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Biomedical subjects

E Pichler

Publications and source records attributed to E Pichler.

At least 19 recordsLinked to original sources

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

[Treatment of Wilms' tumor (author's transl)].

Uniform treatment based on the therapeutic approach of the 1st and 2nd US National Wilms' Tumor Study was decided on in March 1976 by paediatricians, surgeons, urologists and radiotherapists in Austria. Wilms' tumour was diagnosed in 34 children between 1 january 1976 an 29 february 1980 (stage I: n = 11, stage II: n = 8, stage III: n = 8, stage IV: n = 7). Parents of two children refused treatments; both children have since died of metastases. Of the remaining 32 children 29 (90.6%) are alive, 10 for more than 4, 15 for more than 3 and 19 for more than 2 years after diagnosis. 21 children are without need of treatment. Three children have died, one due to postoperative complications, one due to haemorrhagic chickenpox, but free of tumour, and one after insufficient treatment. Two of the five children with a recurrence between 2 1/4 to 15 months after diagnosis had been treated inadequately in the initial phase. The tumour free survival rate in 74.2%. Two children with early occurring or recurrent lung metastases have survived for 53 1/2 and 54 months up to now.

Age Factors

Preventive cranial irradiation in the treatment of acute lymphocytic leukemia in children.

From April 1971 to September 1977, 94 children with acute lymphocytic leukemia, in whom a complete clinical and hematologic remission had been obtained received preventive cranial irradiation at the University Clinic for Radiotherapy of Vienna. So far, only in 2 of the 94 patients an initial meningeal relapse occurred. After a minimum follow-up time of 3 years, the rate of primary CNS relapse is 8%. The combination of cranial irradiation and methotrexate intrathecally, beginning currently in the first month of the complete remission, seems to be the most effective and least hazardous regimen to prevent meningeal and CNS leukemia.

Adolescent

[Systemic lupus erythematosus in twins (author's transl)].

A report is given of systemic lupus erythematosus (SLE) in monozygotic twins. One sister developed coombs-positive haemolytic anaemia at the age of 11 years and suffered from five haemolytic crises over a period of 8 months. SLE could not be proven at that time, but following a 3-year symptom-free period, characteristic clinical and immunological evidence of SLE became manifest. The second sister developed classical SLE at the age of 15 years. Clinical, immunological, etiological and therapeutical aspects of SLE are being discussed.

Adolescent

[Polycythaemia as sole symptom of renal adenoma (author's transl)].

In two children, a 9 year-old boy and a 10 1/2 year-old girl, who presented with polycythaemia as the only symptom, the expected renal tumour was only found after exclusion of all other causes of polycythaemia. The delay in diagnosis was caused by technically inadequate intravenous urograms, which were erroneously passed as normal. In one child low kv X-ray exposition of the kidneys led to the diagnosis of a renal tumour. In the other child high-dose urography and tomography gave the indication for selective angiography. Normalization of the red blood count postoperatively verifies the connection between preoperative erythrocytosis and the renal tumour. Histologically both cases proved to be renal adenomas, which are extremely rare in childhood.

Adenoma

[Diagnostic, therapeutic and prognostic aspects of wilms' tumour (author's transl)].

Within the last 20 years 43 children with Wilms' tumour were seen at the Paediatric Departments of the University Hospitals of Graz, Innsbruck and Vienna. Case histories, clinical details and diagnostic procedures are discussed. Since 1969 19 out of 21 children were treated according to the modern atandard regimen (operation, irradiation and cytostatic therapy for 2 years except in infants with stage I). Since 1969 the survival rate has been higher (17 out of 21 children: 81%) than in the period 1956 to 1968 (7 out of 22 children: 31.8%), when only one child (in stage I) was treated according to current concepts. The better prognosis noted in young infants of this series, as in the literature was due to the earlier stage of the disease in these infants. A further improvement in the survival rate of children with Wilms' tumour should be achieved by earlier diagnosis, thereby ensuring operability, and by cytostatic therapy during the following 2 years. This will only be possible when there is closer cooperation between surgeon, radiotherapist and oncologist. It should be possible to lower the long-term therapeutic complication rate with even more stringent observation measures and with increasing expertise of all doctors concerned.

Age Factors