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Biomedical subjects

R K Winkelmann

Publications and source records attributed to R K Winkelmann.

At least 145 records · Page 8Linked to original sources

Papulonecrotic tuberculid: a neglected disease in Western countries.

Papulonecrotic tuberculid was diagnosed in twelve young patients demonstrating symmetric scattered papulopustular necrotic lesions of the extremities. The diagnosis was supported by a strongly positive Mantoux reaction in all cases, evidence of preexisting or past tuberculosis in eight patients, characteristic histologic findings, and a prompt resolution with antituberculosis therapy. Recurrence of the skin lesions in three patients treated only with isoniazid or with para-aminosalicylic acid and isoniazid indicates the necessity for combination treatment with several antituberculosis drugs. A detailed study of twenty biopsies indicates that the primary lesion is a subacute lymphohistiocytic vasculitis that causes thrombosis and destruction of small dermal vessels. These changes lead to an infarctlike lesion with coagulation necrosis of dermal tissue. In eleven instances a well-marked palisaded histiocytic reaction was seen around necrotic areas, calling into question the differential diagnosis of granuloma annulare or Churg-Strauss granulomatosis.

Adolescent↗

Necrobiotic xanthogranuloma with paraproteinemia. A review of 22 cases.

Cutaneous biopsy specimens from 22 patients showed the distinctive histopathologic pattern of necrobiotic xanthogranuloma within the dermis or subcutaneous tissue (or both). Twenty of the 22 patients had 1 or more serum protein abnormalities, consisting of an IgG monoclonal protein in 16, multiple myeloma in 3, cryoglobulinemia in 3, and an abnormal serum protein electrophoresis in 1. Cutaneous lesions were seen as discrete, slowly developing red nodules and plaques with a xanthomatized hue and a predilection for the face (periorbital region in particular), trunk, and extremities. Ulceration was a notable finding in 10 patients. Histologically, the dermis and lobules of subcutaneous tissue were involved with a granulomatous infiltrate containing bands of hyaline necrobiosis and bizarre foreign body, as well as Touton giant cells. Cholesterol clefts, lymphoid nodules with or without germinal centers, and foci of plasma cells were variable but significant features. Leukocyte monoclonal antibody studies in 6 patients demonstrated helper T cells within the granulomas. Electron microscopy in 3 cases showed lipid vacuoles in macrophages in the dermis and dendritic cells in the epidermis, and study confirmed this entity as a non-X histiocytosis. Pertinent laboratory findings, in addition to the serum protein abnormalities, included elevation of the erythrocyte sedimentation rate, leukopenia with absolute neutropenia, and decreased serum complement levels, as well as decreased levels of C1-esterase inhibitor in some patients. Thirteen of the 22 patients have survived, the mean duration being 9.5 years after the onset of cutaneous disease. While given to only a few patients in the current series, low-dose chemotherapy seems to induce a favorable response in both the cutaneous and the hematologic disease.

Adult↗

The histopathology of localized lipoatrophy.

We studied 11 patients with the clinical diagnosis of lipoatrophy and found two histopathological subsets. Six patients presented with a distinctive picture, which we termed 'involutional' fat, consisting of lobules of small lipocytes embedded in hyaline connective tissue with numerous capillaries. Five of these six patients had a single lesion, usually of the upper arm. Serological studies were normal, and direct immunofluorescence, performed in three cases, showed immunoreactants in the blood vessels in only one. The four patients with inflammation of the fat had multiple areas of localized lipoatrophy. Three had biopsies for direct immunofluorescence and all three showed immunoreactants involving the basement membrane zone (two cases) or blood vessels (one case); and three had serological abnormalities. We suggest that the involutional histopathological pattern is a distinctive subset of localized lipoatrophy.

Adipose Tissue↗

Necrobiotic xanthogranuloma: a report of four cases.

Four patients are described who demonstrate the range of clinical and pathological features seen in necrobiotic xanthogranuloma with paraproteinaemia (NXG). Each patient had the typical periorbital yellow plaques with numerous well demarcated yellow indurated plaques on the trunk. All four patients had evidence of paraproteinaemia. The histopathology showed diagnostic features of intense necrobiosis with xanthomatization, including touton giant cells and the bizarre angulated giant cells of NXG. Previously, the patients had been diagnosed as atypical necrobiosis lipoidica, and the distinction between NXG and other necrobiotic conditions is discussed.

Aged↗

Eosinophilic panniculitis: a clinicopathologic study.

Study of 18 patients with biopsy diagnoses of eosinophilic panniculitis revealed diverse patterns of systemic disease, including Wells' syndrome, vasculitis, atopy, and erythema nodosum as well as localized panniculitis. Significant associated diseases included psychiatric illness, 6 (drug dependency, 4); atopy, 5 (asthma, 3); malignancies, 5; immune complex vasculitis, 4; thyroid disease, 2; Wells' eosinophilic cellulitis, 2; glomerulonephritis and sarcoidosis, 1 each. The skin lesions varied from urticarial papules and plaques to purpura, pustules, and ulcerative lesions but always included a nodular subcutaneous component, frequently as a presenting complaint. Eosinophilic panniculitis is a non-specific finding that can signify localized disease, such as an insect bite or injection lipophagic granuloma in a drug-dependent patient, or systemic lymphoma or immune reactive disease. Eosinophilic panniculitis in erythema nodosum is perhaps its most confusing presentation.

Adult↗

Inflammatory lymphadenoid reactions with dermatofibroma/histiocytoma.

Lymphoid nodules were found associated with 44 of 1,506 dermatofibroma/histiocytoma tumors of the skin. The lymphoid nodules were usually in the adjacent fat. Germinal center formation occurred, and perinodular and perivascular plasmacytosis was frequently associated.

Adipose Tissue↗

The perivascular cell populations in human skin after topical application of leukotriene B4.

Fifteen timed biopsies of human skin were performed after application of 100 nanograms of leukotriene B4 in a Finnchamber for six hours. The number of inflammatory cells were counted per high power perivascular field and compared to three control biopsies. At 24 hours a peak of neutrophiles was observed and subsequently lymphocytes predominated. Eosinophils were never prominent. This pattern of successive cell populations has been described in inflammatory and whealing skin disease.

Administration, Topical↗

Chromosome studies in scleroderma with consideration of anticentromere antibody status and assessment of possible in vitro clastogenic activity.

The constitutional karyotype and frequency of sporadic chromosome abnormalities in peripheral blood leukocytes from 30 scleroderma patients and 15 normal controls were studied. Fifteen of the scleroderma patients were positive for the anticentromere antibody (ACA) and 15 were negative. The constitutional karyotype of all patients and controls were normal. No statistically significant difference in sporadic chromosome abnormalities was detected among the two groups of scleroderma patients compared with the control group. The possibility of clastogenic activity in serum from scleroderma patients was investigated by culturing lymphocytes from three normal individuals in medium enriched with serum from either a normal control, an ACA-negative scleroderma patient or an ACA-positive scleroderma patient. There was no statistically significant difference in the frequency of sporadic chromosomal abnormalities among the cells in these experiments. The results of this study suggest that, contrary to previously reported studies, the frequency of sporadic chromosome abnormalities is not increased significantly in scleroderma patients. In addition, although the anticentromere antibody is reactive with chromosomal material, patients with this antibody do not have increased chromosome breakage or aneuploidy, and the antibody does not induce chromosomal changes in vitro.

Adolescent↗

Cutaneous extravascular necrotizing granuloma in a patient with Takayasu's aortitis.

Cutaneous extravascular necrotizing granulomas (Churg-Strauss granulomas) have been noted in several systemic diseases, most often those producing or associated with systemic vasculitis. A young man with the clinical and histopathologic features of Takayasu's aortitis developed typical extravascular necrotizing granulomas of the skin. Takayasu's aortitis, like allergic granulomatosis and Wegener's granulomatosis, is a granulomatous vasculitis syndrome associated with the cutaneous Churg-Strauss granuloma.

Adult↗

Chromosome studies in 17 patients with the Sézary syndrome.

Chromosome studies were done on phytohemagglutinin-stimulated peripheral blood from 17 patients with Sézary syndrome. A chromosomally abnormal clone was found in five patients: each patient had an abnormal chromosome 6 and four had an abnormal chromosome 1. Six patients without abnormal clones had more than 20% metaphases with random heteroploidy and sporadic structural anomalies. Only normal metaphases were seen in four patients, and no metaphases were found in two. Four of the five patients with an abnormal clone died, and their median survival from chromosome analysis was 6 months; only one of these patients died of lymphoma. The six patients with increased heteroploidy had long survivals and no apparent malignant process. Two of the four patients with normal metaphases died of malignant disease: one had lymphoma and the other squamous cell carcinoma. A third patient with normal chromosomes died of extensive visceral cutaneous T-cell lymphoma.

Adult↗

Amyloid elastosis. A new cutaneous and systemic pattern of amyloidosis.

A patient with papulonodular cutaneous disease was studied until his death from progressive systemic disease. The elastic fibers in the skin, subcutaneous tissue, and serosae were coated with an amyloid-staining material in a unique pattern, as demonstrated by special stains and electron microscopy. Visceral and cutaneous blood vessels from autopsy tissue also showed amyloidosis, particularly in relation to the elastic fibers. These findings seem to represent a unique syndrome of amyloid disease.

Amyloid↗

Atypical fibroxanthoma. A study with antibody to S-100 protein.

Fourteen biopsy specimens from ten patients with atypical fibroxanthoma were studied with the peroxidase-antiperoxidase method for binding of S-100 antibody. Six specimens from four patients were minimally positive. The S-100-positive cells were observed in greatest concentrations at the periphery of the lesions and were associated with perivascular inflammatory cells. By this method, it appears that there is not a Langerhans' cell type of atypical fibroxanthoma. The S-100 antibody can help differentiate desmoplastic juvenile melanoma and malignant melanoma from atypical fibroxanthoma.

Antibodies, Monoclonal↗

Immunofluorescent and histologic study of cold urticaria.

Biopsies of spontaneous and ice-induced wheals of five patients with cold urticaria showed two types of lesions: one with predominant neutrophils and one with predominant lymphocytes. Immunofluorescent studies of the five cases showed nonspecific findings in two cases and negative findings in three cases.

Adult↗

Nodular lymphoid disease of the head and neck: lymphocytoma cutis, benign lymphocytic infiltrate of Jessner, and their distinction from malignant lymphoma.

Skin biopsy specimens from six patients with nodular lymphoid disease of the head and neck were studied by routine histology, direct immunofluorescence microscopy, and leukocyte monoclonal antibodies to T and B cell subsets and monocytes. Initially, these lesions were clinically considered to be benign lymphocytic infiltrates of Jessner, lymphocytoma, or lymphoma. Direct immunofluorescence was negative or showed nonspecific staining in all four patients in whom it was performed. Leukocyte monoclonal antibody stains revealed two distinct patterns of lymphocytes. Lymphocytoma was represented by nodular masses of B lymphocytes with peripheral and intervening zones of T cells. The second pattern consisted of solid nodular masses of T lymphocytes occupying the dermis and subcutaneous tissue. In the specimens interpreted as benign lymphocytic infiltration, the T cells were composed equally of helper and suppressor cells.

Adult↗

Coexistence of lichen sclerosus, morphea, and lichen planus. Report of four cases and review of the literature.

A 39-year-old woman with coexistent lichen planus, lichen sclerosus, and generalized morphea with ulcerations is described. Three additional cases were found in the files of the Mayo Clinic from 1950 to 1983, and these are summarized. Eight cases reviewed in the literature are also summarized. Coexistent lichenoid and sclerodermatous eruptions in graft-versus-host disease after bone marrow transplantation are noted as a model for this combined inflammatory and sclerotic dermatosis.

Adult↗