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Biomedical subjects

R K Winkelmann

Publications and source records attributed to R K Winkelmann.

At least 91 records · Page 5Linked to original sources
PubMed1989

Clinical and pathologic correlations in 96 patients with panniculitis, including 15 patients with deficient levels of alpha 1-antitrypsin.

alpha 1-Antitrypsin levels were determined for 96 patients with various forms of biopsy-proved panniculitis. Fifteen of the 96 patients had alpha 1-antitrypsin deficiency, and 12 of these also had an abnormal alpha 1-antitrypsin phenotype. This group of patients showed substantial and characteristic clinical and histopathologic differences from the group of patients with normal levels and phenotypes of alpha 1-antitrypsin. Spontaneous ulceration and drainage of panniculitis lesions were much more common in patients with alpha 1-antitrypsin deficiency, and biopsy specimens from lesions of panniculitis in these patients were much more likely to show large areas of normal fat adjacent to necrotic lobular and septal areas, which contained many polymorphonuclear leukocytes and histiocytes. Destruction of elastic tissue was more frequent and extensive in patients with alpha 1-antitrypsin deficiency.

Female↗
PubMed1989

Histopathologic features of superficial granulomatous pyoderma.

Study of the histopathologic pattern of 28 cases of superficial granulomatous pyoderma demonstrated an ulcerative, verrucous pyoderma of the superficial dermis. Focal abscesses in the subepidermis or dermis were juxtaposed with granuloma formation and plasma cell inflammation. Hemorrhage and granulation tissue were present. The clinical lesions are indolent, and this feature is confirmed by chronic inflammatory elements, including sinus tract formation and focal fibrosis. Superficial granulomatous pyoderma must be added to the histologic differential diagnosis of the vegetative and ulcerative granulomas: blastomycosis, tuberculosis verrucosa cutis, and bromoderma.

Abscess↗
PubMed1989

Skin lesions associated with circulating lupus anticoagulant.

This retrospective study of 295 patients extends the earlier findings of an association between lupus anticoagulation and thrombosis by demonstrating the occurrence of cutaneous lesions related to the coagulation defect in 70 patients. The most frequent cutaneous associations were thrombophlebitis, skin ulcer, gangrene, haemorrhage, and cutaneous necrosis. Of the patients with skin lesions, 41% had the skin lesion as the first sign of the disease. It is important to recognize this association because nearly 40% of the patients with skin lesions have multisystem thrombotic phenomena in the course of the disease.

Adolescent↗
PubMed1989

Large cell lymphocytoma--a clinicopathological study.

Fourteen patients with large cell lymphocytoma were studied. They presented with solitary or small numbers of grouped nodules on the trunk or head and neck region, which histologically consisted of diffuse and nodular dermal aggregates of lymphoid cells. A proportion of these cells were large with clear cytoplasm and a varying degree of nuclear atypia. An infiltrate of small mature lymphocytes surrounded and extended between the zones of larger cells. Mitotic figures and pleomorphic cells were frequent and, on the basis of this histopathology, an initial diagnosis of cutaneous lymphoma had frequently been made. There were eight male and six female patients with a mean age at presentation of 40 years (range 14-71) who were followed up for a period of between 4 and 31 years (mean = 14.6). Thirteen of the 14 are alive and well, and there has been no evidence of systemic involvement in any of the patients; one patient died of chronic renal failure and pneumonia. Monoclonal antibody studies on paraffin sections showed the presence of lymphoid cells of B and T cell lineage in large cell lymphocytoma. We believe that these patients have a variety of cutaneous lymphocytic infiltrate, large cell lymphocytoma, which has characteristic clinical and histological features and is important to recognize because of its benign course. Large cell lymphocytoma has been used to describe a nodular lymphoid infiltrate of the skin composed principally of large follicular centre cells. Often these lesions had been diagnosed as reticulum cell sarcoma but, on follow-up, all the patients were alive and well.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1989

A timed study of the histopathology, direct immunofluorescence and ultrastructural findings in idiopathic cold-contact urticaria over a 24-h period.

The histopathology, immunofluorescence and ultrastructure of skin in idiopathic cold-contact urticaria have been studied over the 24 h following the application of a cold stimulus sufficient to provoke a confluent weal on the anterior thigh. Biopsies were taken 10 min, 2 h and 24 h after ice removal. Considerable epidermal and dermal oedema was present. Type I and Type II mast-cell degranulation was noted but was not universal. Lymphatics and capillaries were dilated and endothelial cells showed an increase in micropinocytotic activity, without evident vasculitis. In two cases packed platelets were seen within vessel lumina. There was no change in the infiltrating dermal cell population and direct immunofluorescence was negative. The evidence suggests that idiopathic cold-contact urticaria is an exudative rather than an infiltrative process.

Adolescent↗
PubMed1989

Unilesional mycosis fungoides: clinical, microscopic and immunophenotypic features.

Seven cases are reviewed in which the histologic and immunohistologic features were those of mycosis fungoides, although the patients presented with solitary lesions which did not recur after local therapy (excision in six, radiotherapy in one), with follow-up of 10 months to 18 years. Immunophenotypic staining of paraffin-embedded tissue revealed a predominant T-lymphocyte proliferation in all cases, None of the four cases studied for BER-H2(Ki-1) were positive, in contrast to the "activated" types of spontaneously regressing lymphoid lesion, lymphomatoid papulosis and "Ki-1" lymphoma. Electron microscopy in one case demonstrated the highly convoluted nuclei of the T cells within the infiltrate. These seven cases represent examples of unilesional mycosis fungoides in which the disease has not recurred after therapy, despite features indistinguishable from those of typical generalised mycosis fungoides-type of cutaneous T-cell lymphoma.

Adult↗
PubMed1989

Subcutaneous fat necrosis of the newborn: light, ultrastructural and histochemical microscopic studies.

Multiple subcutaneous plaques and nodules appeared on the back and the dorsal proximal area of the extremities of a 9-day-old male infant after a complicated prenatal period necessitating cesarean section. The clinical and histological features were diagnostic of subcutaneous fat necrosis of the newborn. Light microscopy revealed adipocyte necrosis, a lymphohistiocytic infiltrate, and needle-shaped clefts within adipocytes and macrophages. Ultrastructurally, there were aggregations of electron-lucent spaces in the form of spindles and needles arranged in parallel within the altered adipocytes; macrophages surrounded these cells or their fragments and invaded the fat lobules. Enzyme histochemical staining, not previously reported in the literature, showed that acid phosphatase, leucine aminopeptidase, and indoxyl and non-specific esterases were present in the areas of fat necrosis.

Acid Phosphatase↗
PubMed1989

Cytophagic histiocytic panniculitis is not always fatal.

Two patients with benign cytophagic histiocytic panniculitis are presented and compared with the one benign and ten fatal cases previously reported. Benign-appearing histiocytes which engulf blood cells, nuclear debris, and platelets are the most characteristic feature of the disease. This cytophagia tends to be massive in the subcutaneous tissue and involves extracutaneous organs only in the fatal cases. The course is chronic in the benign form. Patients with the fatal form tend to have fever, hepatosplenomegaly, serosal effusions, ecchymoses, peripheral adenopathy, and mucosal ulcers. Anemia, leukopenia, elevated liver enzyme levels, and coagulopathy are present in almost all and hypocalcemia in many patients with a fatal course. Cytophagic histiocytic panniculitis may represent a form of regional histiocytosis primarily involving subcutaneous tissue. It is midway in the spectrum of the cytophagic histiocytoses.

Adult↗
PubMed1989

The current treatment of scleroderma.

The treatment of scleroderma is determined by the stage of the disease, associated organ involvement, or the presence of features overlapping those of other connective tissue disease. Raynaud's phenomenon is responsive to vasoactive medication, but recently heat and plasma exchange have been shown to be more effective, reducing the need for systemic medication. In stages II and III of the disease, administration of non-toxic penicillamine in low doses for 2 to 4 years is the preferred treatment. Plasma exchange may offer some hope in the early stages. The treatment of the renal crisis of scleroderma with angiotensin-converting enzyme inhibitors has reduced mortality from this complication. These drugs are currently the preferred treatment for the hypertension of renal scleroderma. The symptomatic treatment of the pulmonary, gastrointestinal, and soft tissue complications of scleroderma is also discussed.

Angiotensin-Converting Enzyme Inhibitors↗
PubMed1989

Intermittent leukapheresis: an adjunct to low-dose chemotherapy for Sézary syndrome.

Eleven patients with Sézary syndrome were treated with intermittent leukapheresis in addition to low-dose chlorambucil and prednisone. The results were as good as or better than those with chemotherapy alone. We believe the combined program with continuous leukapheresis to be optimal therapy but note that intermittent treatment offers some benefit for patients.

Chlorambucil↗
PubMed1989

[Congenital solitary fibromatosis].

Infantile myofibromatosis is included in fibrous proliferations of infancy, processes with specific clinic, histology, and evolution, that are characterized by nodules in the skin, bones, and viscera. It's prognosis is varied, depending on whether it is manifested in the solitary or multicentric form. We describe a patient with congenital solitary myofibromatosis.

Female↗
PubMed1988

Diffuse dermal neutrophilia in urticaria.

Twenty-three cases of diffuse dermal neutrophilia and urticaria were reviewed. Evidence of vasculitis or other disease was not present. Direct immunofluorescence was done in 19 cases and was positive but nonspecific in 14. The presence of angioedema (10 cases), dermographism (2 cases), atopy (4 cases), drug reactions (6 cases), and infections (6 cases) was similar to that seen in cases of urticaria without diffuse dermal neutrophilia. Nearly 9% of the cases of urticaria show this histologic pattern, and when cases of neutrophilic urticaria are added, 15.8% of cases of urticaria were found to show a major neutrophil component in the inflammation. In urticaria, neutrophilia may be part of an intense stimulus response pattern.

Angioedema↗
PubMed1988

Superficial granulomatous pyoderma: a localized vegetative form of pyoderma gangrenosum.

Twenty-five patients had superficial ulcerative and vegetative pyoderma with granulomatous histologic findings. Healing occurred without systemic corticosteroid therapy in all but three patients. All patients had clinical pyoderma gangrenosum. In five patients the lesion occurred after surgery of the skin. Histopathologic study of 40 biopsy specimens showed focal neutrophilic abscesses of the papillary dermis, often with peripheral palisading histiocytes and foreign-body giant cells. Pseudoepitheliomatous, vegetative hyperplasia and sinus tract formation were observed frequently. All patient had massive areas of plasmacytosis, and 13 had eosinophils. Granulation tissue, hemorrhage, and fibrosis were additional features in some areas. Foreign material in the lesions was considered unimportant, except in one patient with a starch granuloma. Therapy with local corticosteroids, minocycline, tetracycline, or sulfa drugs resulted in healing in 15 patients. We believe that we have identified a localized, limited form of chronic superficial pyoderma gangrenosum with verrucous and ulcerative lesions and a granulomatous histologic appearance that represents a unique pattern of this disease in some patients.

Abscess↗
PubMed1988

Lipomembranous changes in chronic panniculitis.

The clinical and histopathologic findings in 13 patients with lipomembranous changes in the subcutaneous adipose tissue as part of the inflammatory reaction are presented. Nine patients had clinical evidence of vascular disease and four had clinical evidence of connective tissue disease. Histopathologic evidence of endarteritis obliterans, venous stasis, and hemorrhage was present in more than half the patients, and the clinical lesion of liposclerosis was frequently present. These findings suggest that the histologic changes of lipomembranous panniculitis may be the result of an inflammatory reaction in patients who have the liposclerosis of venous insufficiency with connective tissue disease or previous leg ischemia or both.

Adipose Tissue↗
PubMed1988

Histopathologic and immunofluorescence study of skin lesions associated with circulating lupus anticoagulant.

We reviewed the histopathologic findings in 28 specimens from 25 patients who had skin lesions associated with lupus anticoagulant. The clinical lesions were ulcers, gangrene, thrombophlebitis, hemorrhage, and cutaneous necrosis. Noninflammatory thrombosis of small dermal vessels was observed in all 8 biopsy specimens from gangrene lesions, 10 of 13 specimens from ulcer lesions, and 2 of 5 specimens from thrombophlebitis lesions. Necrotizing vasculitis was not significant in these biopsy specimens. Immunofluorescence findings included a positive lupus band test in lupus erythematosus-associated disease and nonspecific deposits in occasional cases. Focal noninflammatory intravascular coagulation is responsible for the microscopic and clinical skin lesions in these patients.

Blood Coagulation Factors↗
PubMed1988

Cutaneous reactions to vitamin K.

In four patients the development of erythematous plaques after vitamin K injections was apparently due to delayed hypersensitivity reactions. The 36 cases described in the literature indicate that most patients with the reaction received vitamin K injections for liver disease and that a late sclerodermatous reaction develops rarely.

Adolescent↗