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D Lipsker

Publications and source records attributed to D Lipsker.

At least 37 records · Page 2Linked to original sources

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↗

[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↗

[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↗

[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↗

[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↗

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↗

[Lymph node and cutaneous syndrome associated with bone plasmacytoma].

INTRODUCTION: We report two patients in whom a slowly growing erythematous thoracic lesion led to the diagnosis of an underlying plasmocytoma. After the treatment of the latter, the cutaneous lesions disappeared, strongly suggesting a link between the two manifestations. CASE REPORTS: The two male patients, aged respectively 66 and 73 years old, had erythematous thoracic plaques. In both cases, extensive laboratory work-up and a histological examination of a cutaneous biopsy did not allow a precise diagnosis. The two patients had a solitary bony plasmocytoma located beneath the cutaneous plaques. The plasmocytomas were discovered respectively 2 and 4 years after the first cutaneous manifestations and were associated to histological non-specific lymph node hyperplasia. Treatment of the plasmocytoma led to the disappearance of skin lesions in both patients. DISCUSSION: In both cases, an erythematous scleroderma-like plaque associated to palpable peripheral and mediastinal lymph nodes, was located over a solitary bony plasmocytoma. Treatment of the plasmocytoma led to the disappearance of the cutaneous lesions, strongly suggesting a link between the two manifestations. Such an association has not been previously reported. We think this is a new entity, characterised by a scleroderma-like cutaneous plaque overlying a solitary bony plasmocytoma and associated to superficial and deep lymph node hyperplasia. We suggest to call it "plasmocytoma associated cutaneous lymph node syndrome". Its relationship to the POEMS syndrome and scleromyxedema are discussed. The pathophysiology remains completely unknown; the syndrome regresses after the treatment of the plasmocytoma.

Aged↗

[Primary cutaneous monomelic B-cell lymphoma].

BACKGROUND: Cutaneous B cell lymphomas, especially when appearing as a monomelic papulonodular eruption, are rare. PATIENT: Ms H. 87-year-old, consulted for a papulonodular eruption of the left lower limb which developed during the past 5 months. This limb had been the site of a lymphedema since a traumatism 8 years ago. Histopathological analysis and immunostaining of a nodule showed that it was a large cell lymphoma of follicular stem cells. There was no extracutaneous involvement and the patient was successfully treated with radiotherapy. Two months after the completed radiotherapy a cutaneous relapse on the trunk and the upper limbs was treated with cyclophosphamide-vincristine-prednisone chemotherapy. DISCUSSION: Lymphedema probably played a role in the genesis of this lymphoma presumably by reducing the local immune response. It may have harmed endothelial cells and maintained an antigenic stimulation leading first to lymphocyte hyperplasia and eventually to a true lymphoma, in the same way this has been proved for some MALT lymphomas.

Aged↗

[Macrophage activation syndrome disclosing leukemic transformation of mycosis fungoides].

BACKGROUND: Mycosis fungoides can mimic pigmented purpuric dermatitis. We report such a case which progressed to peripheral T-cell lymphoma; progression was revealed by reactive hemophagocytic syndrome (RHS). CASE REPORT: A 65-year old male patient was hospitalized for a pigmented and purpuric eruption. The skin lesions appeared 2 years earlier and at that time biopsy had shown pigmented and purpuric dermatitis. One month before hospitalization, general signs appeared. On admission, he had papular and purpuric rash, mainly on the trunk, hepatosplenomegaly, enlarged axillar and inguinal lymph nodes, and fever at 38.2 degrees. A skin biopsy showed histologic changes typical of mycosis fungoides. He also had bicytopenia, hepatitis, and increased triglyceride and ferritin levels suggesting RHS which was proved by means of bone marrow biopsy. These tests also evidenced peripheral T-cell lymphoma. The patient was treated with two courses of chemotherapy (CHOP) but the disease progressed and he deceased. DISCUSSION: Mycosis fungoides can occasionally begin with an eruption very closely resembling pigmented purpuric dermatitis. Therefore, repeated biopsies should be done in case of widespread permanent pigmented purpuric dermatitis of no apparent origin. RHS is a life-threatening disease. The diagnosis should be suspected in any cytopenic patient with fever, increased triglyceride levels and abnormal liver tests. A search for an etiology must then be undertaken a prompt treatment is needed.

Aged↗

[Blaschkolinear eruption with biological signs of lupus].

INTRODUCTION: Acquired inflammatory dermatoses of the adult with a blaschkolinear arrangement are rare. Their cause is unknown. CASE REPORT: We report the case of a 35-year-old man who presented an unilateral skin eruption covering the forearm and with secondary blaschkolinear extension to the arm, the axillary region and the chest. In this particular case, the patient was positive for RNP type anti-nuclear antibodies and direct immunofluorescence was positive in both biopsies of the lesions and of healthy skin. The eruption lasted 6 months and regressed spontaneously. DISCUSSION: Such cases are exceptional and the nosology is difficult to establish. A particular form of lupus erythematosus? Fortuitous coexpression of lupus antibodies and blaschkitis in an adult?

Adult↗