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Biomedical subjects

E Pichler

Publications and source records attributed to E Pichler.

At least 19 recordsLinked to original sources

[Spondylitis: borderline findings in magnetic resonance tomography].

MR studies of 41 patients with confirmed spondylitis were evaluated with regard to imaging findings resembling metastases or fracture. 30 patients had MR results considered typical for spondylitis (contiguous changes in two vertebrae and disc, soft tissue tumour). 11 patients had MR studies differing from this pattern. Absence of soft tissue involvement and discontinuous marrow changes may be misdiagnosed as bone marrow metastases.

Adult

Amyloid elastosis: analysis of the role of amyloid P component.

We report the second case of amyloid elastosis. Our patient had an underlying primary systemic amyloidosis with lambda light chain paraproteinemia. Salient clinical features included a sclerodermatous facial appearance, cordlike thickening of superficial blood vessels, neck skin resembling that in pseudoxanthoma elasticum, livedo reticularis-like changes on the trunk, Raynaud's phenomenon, arterial and venous thromboses, and the nephrotic syndrome. Amyloid deposits were present in the dermis, around appendages, in blood vessel walls, and in a striking distribution surrounding individual elastic fibers, that appeared shortened and fragmented. Immunofluorescence, electron microscopic, and immunoultrastructural studies with antibodies to lambda light chain, localized the amyloid deposits to the region of the elastic fiber microfibrils, with which amyloid P component (AP) is invariably associated in normal tissues. Because AP binds amyloid fibrils, codistribution of amyloid deposits and AP in amyloid elastosis strongly supports the theory that elastic fiber-associated AP may act as a nidus for amyloid deposition.

Amyloidosis

[AIDS--mucous membrane manifestations].

In the course of the infection with the human immunodeficiency virus (HIV), we frequently observe disorders of the mucous membranes and, occasionally, they present the first manifestation of HIV-induced immunodeficiency. Like in other organs, opportunistic infections and malignant tumors prevail as a result of the impaired immune system. Opportunistic infections are characterized by frequency (candidiasis), aggressive expansion, persistence, frequent recurrences, and resistance to therapy (gingivitis, parodontitis, herpes simplex, warts). Oral hairy leucoplakia is considered a specific lesion of HIV infection. Malignant tumors, such as Kaposi's sarcoma, non-Hodgkin's lymphoma, and squamous cell carcinoma, may cause marked morbidity in AIDS patients; occasionally, the clinical picture of Kaposi's sarcoma and non-Hodgkin's lymphoma is rather uncharacteristic. Other manifestations on the mucous membranes may arise in association with systemic reactions, such as drug eruptions, thrombocytopenic purpura, or acute HIV infection. The etiology of still other lesions of the mucous membranes (e.g. chronic recurrent ulcers, xerostomia, disorders of pigmentation) is incompletely understood. The awareness of these disorders of the mucous membranes in HIV infection is of diagnostic, therapeutic and epidemiological importance.

Acquired Immunodeficiency Syndrome

[Perioral dermatitis].

A unifying concept of the pathogenesis of perioral dermatitis is presented: perioral dermatitis is a cutaneous intolerance reaction linked to constitutionally dry skin and often accompanied by a history of mild atopic dermatitis. It is precipitated by the habitual use of one or - more often - a variety of moisturizing creams. The type and content of the creams used appear to play a much less important role than the mode of application (regular and abundant). The proposed pathomechanism is: persistent hydration of the horny layer, impairment of barrier function, an proliferation of the skin flora. Topical corticosteroids can aggravate but do not cause perioral dermatitis. This concept of pathogenesis leads to a simple and effective concept of therapy: reduction of the frequency with which moisturizing creams are applied from "regularly" to "as required" (only when dry and smarting skin makes this necessary) generally leads to lasting disappearance of symptoms within approximately 1 month.

Adolescent

Multiple eccrine poromas arising in chronic radiation dermatitis.

A 70-year-old white man developed 7 eccrine poromas in an area of chronic radiation dermatitis of his right lower extremity over a period of 37 years. To our knowledge, multiple eccrine poromas unequivocally linked to chronic X-ray damage are hitherto unreported.

Adenoma, Sweat Gland

[Cystic Kaposi's sarcoma].

A patient with the rare, cystic variant of classic Kaposi sarcoma is presented. The immunohistochemical and enzyme-histochemical phenotype of the cells lining the cysts is the same as that of lymphoendothelial cells. The response to electron beam radiation therapy is good.

Aged

Macular amelanotic melanoma in situ.

This report aims at directing the attention to the rare entity of amelanotic melanoma in situ, as exemplified in a patient who had an amelanotic lentigo maligna 10 years ago and a recurrent lesion of identical clinical and histological (except for pagetoid tumor cells in the epidermis) appearance 4 years ago. Amelanotic melanomas in situ appear as inconspicuous reddish macules, which can hardly be diagnosed or even suspected on clinical grounds.

Cheek

Hemangiosarcoma in chronic leg ulcer.

We recently evaluated a patient with an angiosarcoma arising in a long-standing ulceration of his leg, which appeared representative of a characteristic, though fairly uncommon, subgroup of angiosarcomas. The clinical and immunohistochemical features of the tumor enabled us to define its origin from vascular endothelial cells.

Aged

[Mycetoma caused by Petriellidium boydii: treatment with ketoconazole].

A case of mycetoma of the lower leg (Madura foot) with bone involvement caused by Petriellidium boydii is presented. After an initially favourable therapeutic response to ketoconazole (2 X 200 mg/die) the process recurred despite proven sensitivity of the fungus in vitro. It could not be controlled with conservative treatment and ultimately necessitated amputation of the lower leg. A striking, hitherto unreported feature of mycetoma was the episodic occurrence of circulating immune complexes with febrile monoarthritis of the adjacent ankle.

Adult

[Xeroderma pigmentosum: prevention of tumors with etretinate].

A 46-year-old woman with xeroderma pigmentosum has had more than one hundred skin tumours in her life, in recent years largely keratoacanthomas. Two and a half years ago, a low-dose prophylactic treatment with etretinate (25 mg/day) was initiated. Since then, no further skin tumours have arisen except for several very small, rapidly resolving keratoacanthomas.

Etretinate

Results of LSA2-L2 therapy in 26 children with non-Hodgkin's lymphoma.

Twenty-six children with non-Hodgkin's lymphoma (NHL), 17 boys and nine girls, were treated with the LSA2-L2 protocol. Seven children had stage I or II, 16 Stage III and three Stage IV according to Murphy's staging system. Eight children had their primaries in peripheral lymph nodes, eight in the abdomen, six in the mediastinum and four in other sites. All tumors were classified histologically according to four different classifications. Overall disease-free actuarial survival is 53.6%. Complete responders show a disease-free survival of 77.8%. Fourteen children survived for 9-56 months. Included are all seven children with Stage I or II who survive irrespective of histologic type of the tumor. Of the remaining 12 children in Stage III or IV three children died in remission and nine of progressive disease. Eight of these nine patients did not attain complete remission. Whereas four of five children with the convoluted type of NHL survive, four of five patients with the Burkitt's type (small noncleaved follicle center cell lymphomas) died of progressive disease. According to Rappaport's classification, four of six children with diffuse undifferentiated lymphoma (DUL) are dead due to tumour progression. Considering the classification of lymphoblastic lymphomas introduced by Nathwani et al., 23 five of seven children suffering from lymphoblastic lymphomas but only two of eight children with nonlymphoblastic lymphomas belong to the survivors. Therefore histologic findings do hold prognostic significance in our series of children with NHL.

Adolescent

[Progress in the treatment of juvenile leukemias].

Between January 1979 and December 1980 64 children with acute lymphoblastic leukemia were treated in 9 pediatric clinics in austria according to the BFM study 76/790-protocol. For remission induction all patients received an 8 week multidrug regimen (West-Berlin ALL-protocol). High risk patients were defined according to a risk score at diagnosis and additionally treated with a 6 week reinforced reinduction protocol during the first half year after diagnosis. Maintenance therapy was stopped after about 22 months. The life table-analysis after 30 months showed a 75.5% disease free survival for the total group of patients. Compared with a control group of 228 patients treated between 1974 and 1980 in 9 different clinics in Austria according to 3 consecutive national treatment regimens (modifications of Memphis protocol VII and VIII), therapeutic results were markedly improved. After a follow-up of 36 to 90 months the overall oumulative remission rate was 37.7%. The results could be improved especially in the group of high risk patients for replase by 35% in contrast to the historical studies. A prognostic difference between low- and high risk-patients was not seen in the BFM study (84.3% vs. 69.9%). Without doubt, the marked improvement of prognosis is due to the intensification of therapy.

Antineoplastic Agents

[Diagnostic and therapeutic problems of nasopharyngeal carcinoma in childhood (author's transl)].

Between 1974 and 1978 three children with a nasopharyngeal carcinoma (stages: T3N3MO, T4N2MO, T4N1MO) were treated with primary radiotherapy (tumor dose: 45-67 Gy). Local control was achieved in two children, one of these (T4 primarily) had a local relapse five months after diagnosis. Only one of the three children had radiotherapy to the neck region. This child developed lung and mediastinal metastases with hypertrophic osteoarthropathy twelve months after diagnosis. In the other two children lung metastases were observed two and seven weeks after the diagnosis respectively. In these two radiotherapy of metastases and chemotherapy were considered more important than radiotherapy of the neck region. All three children showed disappearance or considerable reduction of lung metastases due to radio- and chemotherapy. Yet, all three died of progressive disease within a few months. Early diagnosis of nasopharyngeal carcinoma in children will only be possible when pediatricians are more aware of this disease, also in our regions. Radiotherapy should be done only with megavoltage equipment using extensive radiation fields for the primary tumor area and elective radiation of the neck region. The use of additional primary chemotherapy is suggested for stages T3/T4. This mode of management may help to prevent distant metastases.

Age Factors