Biomedical subjects
Publications and source records attributed to R K Winkelmann.
Cutaneous necrotizing vasculitis of small- and medium-sized vessels developed in a patient with adult celiac disease who later was found to have visceral lymphoma with erythrophagocytosis. Immunologic and genetic probe studies showed the lymphoma to be of T cell differentiation. Celiac disease and T cell lymphoma have been associated previously with the development of cutaneous vasculitis.
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We describe five patients aged 50 years or above who had severe atherosclerosis and necrotizing vasculitis. The vasculitis was seen in the small vessels of four patients and in the medium-sized vessels of one. All five patients had multiple necrotic ulcerative skin lesions, and two underwent amputation. Our cases suggest that the relationship between the two disorders changes and exacerbates the clinical and pathological expression of each disease.
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A patient with atrophoderma (Pasini-Pierini) was studied. Microscopic examination showed small collections of mononuclear cells around dermal blood vessels. Electron microscopic study demonstrated macrophages and lymphocytes around vessels and between fibers in the dermis; the epidermis, dermis, collagen, and elastic fibers appeared normal. Monoclonal antibody studies of the cells in the perivascular infiltrate demonstrated cells reacting with anti-Leu-1 (pan-T-cell antibody), anti-Leu-3a (the helper/inducer T-cell antibody), and OKM 1 antibody-reacting cells (macrophages). Direct immunofluorescent studies showed IgM and C3 staining in the small blood vessels of the papillary dermis, scattered IgM cytoids at the basement membrane, and focal fibrinogen in the mid-dermis. Mononuclear cells in the perivascular infiltrate, similar in type and percentage concentration, have been demonstrated also in patients with anetoderma, another rare atrophic cutaneous disorder. Macrophages and T lymphocytes around papillary dermal blood vessels may play a role in the pathogenesis of atrophoderma and anetoderma.
The histopathologic findings in 331 cases of necrobiosis lipoidica diabeticorum seen during a 50-year period were reviewed. Three cases showing cholesterol cleft formation were found. All 3 cases were associated with severe diabetes mellitus. The differential diagnosis of importance is necrobiotic xanthogranuloma. Common features included extensive hyaline necrobiosis and foreign-body giant cells. Atypical and Touton-type giant cells are more common in necrobiotic xanthogranuloma. Vascular changes in necrobiotic xanthogranuloma may include granulomatous involvement of muscular walls with thrombosis. Explanations for cholesterol cleft formation are offered. When cholesterol clefts are seen in biopsy specimens of necrobiosis, necrobiotic xanthogranuloma must be ruled out. In addition, when found in necrobiosis lipoidica diabeticorum, these clefts may imply diabetes mellitus with complications.
We have found a previously undescribed histopathologic feature of necrobiosis lipoidica diabeticorum among 310 biopsied cases: lymphoid nodules. This feature does not correlate with unique clinical lesions or forms of the disease. The lymphoid nodules are similar in appearance to those in other chronic dermal inflammations.
This is the first report of spinous and follicular lichen nitidus associated with perifollicular granuloma formation. Clinicians and pathologists need to distinguish this stage of the disease from lichen scrofulosorum.
Another histological variant of basal cell carcinoma (BCC) is described. This clear-cell variant appeared as a recurrent lesion on the back of an elderly man. Histological examination revealed that part of the tumor was composed of clear cells with faintly eosinophilic cytoplasm. Degenerative changes were present, along with calcium deposition. Mucin was present within the stroma and within the degenerative areas of the tumor. Staining with anti-S-100, anti-keratin, and anti-carcinoembryonic antigen antibodies was negative. Electron microscopy demonstrated epithelium-derived cells with marked phagolysosomal accumulation within the cytoplasm. We conclude that this clear-cell variant is due to degenerative change within a BCC.
Passive transfer experiments in cholinergic urticaria were carried out from 16 patients to a Macaca cymnologous monkey. Intravenous Evans blue dye was used to demonstrate vascular permeability. The animal was challenged after 24 h first by heating the serum-injected dorsal skin to 45 degrees C and secondly by superinjection of acetylcholine into serum-injected sites, and a control site. Local heat proved insufficient to evoke a response. Seven of 16 serum-injected sites showed positive reaction to acetylcholine, control injection of acetylcholine did not. These experiments suggest the presence of a serum factor in cholinergic urticaria which, with acetylcholine, causes increased vascular permeability.
Eight patients with neutrophilic urticaria were identified in a 5-year biopsy experience (1980-1984). All patients had a neutrophilic venulitis without fibrinoid necrosis, hemorrhage, or leukocytoclasia. Four patients had a history of angioedema, and two had a personal history of atopic disease. Results of laboratory studies, including complement and protein values and antibody serologic tests, were normal. All patients responded to antihistamine agents. Despite occasional clinical or histologic diagnoses of vasculitis for such cases in the past, the clinical, laboratory, and histologic features and the therapeutic course of these patients are compatible with a phase or type of chronic urticaria.
Sixteen biopsy specimens from 11 patients with cutaneous large-cell lymphocytoma were studied by a peroxidase method using monospecific antisera to kappa and lambda Ig light chains. Five specimens had polyclonal presence of both kappa and lambda chains. Four specimens were graded as devoid of immunoreactivity, and eight were graded as non-specific because the albumin control staining was as intense as or more intense than the immunoglobulin light chains. Two specimens showing monoclonality for lambda light chains had the cells in the interfollicular spaces only, and the patients have survived for 9 and 12 years without evidence of lymphoma.
Among 14 patients with carcinoma of the lung and scleroderma, 9 had no history of smoking. The 14 cases of primary lung carcinoma occurred in a population of 3550 patients with a diagnosis of scleroderma. Scleroderma preceded the diagnosis of lung cancer by at least 6 years in 8 cases. Scleroderma and lung carcinoma were diagnosed within 3 years of each other in 4 cases. The most frequent type of carcinoma in our series was small cell carcinoma, which accounted for 5 of the 14 cases. Our data indicate an increased risk of carcinoma of the lung in patients with scleroderma, even among nonsmokers. Small cell carcinoma of the lung is probably much more common in patients with scleroderma in whom lung carcinoma develops than is indicated by previous reports.
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Stasis dermatitis, a chronic varicose inflammation of the skin of the lower legs, frequently is complicated by the development of contact hypersensitivity. This retrospective study of forty-six patients with stasis dermatitis found a 60.9 percent incidence of at least one significantly positive patch test reaction. The two allergens that elicited positive reactions most commonly were neomycin and epoxy resin. These results and those of other authors reinforce our contention that patch testing should be a part of the routine work-up of patients with stasis dermatitis.