and course, one patient was thought to have contact dermatitis and three patients were thought to have a purpuric pigmented dermatitis-like drug eruption (meprobannate, pefloxacine and lorazepam or aspirin). The mean follow-up was one year. During follow-up, two patients developed cutaneous T-cell lymphoma after two and four years respectively and one patient developed Hodgkin's disease with skin and lymph node involvement. Another patient who suffered from purpuric pigmented dermatitis for four years had a monoclonal T cell population within the dermal infiltrate. Two patients died of their lymphoma. DISCUSSION: This study shows that purpuric pigmented dermatitis can be associated to or evolve into lymphoproliferative disease. This course should be suspected when purpuric pigmented dermatitis is extensive, long-lasting (> 1 year), has a reticular arrangement and negative patch-testing. In this situation, a long-term follow-up and treatments indicated in the early patch stage of mycosis fungoides (PUVA therapy, chlormethine) seem adequate.

Adolescent↗
PubMed1999

Eccrine porocarcinoma, tricholemmal carcinoma and multiple squamous cell carcinomas in a single patient.

A 75-year old woman without remarkable medical history presented with a large eccrine porocarcinoma of the trabelucar type located on her leg. This tumor was associated with two invasive squamous cell carcinomas of the legs, two Bowen's diseases of her thigh and arm and multiple actinic keratoses of the face. Despite major surgery, local recurrence of the porocarcinoma was observed 6 months later. During the 3-year follow-up after a second surgical intervention, this lesion did not reccur, but the patient developed a rapidly enlarging ulcerated tumor of the forehead which proved to be tricholemmal carcinoma. Although no carcinogenetic factor or familial history of multiple neoplasms could be evidenced, the occurrence of multiple tumors of various histogenesis over a 1 year period of time is unlikely to be fortuitous. Such an association of rare adnexal neoplasms has not yet been described.

Acrospiroma↗
PubMed1998

The significance of the 'dust-like particles' pattern of immunofluorescence. A study of 66 cases.

Subacute cutaneous lupus erythematosus (SCLE) is a marker of a unique subset of lupus erythematosus patients. A 'dust-like particles' direct immunofluorescence (DIF) staining pattern, which consists of fine granular particles of immunoglobulin(s) scattered through the epidermis and the cellular infiltrates of the dermis, was reported to be specific for SCLE. In this study, we assessed the real specificity of this staining pattern, which had not yet been evaluated. We systematically searched for the dust-like particles staining pattern among the 4374 skin biopsy specimens submitted for direct cutaneous immunofluorescence during a 7-year period (1989-96). The corresponding patient records were reviewed. Dust-like particles were observed in 66 samples originating from 60 patients. Only 53% of the patients had SCLE. The remaining patients had systemic lupus erythematosus with visceral involvement (17%), discoid lupus erythematosus (3%), mixed connective tissue disease (2%). Sjögren syndrome (2%) and other diseases. Eighty-five per cent of the patients had connective tissue disease. Seventy-seven per cent of the patients were positive for antinuclear antibodies, but only 36% were positive for anti-Ro (SSA) antibodies. This study shows that the dust-like particles staining pattern is not specific for SCLE, but is highly suggestive of connective tissue disease. The nature of the antigen responsible for the immunoglobulin deposition and the prognostic significance of this DIF pattern remain to be established.

Antibodies↗
PubMed1998

[Melanoma in the Strasbourg University Hospital. A 30-year study].

INTRODUCTION: The incidence of melanoma has increased more than the incidence of any other cancer in the past twenty years. There is no cure for advanced-staged melanoma and it's early diagnosis has become a main issue of health policy. Therefore, precise epidemiological data are needed. These data depend on the geographical setting and very few data are available for France. We studied these data in Strasbourg. PATIENTS AND METHODS: All patients hospitalized for melanoma between 1960 and 1989 at the academic dermatology department, Strasbourg, were included in this retrospective, monocentric, study. The diagnosis of melanoma was confirmed in all cases by a dermatopathologist. Clinical, histopathologic and epidemiological data of the patients were recorded. RESULTS: Six hundred seventeen patients with a mean age of 52 years were included. The number of new patients hospitalized for melanoma grew steadily. The mean tumor thickness was 2.31 mm (+/- 1.59) and it remained unchanged between 1970 and 1989. The mean duration between the first clinical signs of melanoma and excision of the tumor was 22 months. Only 4 p. 100 of the melanomas were diagnosed by means of routine examination, and this concerned almost exclusively patients hospitalized after 1980. Eighty five p. 100 of the patients had localized melanoma (stage I) at time of diagnosis. Forty two p. 100 of the patients developed metastasis. The mean five year survival rate was 68 p. 100. CONCLUSION: The number of new patients hospitalized each year for melanoma grew steadily, but the mean tumor thickness remained unchanged. This indicates that the ratio "thick"/"thin" melanomas has remained unchanged between 1960 and 1989 and that the number of melanomas of any thickness has increased. Clinical data show an unawareness of the local population of the dangers of pigmented lesions during the reference period. This unawareness can be partially explained by the fact that no specific information campaign has ever taken place in this area. These data suggest that such a campaign should be recommended.

Adult↗
PubMed1998

[Photosensitivity as presenting sign of HIV infection. Control with triple antiretroviral therapy].

BACKGROUND: We report a case of photosensitivity which occurred as the presenting sign of HIV infection. Photosensitivity regressed completely after introducing antiretroviral tritherapy. CASE REPORT: A 44-year-old woman developed a photo-distributed eczematous eruption which did not respond to topical steroids or hydroxychloroquine. Histologic examination showed eczematous dermatitis. T-cell marker analysis showed a majority of CD8 cells in the infiltrate. The patient was found to be HIV-positive and CD4 counts were markedly reduced to 190/mm3 while CD8 counts were increased to 1260/mm3. Antiretroviral tritherapy cured the photosensitivity. Cure was apparently related to increased CD4 lymphocyte and normalized CD8 lymphocyte counts. DISCUSSION: Photosensitivity was the presenting disorder of HIV infection in this case. Cure of the photosensitivity with antiretroviral tritherapy has not been reported previously. CD8 T-cell infiltration and very low CD4/CD8 ratio would appear to play a key role in the pathogenesis of photosensitivity in these patients.

Adult↗
PubMed1998

[Interstitial granulomatous dermatitis and paraneoplastic rheumatoid polyarthritis disclosing cancer of the lung].

INTRODUCTION: Interstitial granulomatous dermatitis is a histological entity usually associated with systemic auto-immune conditions or malignant lymphoproliferation. Its spontaneous regression after surgical treatment of a lung cancer suggests an eventual paraneoplasic variant. OBSERVATION: A 62 year old male patient, a smoker, was hospitalized for weakness, pruritus and symmetrical arthritis of small and large joints. Numerous rheumatoid nodules were located on the extensor aspects of the involved joints. During hospitalization, he developed an inflammatory plaque located on a thigh, which showed histologic features consistent with the diagnosis of interstitial granulomatous dermatitis. He had elevated E.S.R., blood eosiniphilia, and the search for antinuclear antibodies and antineutrophilic leukocyte cytoplasm antibodies (ANCA), of the p-ANCA type, was positive. A lung C.T. scan showed a cavitary tumor. Surgical removal of this tumor evidenced a bronchial squamous cell carcinoma. Four week after surgery, clinical signs and eosinophilia disappeared. After 6 months, ANCA became undetectable. DISCUSSION: Parallelism between the evolution of the cutaneous and articular symptomatology and of the cancer are diagnosis of paraneoplastic rheumatoid arthritis and paraneoplastic interstitial granulomatous dermatitis. Paraneoplastic rheumatoid arthritis is very unusual and this is the first reported case of simultaneously occurring paraneoplastic interstitial granulomatous dermatitis.

Antibodies, Antineutrophil Cytoplasmic↗
PubMed1997

[Histiocytic cytophagic panniculitis].

A HISTOPATHOLOGICAL DIAGNOSIS: Histiocytic cytophagic panniculitis is the specific skin finding in the reactive hemophagocytic syndrome. It is a feverish nodular eruption which accompanies the other clinical and biological features of the reactive hemophagocytic syndrome. Histopathologic changes are diagnostic and consist in a lobular panniculitis characterized by the presence of a histiocytic infiltration of the fat. Histiocytes are benign in appearance and show variable degree of phagocytosis. INFECTION AND IMMUNE ANOMALIES: In more than 50% of the cases, the disease is triggered by an infection (mainly a viral infection from the herpes-virus family) in a patient with altered immune function (immunodeficiency, autoimmune disease, hematological disease). Search for subcutaneous T-cell lymphoma is mandatory, but is a diagnostic challenge. Such lymphomas are associated with histologic features of histiocytic cytophagic panniculitis. PATHOPHYSIOLOGY: Histiocytes in the histiocytic cytophagic panniculitis are activated by an abnormal cytokine secretion (interferon gamma, granulocyte/macrophage colony stimulating factor, phagocytosis inducing factor, ...) from neoplastic or reactive T cells. CLINICAL TRIALS: Untreated, the course of the disease is usually lethal. Etiologic treatment should be provided whenever possible. Symptomatic treatment consists in transfusion and corticosteroid therapy. Etoposide, and sometimes cyclosporin, have been reported to be efficient. Some authors recommend that histiocytic cytophagic panniculitis should always be treated with a CHOP-like polychemotherapy. More recently, good results were obtained with high dose intravenous immunoglobulin and this treatment therefore deserves further evaluation.

Diagnosis, Differential↗
PubMed1997

Annular atrophic lichen planus and Sneddon's syndrome.

We report the case of a patient who had 2 rare diseases, annular atrophic lichen planus (AALP) and Sneddon's syndrome (SNS). This patient had also digital nodules with histological abnormalities suggestive of SNS vasculopathy, which have not been reported so far. AALP is the most rare of all varieties of lichen planus since this case is the third reported to date. The association of livedo racemosa and cerebrovascular disease is the hallmark of SNS, the incidence of which is estimated to be 4 cases per year per million inhabitants. In both diseases, an abnormal production of elastic-tissue-degrading enzymes or a constitutional abnormality of the elastic tissue can be postulated, since SNS is characterized by arteriolar changes with deterioration of the internal elastic lamina and AALP by destruction of the dermal elastic tissue.

Adult↗