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Biomedical subjects

R K Winkelmann

Publications and source records attributed to R K Winkelmann.

At least 181 records · Page 10Linked to original sources

Monoclonal gammopathy and mycosis fungoides. Report of four cases and review of the literature.

Of four patients with mycosis fungoides and monoclonal gammopathy, one died of multiple myeloma that developed 4 years after the mycosis fungoides. The other three had monoclonal gammopathy of undetermined significance. This association does not appear to occur with a high frequency in patients with mycosis fungoides than in the general population. However, it demonstrates that B cell proliferation can occur in patients with primary cutaneous T cell disease.

Aged↗

Pre-Sézary syndrome.

Eighteen patients with erythroderma, recurrent cycles of circulating Sézary cells of less than 1,000 cells/mm3, and a chronic course were followed for a mean time of nearly 5 years and were diagnosed as having pre-Sézary syndrome. Only one patient died, and none developed lymphoproliferative disease. All ten patients who underwent patch testing showed positive results. The elevation of IgE was striking when this group was compared with a group with Sézary syndrome. Most patients achieved partial or complete remission on low-dose chlorambucil and prednisone therapy. Some patients had lymphocytic or lymphomatoid bands on skin biopsy specimens and were like previously reported patients with pre-Sézary syndrome whose condition progressed to Sézary syndrome. A nontoxic chemotherapy or an anti-T cell treatment program can control this chronic erythroderma state.

Adult↗

The treatment of Sézary syndrome.

The results of treatment programs were studied in forty patients with Sézary syndrome. Thirty-three patients died. The principal treatment was a low-dose chlorambucil and prednisone regimen. Patients so treated lived longer from diagnosis of erythroderma (median survival, 6.2 yr) than did patients on other regimens (median survival, 3.05 yr). The addition of x-ray or chemotherapeutic programs did not increase significant long-term benefit. Seven patients receiving chlorambucil and prednisone had remission (1 yr or more); most patients had partial remission, but two patients did not have a response to this program. The complications of sepsis, progressive disease, and lymphoma occurred both in patients who were and in those who were not treated with chlorambucil and prednisone. We believe that a regimen of low-dose chlorambucil and prednisone is satisfactory treatment that can be the basis for comparison of new therapeutic approaches to Sézary syndrome.

Adult↗

Lymph node histopathology in Sézary syndrome.

Twenty-six lymph node biopsy specimens from twenty-three patients with Sézary syndrome were microscopically evaluated and compared with nodal specimens from six patients with pre-Sézary syndrome and four control patients with dermatopathic lymphadenopathy. Three phases of nodal involvement by cerebriform lymphocytes were perceived. A trend toward greater effacement of lymph node tissue (phase II or phase III) could be related to longer duration of disease. Unchanged stable histologic features were seen in repeated specimens at intervals as long as 6 years. Cases of pre-Sézary syndrome were of phase 0 or phase I, with nodal histology overlapping that of many of the cases of typical Sézary syndrome. In contrast to mycosis fungoides, Sézary syndrome, with its hematogenous circulation of cerebriform cells, features early recognizable nodal infiltration. The degree of nodal involvement does not consistently correlate with the prognosis.

Adult↗

Treatment of varicella-zoster pneumonia with transfer factor.

A 29-year-old woman with a long history of immunoreactive disease--thrombocytopenic purpura, bullous pemphigoid, nephropathy, and hemolytic anemia--contracted generalized herpes zoster and varicella pneumonia. Respiratory failure requiring assisted respiration accompanied progressive chest findings. She recovered rapidly simultaneous with the administration of transfer factor from a healing herpes zoster patient. We believe that this therapy should be attempted in similar desperate circumstances.

Adult↗

Ultrastructural findings in the skin lesions of patients with anetoderma.

Eight biopsy specimens from the skin lesions of five patients with anetoderma were studied for their ultrastructural findings. In all of them, normal elastic fibers were absent and a few very thin, irregular elastic fibers with a more or less complete loss of the amorphous substance and a relative conservation of the microfibrils were observed. The collagen fibers were normal. Inflammation composed of macrophages and lymphocytes, with some plasma cells, was a prominent finding. It is suggested that anetoderma and acquired cutis laxa are part of the same spectrum of elastolytic disease.

Adolescent↗

Total plasma kininogen in psoriasis and atopic dermatitis.

Total plasma kininogen was measured in 75 normal individuals and in patients with extensive dermatoses. Elevated levels were found in 20 of 30 hospitalized patients with atopic dermatitis. Elevated levels were observed in 33 psoriasis patients. Nine atopic patients responded to intravenous calcium gluconate with elevated levels during three hours of observation in contrast to no response to control injections or in control individuals. Extensive chronic inflammatory skin disease correlates with elevated total plasma kininogen.

Adult↗

Thalidomide treatment of prurigo nodularis.

Four patients with classic recalcitrant prurigo nodularis had symptomatic and physical responses to thalidomide with remissions. Three of the four patients had increased IgE levels that decreased during therapy. In two patients, short-term treatment (2 to 3 months) was not sufficient to produce remission, but retreatment was effective. Two patients had long-term remission with more than 6 months of treatment. No significant side effects occurred.

B-Lymphocytes↗

T cells and T-cell subsets in mycosis fungoides and parapsoriasis. A study of 18 cases with anti-human T-cell monoclonal antibodies and histochemical techniques.

Skin lesions from 15 patients with mycosis fungoides (MF) and from three with parapsoriasis were studied immunohistochemically with monoclonal antibodies against T cells (Leu 1) and against T-cell subsets (Leu 2a, Leu 3a). Lymphoid cell reactivity was diverse among these sampled cases. In two cases of parapsoriasis and nine of MF, there was a predominance of helper/inducer (Leu-3a-reactive) cells over suppressor/cytotoxic (Leu-2a-reactive) cells. In one case of parapsoriasis and one (advanced tumor stage) of MF, there was suppressor/cytotoxic cell predominance. One case of MF showed strong reactivity for both T-cell subset markers. Four cases of MF (two plaque-stage and two tumor-stage) featured a predominant cell type in the dermis which was nonreactive for all three antibodies. The intraepidermal lymphoid cellularity was Leu-1-reactive in ten cases of MF and two of parapsoriasis. Among these 12 cases, the intraepidermal cellularity was Leu-2a-reactive in four and Leu-3a-reactive in three. The use of such studies of T-cell subsets on in situ cutaneous lymphoid infiltrates may demonstrate a correlation with cytomorphology, clinical stage, and disease prognosis.

Adult↗

Subcutaneous sarcoidosis.

Subcutaneous sarcoidosis is a rare cutaneous expression of systemic sarcoidosis. It is first observed as subcutaneous nodules, principally on the extremities, which show a typical histopathologic appearance of sarcoidosis localized to the subcutaneous tissue. It may be associated with the early benign hilar adenopathy syndrome of sarcoidosis or with the later stages of progressive sarcoidosis. We present three previously unreported cases of subcutaneous sarcoidosis and a review of the literature on this disorder. In the past, the term Darier-Roussy sarcoid had been used for many different forms of granulomatous panniculitis, including sarcoidosis. This usage has caused confusion, and we prefer the term subcutaneous sarcoidosis.

Adult↗

Histopathologic findings in anetoderma.

We reviewed the histopathologic findings in 34 biopsy specimens from 15 patients with anetoderma. Focal loss of normal elastic fibers, necessary for the diagnosis of anetoderma, was found in all 15 cases. The persistence of fine, irregular, or twisted elastic fibers is common. A perivascular infiltrate composed of lymphocytes was found in all specimens. Plasma cells were observed in specimens from six patients, and histiocytes with some granuloma formation were found in specimens from six patients. Variations in the intensity of inflammation or in the loss of elastic tissue were not related to the clinical findings, course, or associated diseases. All anetoderma lesions appeared to have an inflammatory pathogenesis.

Atrophy↗

Gynecologic carcinoma associated with dermatomyositis-polymyositis.

Dermatomyositis-polymyositis was associated with gynecologic carcinoma in ten patients treated at the Mayo Clinic between 1952 and 1982. Only two patients did not manifest the typical skin changes of acute dermatomyositis. In all ten cases, the onset of dermatomyositis-polymyositis preceded evidence of carcinoma. The intervals between initial symptoms of dermatomyositis-polymyositis and subsequent diagnosis of carcinoma ranged from three months to six years. Usually, the carcinoma became evident within two years. The carcinomas were ovarian in five cases, cervical in three, endometrial in one, and vaginal in one. Ages of the ten patients ranged from 40 to 66 years (mean, 53 years). In most cases, symptoms of dermatomyositis-polymyositis regressed markedly after diagnosis and treatment of the underlying carcinoma. In selected patients for whom more detailed follow-up information was available, the manifestations of dermatomyositis-polymyositis progressed and regressed in synchrony with tumor spread and effective treatment, respectively.

Adenocarcinoma↗

Metastatic prostate carcinoma manifesting as penile nodules.

In three patients with prostate carcinoma, metastasis was manifested as nodular lesions about the corona and glans penis. One patient had priapism in addition. The patients had no other cutaneous metastatic lesions. Two of the patients died within two years of the appearance of the penile nodules, but the lesions in the third patient, followed up for one year to date, responded to intravenously administered mitomycin.

Adenocarcinoma↗

Atopic dermatitis and Sézary syndrome.

A patient with severe atopic dermatitis had pre-Sézary and Sézary syndromes. Although elevated serum IgE values in mycosis fungoides and Sézary syndrome are not infrequent, the direct sequential occurrence of atopic dermatitis and Sézary syndrome is uncommon. The clinical and laboratory changes we observed imply that the relationship of these disorders may be conditioned by changes in immunoresponsiveness.

Adult↗

Sézary syndrome. A clinicopathologic study of 39 cases.

The histopathologic and clinicopathologic characteristics of 121 skin biopsy specimens from 39 patients with Sézary syndrome were reviewed. The most frequently noted histologic type was a lymphomatoid subepidermal band infiltrate, composed predominantly of atypical lymphoid cells with cerebriform nuclei, found in 53 (44%) of the skin biopsy specimens. A lymphocytic band infiltrate, characterized by a predominance of small lymphocytes and a variable admixture of atypical lymphoid cells, was found in 47 (39%) of the specimens. Only 18 (15%) of the lymphomatoid band specimens from ten patients demonstrated epidermal involvement suggesting mycosis fungoides. Twenty-one (17%) of the biopsy specimens were interpreted as showing changes consistent with those of chronic dermatitis. Despite multiple skin biopsy specimens, there was a weak association noted between the histologic patterns and the clinical stage of disease or the prognosis.

Adult↗

Cutaneous histiocytosis X. The presence of S-100 protein and its use in diagnosis.

The cellular localization of glial S-100 protein was investigated in paraffin-embedded sections of cutaneous histiocytosis X and in a variety of cutaneous infiltrative disorders, including juvenile xanthogranuloma, necrobiotic xanthogranuloma, papular xanthoma, eruptive histiocytoma, and reticulohistiocytosis. Immunoperoxidase staining with a rabbit anti-calf brain-S-100 antibody demonstrated strong and consistent activity in atypical histiocytes in all histiocytosis X specimens. No detectable S-100 protein was demonstrated in either histiocytes or giant cells in the non-X histiocytic disorders investigated. Positive controls were included within both groups of disorders in respect to melanocyte, epidermal Langerhans' cell, and dermal Schwann's cell staining. These findings are interpreted as evidence for diversity in the mononuclear phagocyte system and demonstrate the practicality of such a simple test in diagnostic problems involving infiltrative histiocytic disorders of the skin.

Histiocytosis, Langerhans-Cell↗

Plasma exchange: a controlled study of the effect in patients with Raynaud's phenomenon and scleroderma.

Six female patients with stage I and II vascular scleroderma and Raynaud's phenomenon were treated with plasma exchange and placebo plasma exchange. Placebo exchange consisted of the return of the patient's own separated plasma. No consistent long-term objective improvement was demonstrated in patients treated with either plasma exchange or placebo plasma exchange. Immediate increases in pulse volume, digital blood pressure, and skin temperature occurred in some patients with both procedures. Mechanisms of change remain unexplained, and further study is warranted.

Adult↗