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Biomedical subjects

R K Winkelmann

Publications and source records attributed to R K Winkelmann.

At least 199 records · Page 11Linked to original sources
PubMed1983

Cutaneous malignant histiocytosis: a clinical and histopathologic study of eight cases, with immunohistochemical analysis.

Eight cases of autopsy-proved malignant histiocytosis (MH) are reported, in which cutaneous involvement was a prominent finding at initial clinical presentation. In two patients, the disease remained confined to the skin for significant periods before systemic dissemination appeared. Immunohistochemical analysis of cutaneous infiltrates of MH showed uniform negativity of the atypical cells for lysozyme, immunoglobulin light chain, glycogen, and chloroacetate esterase content. In five cases, cytochemical stains for acid phosphatase, butyrate (nonspecific) esterase, chloroacetate esterase, and peroxidase activity were performed on bone marrow aspirates containing malignant cells; all demonstrated diffuse positivity for acid phosphatase and nonspecific esterase, and negativity for peroxidase and chloroacetate esterase. Touch-imprint preparations of cutaneous lesions in one of these cases yielded identical results. These findings indicate that cytochemical methods are preferable to immunohistochemical technics in identification of histiocytic malignancies and that foci of skin involvement may provide a useful source for such diagnostic evaluation.

Acid Phosphatase↗
PubMed1983

The clinicopathologic spectrum of lymphomatoid papulosis: study of 31 cases.

Herein we review the Mayo Clinic experience with thirty-one cases of lymphomatoid papulosis seen since 1965. All patients had chronic, recurrent, and self-healing erythematous papulonodular lesions, which often became pustular, ulcerated, and resolved with scarring. The clinical features often corresponded to those seen in Mucha-Habermann disease; however, the predominant histopathologic feature was an infiltrate composed primarily of atypical lymphoid cells suggestive of malignant lymphoma. In six patients, a lymphoproliferative disorder was eventually diagnosed. There were two cases of mycosis fungoides (stage I), one case of nodular sclerosing Hodgkin's disease, and three cases of malignant lymphoma--one diffuse mixed large and small cell type with features of T-immunoblastic type, one diffuse large cell type, and one follicular small cleaved cell type. The clinical course of the lymphomatoid papulosis was unaffected by chemotherapy for the lymphoproliferative disorder. Our data indicate that, with sufficient duration of follow-up, malignant lymphoma may develop in some patients with lymphomatoid papulosis.

Adolescent↗
PubMed1983

Keratoderma and spastic paralysis.

Punctate keratoderma and spastic paralysis occurred in three generations of a family. Several members had keratoderma of the palms and soles or spastic paralysis or both. The family history was consistent with autosomal-dominant inheritance. The age at onset and the rate of progression of symptoms were variable. The concurrence of these lesions can be interpreted to mean either that the keratoderma and the paraplegia are the pleiotropic effects of the same mutant gene or, less likely, that they are the manifestations of two different autosomal mutations segregating in this family. We are not aware of a similar syndrome having been previously reported.

Adult↗
PubMed1983

Malignant pyoderma.

Malignant pyoderma is a rare, chronic, destructive ulcerating skin disease of unknown cause that affects young adults, usually males. In the seven previously reported cases, the lesions were distributed mainly about the head and neck region, and some were associated with neurological disturbances. The disease is progressive but responds to high doses of systemically administered steroids. We here describe an additional case and a follow-up of a previously reported case that was managed successfully with corticosteroid and cyclophosphamide therapy.

Adolescent↗
PubMed1983

Vascular ulcers in scleroderma.

Seven patients with scleroderma and either livedo reticularis or atrophie blanche lesions had ulcers of the lower extremity. Livedoid vasculitis, periarteritis nodosa, or endarteritis obliterans lesions were associated with the ulcers. Five patients had livedoid vasculitis, one patient had associated lupus panniculitis, and one patient had rheumatoid arthritis. Four patients had elevated ESRs, four had positive antinuclear antibody tests, and two had positive tests for rheumatoid factor. Patients with scleroderma and livedoid vasculitis or livedo reticularis and ulcers should be examined to rule out underlying vascular disease or endarteritis obliterans.

Adolescent↗
PubMed1983

The T-lymphocyte and cutaneous Churg-Strauss granuloma.

Studies of biopsy specimens from 4 patients with Churg-Strauss granulomas of the skin were performed with monoclonal antibodies to T-lymphocyte subset antigens. The finding of a predominance of a LEU3 antibody-reactive helper T-cell population in the perivascular areas and in the palisading and central granuloma areas was noteworthy. Pronounced reduction of suppressor T-cells in the lesions was observed in 2 patients; the lesions were associated with vasculitis and rheumatoid arthritis in one patient and with vasculitis and lupus erythematosus in the other. The Churg-Strauss granuloma demonstrated features of a cutaneous helper T-cell granuloma.

Antibodies, Monoclonal↗
PubMed1983

Identification of T-cell subpopulations in granuloma annulare.

Granuloma annulare is a lymphohistiocytic process of unknown cause characterized by necrobiotic dermal papules. Acetone-fixed frozen sections of eight granuloma annulare lesions were studied for the presence of T-cell subsets, using monoclonal antibodies to T-cell surface antigens. The palisading and perivascular lymphocytic infiltrate shows that most of the mononuclear cells are reactive with LEU-1 antibody, which characterizes peripheral T cells and activated T cells. Numerous cells were reactive with LEU-3a antibody, which identifies the helper-inducer T-cell subset. Some cells were positive with LEU-2a antibody, which detects suppressor-cytotoxic cell populations. Masses of histiocytes were identified infiltrating between the collagen fibers and surrounding the central necrobiosis area; the histiocytes showed a very strong diffuse acid phosphatase and nonspecific esterase activity. These findings suggest that a cell-mediated immune response may be the dominant pathogenic event in granuloma annulare.

Antibodies, Monoclonal↗
PubMed1983

Pre-Sézary erythroderma evolving to Sézary syndrome. A report of seven cases.

Seven patients with pre-Sézary erythroderma were followed up for three to 16 years as Sézary erythroderma syndrome developed. With the transition, the clinical and skin manifestations of the disease became chronic, more florid, and more resistant to therapy. Four patients had multiple contact allergies or drug reactions, and one patient had severe atopic dermatitis. Lymphoma developed in one patient, and multiple myeloma developed in a second patient. An increased absolute number of Sézary cells to more than 1,000/cu mm occurred in all patients with Sézary syndrome. Histologic study showed chronic dermatitis in most patients with the pre-Sézary condition and subepidermal lymphocytic band inflammation in most patients with the Sézary syndrome.

Aged↗
PubMed1983

T-cell subsets in cutaneous sarcoidosis.

Skin lesions from four patients with systemic and cutaneous sarcoidosis were studied, by the use of monoclonal antibodies, for the presence of T cells and T-cell subsets. Large numbers of lymphoid cells reacting with anti-pan T-cell (LEU-1) and anti-helper and inducer subset (LEU-3) monoclonal antibodies were observed around and within the sarcoid granulomas in three of the four patients. Only rare LEU-2-reactive suppressor cells were observed in all four patients. Activated T lymphocytes with focal acid phosphatase activity, together with epithelioid cells and multinucleated giant cells with strong diffuse activity of acid phosphatase and nonspecific esterase, were identified within the granulomas. The two patients with active disease demonstrated substantially more T cells in the sarcoid granulomas than did the two patients with chronic disease. Our study results suggest the importance of helper T cells in the formation of the sarcoid granuloma by mononuclear phagocytes and imply that the activity and duration of disease may be related to the T-cell populations.

Acid Phosphatase↗
PubMed1983

T cells in cutaneous lesions of Sézary syndrome and T-cell leukemia. Characterization by monoclonal antibodies.

Anti-T-cell monoclonal antibodies (LEU series) immunoperoxidase technique study for the presence of T cells in cutaneous lesions from four patients with Sézary syndrome and one patient with chronic T-cell leukemia showed that most dermal-lymphoid cells from three patients with Sézary syndrome were reactive with monoclonal antibodies to anti-pan T-cell (LEU-1) and helper T-cell (LEU-3a) subsets but not with those to suppressor-cytotoxic T-cell (LEU-2a) subsets. One patient with progressive disease had atypical dermal-lymphoid cells positive for pan T-cell (LEU-1). Epidermotropic cells were reactive to LEU-1 in all four patients, LEU-2a in one patient, and LEU-3a in one patient. Neoplastic cells in skin lesions of chronic T-cell leukemia showed strong positive staining with LEU-1, but were reactive with both anti-T-cell subset, monoclonal antibodies. The atypical, dermal-lymphoid cells in Sézary syndrome represent mature, helper T cells in most cases. The absence of T-cell subset antigens in one patient with fulminant Sézary syndrome and the finding of both T-cell subset antigens on T-cell leukemia cells suggest the presence of actively proliferating, immature T cells in those cases.

Adult↗
PubMed1983

Panniculitis in connective tissue disease.

Specific and nonspecific panniculitis occur in connective tissue diseases. Microscopic studies of the epidermis and dermis, immunofluorescent microscopy, and extensive serologic testing may aid in the diagnosis and classification of these unusual syndromes. Connective tissue panniculitis and focal lipoatrophy may be forms of immunoreactive panniculitis or connective tissue disease (or both) in which typical microscopic, serologic, or other events related to classic connective tissue disease have not occurred.

Adult↗
PubMed1983

Porphyria cutanea tarda and chronic renal failure.

Bullous dermatosis developed in a patient with renal failure who was on hemodialysis. The results of standard tests for porphyria cutanea tarda--quantitation of urinary and fecal porphyrins by fluorometric assay--were within normal limits or were nondiagnostic. Assay of plasma using high-pressure liquid chromatography, however, revealed that this patient had a porphyrin profile consistent with porphyria cutanea tarda. A porphyria-like bullous eruption may occur in patients with chronic renal failure; however, in only a few cases have laboratory studies confirmed the diagnosis of porphyria cutanea tarda. Our experience suggests that, in patients with renal failure, a plasma assay may be more reliable than tests of the urine and feces in establishing a diagnosis in those patients who actually have disorders of porphyrin metabolism. We also report the results of assay of erythrocyte uroporphyrinogen decarboxylase activity and fecal isocoproporphyrin.

Adult↗
PubMed1983

Subcorneal pustular dermatosis and pustular psoriasis. A clinicopathologic correlation.

The relationship between subcorneal pustular dermatosis (SCPD) and pustular psoriasis is discussed on the basis of a study of 23 patients with SCPD seen at the Mayo Clinic, Rochester, Minn, since 1956. In seven of the 23 patients, pustular psoriasis subsequently developed, and in three other patients there was associated psoriasis vulgaris. It was impossible to make a histopathologic distinction between SCPD and some cases of pustular psoriasis. The response to therapy with sulfones was erratic, if not ineffective. Our data suggest that disease identical to SCPD is, in many instances, strongly associated biologically, clinically, and histopathologically with psoriasis, and that continued observation of the clinical course may further substantiate this relationship in many cases.

Adult↗
PubMed1983

Further experience with toxic vitamin A therapy in pityriasis rubra pilaris.

A patient with short-duration pityriasis rubra pilaris was successfully treated with high-dose, toxic vitamin A (retinol), but the disease subsequently recurred in new areas. Serum levels of vitamin A were highest four hours after treatment and returned to the base level within twelve hours. Fasting blood levels of vitamin A during treatment increased to five times the pretreatment level. Ultrastructural changes in the keratinocytes were notable vacuolation, granularity of the cytoplasm, and a decrease in tonofilament masses, all indications of the cellular effect of the vitamin A. We believe that patients with long-duration pityriasis rubra pilaris should be considered for this treatment.

Aged↗