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Biomedical subjects

R K Winkelmann

Publications and source records attributed to R K Winkelmann.

At least 127 records · Page 7Linked to original sources
PubMed1988

Chronic dermatitis evolving to mycosis fungoides: report of four cases and review of the literature.

In four patients with long-standing chronic dermatitis, evolution into cutaneous T-cell lymphoma was observed. Two patients were men and two were women; they were aged forty-nine to sixty-five years at first presentation. Duration of dermatitis at admission was from two months to fifteen years, with the clinical and histologic diagnosis documented on initial examination. After another five to nine years, clinically and histologically verified mycosis fungoides occurred. In two patients, contact factors were verified as causative by results of patch testing; sensitivities documented included nickel, potassium dichromate, and formaldehyde. In a third patient, contact factors were implicated as causes, but patch testing was not performed. We propose that certain instances of cutaneous T-cell lymphoma may be caused by chronic antigenic immunostimulation with resultant malignant lymphocyte clonality.

Aged↗
PubMed1988

A histological study of the evolution of solar urticaria.

The histological evolution of solar simulator-induced lesions of solar urticaria was investigated in four severely affected white patients. A series of two to 32 minimal whealing doses of radiation, each much lower than the 24-hour minimal erythema dose, was administered to separate buttock sites. Biopsy specimens were obtained from the exposed areas at five minutes and two and 24 hours later, as well as from adjacent nonexposed skin. Lesions showed a statistically significant dose-dependent increase, predominantly perivascular, in upper dermal neutrophil and eosinophil numbers at five minutes and two hours, but not at 24 hours, and at higher radiation doses in mononuclear cell numbers by 24 hours. Nonirradiated patient skin and irradiated control subject skin was not similarly affected. These changes may be associated with the pathogenesis of solar urticaria.

Adult↗
PubMed1987

Cholinergic urticaria. A clinical and histologic study.

We studied the natural history, the prevalence of atopy, and the frequency of systemic symptoms during attacks in 35 patients with cholinergic urticaria, the histologic condition of the eruption in seven patients (20%), and the response to intradermal injections of acetylcholine, histamine, and methacholine in 18 patients (51%). In most patients symptoms began between the ages of 10 and 30 years, persisted for many years, and caused them to modify their activities to avoid the provoking factors of exercise, emotion, and heat. The condition usually improved with time, and five patients (14%) had a spontaneous remission. Atopy, present in about 12 (34%) of the patients, was more frequent than in the general population. Systemic symptoms were uncommon during attacks, and patients had no greater responses than controls to the intradermal tests. The histologic study revealed neutrophils in and around the walls of superficial subpapillary dermal vessels.

Adolescent↗
PubMed1987

Familial anetoderma.

Two families with anetoderma are described. Unlike previous reports of familial anetoderma, the disease process seemed to be limited to the skin, and there were no associated ocular, gastrointestinal, or orthopedic anomalies in the affected patients or in any other family members. Although infrequently reported, anetoderma may occur in families, and patients must be examined for associated systemic abnormalities for a thorough assessment of their skin disorder.

Adult↗
PubMed1987

Extracellular deposition of eosinophil granule major basic protein in pressure urticaria.

Utilizing affinity chromatography-purified antibody to the eosinophil granule major basic protein and formalin-fixed paraffin embedded tissue, we investigated the localization of major basic protein by immunofluorescence in twenty-four skin biopsy specimens from ten patients with pressure urticaria. Fourteen of twenty-four biopsy specimens were obtained from spontaneously occurring urticarial lesions of 4 to 48 hours' duration, and ten of the twenty-four specimens were from dermographometer-induced lesions that had been present from 40 minutes to 24 hours. Twenty-one of twenty-four biopsy specimens showed extracellular fluorescence of eosinophil granule major basic protein within the dermis. The extent and intensity of extracellular staining were not related to the presence or degree of tissue eosinophilia. Serial section controls from each block were stained with protein A purified rabbit IgG and were negative. Previous immunofluorescence studies have demonstrated deposition of major basic protein in lesions of chronic idiopathic urticaria, episodic angioedema, and facial edema. Major basic protein causes release of histamine from human basophils and induces wheal-and-flare reactions on intradermal injection. The present observations add further evidence to support a role for eosinophil mediators, particularly major basic protein, in the pathogenesis of cutaneous disease characterized by edema.

Adult↗
PubMed1987

Familial granuloma annulare. Report of two cases and review of the literature.

We report two sisters with granuloma annulare who possessed identical histocompatibility antigens. The occurrence of granuloma annulare in families and the demonstration of particular human lymphocyte antigens (HLA) with granuloma annulare may indicate that hereditarily predisposed individuals could develop this specific cell-mediated immune reaction in response to an unknown antigen (e.g., connective tissue or infectious or toxic substances).

Adolescent↗
PubMed1987

Localized scleroderma (morphea) and antibody to Borrelia burgdorferi.

Enzyme-linked immunosorbent assay (ELISA) and immunofluorescence assay (IFA) were performed in 25 and 32 cases of morphea, respectively. The more sensitive and specific ELISA was positive in only 1 of 25 cases and the mean value was lower in cases of morphea than in controls. IFA showed minimally reactive titers in 6 (19%) of 32 cases. There were an additional six cases with borderline titers. These data indicate that there is no specific association between Borrelia burgdorferi infection and morphea but that patients with morphea tend to have circulating antibodies that are cross-reactive.

Antibodies, Bacterial↗
PubMed1987

Cutaneous manifestations of Takayasu's arteritis. A clinicopathologic correlation.

Takayasu's arteritis is a chronic, granulomatous, large-vessel arteriopathy of unknown cause. We retrospectively reviewed the medical records of 38 patients with Takayasu's arteritis and identified 21 with cutaneous findings. Seven patients had lesions that were related to their systemic vasculitis. We found a Churg-Strauss granuloma, a pyodermatous leg ulcer, and inflammatory leg nodules in these patients. Biopsy specimens from three patients with presumed "erythema nodosum" did not support the clinical diagnosis but did show arteritis. In patients with Takayasu's arteritis, small-vessel inflammation, and other inflammatory lesions may be present, in addition to large-vessel disease. Histopathologic study is necessary to categorize the nature of inflammatory leg nodules of these patients.

Adolescent↗
PubMed1987

Symptomatic dermographism (factitious urticaria)--passive transfer experiments from human to monkey.

Passive transfer experiments were carried out on three species of monkey, Macaca mulatta, Macaca nemestrina and Macaca fascicularis, using human serum from patients affected with severe symptomatic dermographism (factitious urticaria), cholinergic urticaria, chronic idiopathic urticaria and normal subjects. The monkeys were tested for dermographism by means of a calibrated dermographometer 24 h after intradermal injection of the serum, using Evans blue as a marker. Positive responses were seen initially in the M. nemestrina. Four sites injected with serum from patients with severe symptomatic dermographism gave positive responses, one site injected with serum from a normal subject produced a faint response. One of the four responses was reproduced one month later in M. fascicularis. These results indicate that passive transfer of dermographism is possible from human to monkey.

Animals↗
PubMed1987

The cellular inflammatory response in nicotinate skin reactions.

Sequential skin biopsies of nicotinate-treated skin from nine normal subjects, three aspirin-pretreated normal subjects and six atopic eczema patients were examined. An erythematous skin reaction was seen in the nine normal subjects and to a lesser degree in one atopic eczema patient, but not in the aspirin-pretreated subjects nor in five remaining atopics. Accumulation of a mononuclear cell perivascular infiltrate was seen from 15 min onwards in the normal subjects. Neutrophils became the predominant cell invading thickened vessel walls and in the perivascular space beginning at 2 h and persisting up to 48 h. Leucocytoclasis was observed at 24 h. Immunofluorescence studies showed only non-specific fibrinogen deposits in papillary capillaries in the three groups of subjects. The chloroacetate esterase reaction and immunohistochemical labelling with OKM I confirmed a marked neutrophilia at 2 h and 24 h. Neutrophils were seen in one atopic eczema patient, but were not observed in the remainder, nor in the skin of the aspirin-pretreated normal subjects. Topical application of nicotinate causes non-allergenic, leucocytoclastic vascular damage in normal skin which can be inhibited by aspirin and which is reduced or absent in atopic eczema.

Adult↗
PubMed1987

Sensitivity to wild vegetation.

74 patients suspected of having allergic contact dermatitis to wild vegetation were patch tested with either extracts of 13 plants of the family Compositae and 7 other weeds or trees. Anthemis cotula (dog fennel) and Xanthium strumarium (cocklebur) gave the most frequent positive results, demonstrating a change of frequency in sensitivity compared to the 1950s, when Ambrosia artemisiifolia (ragweed) was recognized as the most frequently sensitizing weed. The reasons for these changes of incidence and clinical patterns are examined.

Dermatitis, Contact↗
PubMed1987

Histopathology of necrobiotic xanthogranuloma with paraproteinemia.

Fifty-three cutaneous biopsies from 22 patients with necrobiotic xanthogranuloma (NXG) were reviewed. One or more biopsies from each patient displayed a typical pattern of palisading histiocytic xanthogranuloma with bands of hyaline necrobiosis. Multiple foam cells and Touton giant cells were present, and atypical, bizarre-appearing foreign body giant cells were characteristic. Cholesterol clefts were found in 18 specimens, and lymphoid nodules were found in 24, 8 of which had germinal center formation. Twenty-one specimens contained foci of plasma cells, and these were located perivascularly, at the periphery of lymphoid nodules, and, as infiltrates, between dermal collagen bundles. Unique features were xanthogranulomatous panniculitis, often appearing as Touton cell panniculitis, and a rare but distinctive palisading cholesterol cleft granuloma. The cytoplasm of giant cells and histiocytes contained PAS-positive, diastase-resistant polysaccharide. Examination of 3 cases by electron microscopy revealed dendritic cells in the epidermis and vacuolated dermal histiocytes. S-100 antibody was negative in 3 cases. Leukocyte monoclonal antibody studies in 6 patients showed predominantly T-helper lymphocytes within the granuloma. NXG is a T-helper cell, non-X histiocytic granuloma with a distinctive histopathology.

Granuloma↗
PubMed1987

Alpha 1-antitrypsin deficiency panniculitis: a histopathologic and immunopathologic study of four cases.

Four cases of alpha 1-antitrypsin deficiency associated with panniculitis were reviewed. The following histopathologic characteristics were typically present: (a) large areas of normal panniculus adjacent to severe necrotic panniculitis; (b) acute panniculitis--masses of neutrophils causing necrosis and replacing fat lobules; (c) chronic inflammation and hemorrhage at the periphery of acute panniculitis; (d) focal collection and proliferation of histiocytic cells and lipophages; (e) secondary leukocytoclastic vasculitis in areas of necrosis and lymphocytic vasculitis in areas of severe inflammation, but no evidence of primary vasculitis; (f) phlebothrombosis; and (g) in partial (heterozygous) deficiency, pronounced lipophages and giant-cell replacement of fat cells. Endarteritis obliterans was noted. Direct immunofluorescence study showed C3 deposition in the blood vessels of the panniculus or dermis (or both) in all four cases, and IgM was present in the blood vessels of three. Weak granular deposits of IgM or C3 were seen at the epidermal basement membrane zone in two cases. C3 in endothelial cells of the blood vessels was detected in one case. In general, the immunoreactants in the blood vessels were not dense and probably represent secondary vasculitis. We conclude that all patients with severe panniculitis and ulceration should have alpha 1-antitrypsin levels determined. A deep excisional biopsy specimen with abundant panniculus tissue is required for histopathologic diagnosis.

Adipose Tissue↗
PubMed1986

Juvenile xanthogranuloma with central nervous system lesions.

We describe two boys in whom extensive normolipemic cutaneous papulonodular xanthomatous lesions developed and disabling central nervous system disease later developed. Histologically, lesional skin from each patient was consistent with juvenile xanthogranuloma. Electron microscopic studies in each case failed to reveal Langerhans (Birbeck) granules. Results of S-100 protein studies done with the peroxidase-immunoperoxidase technic were negative in each case. Radiation therapy and systemic corticosteroids have been partially successful in controlling the cerebellar ataxia experienced by one of the patients. The other patient had progressive central nervous system disease and died 8 years after the onset of his skin lesions despite radiation therapy and systemic chemotherapy. We believe that these cases are examples of normolipemic, xanthomatous, non-X histiocytosis with features of juvenile xanthogranuloma and aggressive central nervous system disease.

Brain Diseases↗
PubMed1986

Bullous pemphigoid and glomerulonephritis. Report of four cases.

We report on four patients with bullous pemphigoid and other autoimmune diseases who had or developed glomerulonephritis. The relationship between these two immunologic disorders involving the basement membrane is discussed. Our data suggest a coincidental phenomenon in patients who successively or concurrently have multiple diseases of autoimmunity.

Adult↗
PubMed1986

Cutaneous granulomatous vasculitis: its relationship to systemic disease.

Microscopic and medical review of twenty-six patients with skin biopsy specimens that showed granulomatous vasculitis demonstrated vascular histiocytic granulomas with fibrinoid destruction of blood vessels in the dermis and panniculus. Cultures of the biopsy specimens were nonspecific. The skin lesions varied from erythema to papulonodular and vesicular eruptions; they were usually on the extremities but also involved the trunk. Eight patients had systemic lymphoproliferative diseases: three, lymphoma; two, angioimmunoblastic lymphadenopathy; two, preleukemia; and one, chronic granulocytic leukemia. Five of these eight patients died within 2 years after the onset of skin lesions. The four patients with systemic vasculitis died within 1 year after the onset of skin lesions. Five patients with arthritis, four with gastrointestinal disease, three with systemic sarcoidosis or sarcoidlike disease, and one with tuberculosis had a more favorable prognosis. The histologic pattern of cutaneous nonlymphomatoid granulomatous vasculitis is associated with significant systemic disease, especially lymphoproliferative disorders. Patients with lymphoproliferative disorders or systemic vasculitis have a much poorer prognosis than those with inflammatory or infectious granulomatous disease.

Adult↗