Serum angiotensin-converting enzyme in granuloma annulare.
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Biomedical subjects
Publications and source records attributed to R K Winkelmann.
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Isoantigens A, B, H(O) in biopsy specimens of 26 normal skin and in 35 adnexal tumors were studied by the red cell adherence (RCA) test of Davidsohn. Isoantigens were detected in the stratum corneum, stratum granulosum, stratum spinosum, acrosyringium, keratogenous zone of hair follicle, eccrine duct, and eccrine gland. The apocrine gland and duct consistently had negative test results for isoantigens. Seventy percent of all tumors tested contained isoantigens and included syringoma, hidrocystoma, syringocystadenoma papilliferum, spiradenoma, eccrine poroma, dermal duct tumor, clear cell hidradenoma, and cylindroma. We interpreted the presence of isoantigens in adnexal tumors as being evidence of immunologic maturity with differentiation toward eccrine structures. The RCA test is a new, sensitive, immunologic technique that can complement enzyme histochemical stains and electron microscopy in the study of the histogenesis of adnexal skin tumors.
A 24-year-old woman had massive purulent and verrucous vegetations with fissuring in the groin and axillae. She also had scaling, crusted, bullous, and purulent lesions on the scalp, face, axillae, umbilicus, groin, and hands. Biopsy specimens of skin for histopathologic examinations and direct immunofluorescent study showed typical bullous pemphigoid, and serial serum samples tested by indirect immunofluorescent study confirmed the diagnosis. A basement membrane antibody was presented on four occasions at titers of 320, 1,280, 169, and 320. The differential diagnosis included dermatitis vegetans and pemphigus vegetans. An appropriate term for this manifestation of bullous pemphigoid is "pemphigoid vegetans."
Scleromyxedema is an uncommon cutaneous fibromucinous disease with a monoclonal protein, which has resisted a number of therapies. Eight cases followed up for as long as 12 years have provided an opportunity to observe the effects of melphalan treatment in this disease. The fibrohistiocytic and mucinous change of the skin in scleromyxedema and often the monoclonal protein can be controlled by low-dose chemotherapy. Although melphalan does not usually produce clinical toxic effects of importance, it is a myelotoxic drug and cytopenia is common; one patient died of acute myelomonocytic leukemia after ten years of successful therapy of the scleromyxedema, thus implying that long-term therapy may be dangerous by itself. These patients require close supervision. Leukocyte and platelet counts must be performed every three weeks, and the dosage of melphalan adjusted accordingly.
A review of inflammatory subcutaneous disease revealed that lymphoid follicles are observed frequently in lupus panniculitis. They are found rarely in other lymphocytic inflammatory diseases involving the panniculus, such as morphea, erythema nodosum, and erythema induratum. The lymphoid follicle may be an indication of immunoreactive and connective tissue disease, and cases should be evaluated with this in mind.
Acanthosis nigricans of the groin, axillae, and neck developed in a patient on long-term steroid therapy for dermatomyositis after eight years. The dermatosis resolved spontaneously ten years after the steroid use had been discontinued. Review of three personal cases and the literature suggests that acanthosis nigricans may have a hormonal basis.
Of 2,141 patients with scleroderma who were seen at the Mayo Clinic, Rochester, Minn, between 1959 and 1975, 78 had 87 internal malignancies. The relative frequency of the types of cancers was similar to that for the general population. The increased numbers of patients with breast and uterine carcinomas were consistent with the female preponderance in scleroderma. Contrary to previous reports, carcinoma of the lung was not the most frequent malignancy associated with scleroderma. The lymphoma-leukemia malignancies were second only to breast carcinoma in frequency, comprising about 17% of the malignancies. Both conditions developed within a three-year period in 68% (45/66) of our patients affected with systemic scleroderma, and this subgroup comprised a high mortality group. This study is not an epidemiologic one in the strictest sense, but it is a review of the experience of the Mayo Clinic with patients who have had both scleroderma and an internal malignancy.
Calcification in cutaneous blood vessels is an uncommon finding in biopsies submitted for dermatopathological examination. Of 14 biopsy specimens showing the phenomenon that was studied by us, the greater number was from women who had a combination of severe diabetes, hypertension, and atherosclerosis. Unusual clinical syndromes as the bases for the vascular calcification were hyperthyroidism in three patients and arteritis in two patients. Three patients died as a consequence of massive cutaneous infarction and sepsis, probably stemming from cutaneous vascular calcification. Vascular calcification in biopsy of skin may result from metabolic, inflammatory, or degenerative diseases of blood vessels.
The histochemical and electron microscopic findings in seven cases of dermal duct tumor are described. All seven of the patients were elderly, and five were women. The tumors were small flesh-colored or red papules located most often on the head and neck region. Intense phosphorylase and moderate respiratory enzymes characterized the histochemical reaction of the tumors. The following three types of tumor cells were recognized ultrastructurally: clear, dark, and luminal. The tumor was principally composed of clear cell proliferation around a regular ductal lumen. In addition, melanocytes, Langerhans' cells, and lymphocytes were occasionally seen within the tumor masses. An intact basal lamina and few hemidesmosomes with no detectable actin-like microfilaments were the features of the tumor-stroma interface. Our study results showed that the dermal duct tumor is a benign tumor with eccrine dermal ductal differentiation.
Sézary cells in the circulation were counted daily in 14 hospitalized dermatology patients: six with contact dermatitis, five with atopic dermatitis, and three with exfoliative psoriasis. All had circulating Sézary cells, but absolute counts of Sézary cells of more than 1000/mm3 appeared to correlate with a severe refractory course in two patients. It is probable that low, intermittent levels of circulating Sézary cells accompany many inflammatory dermatoses, and it is reasonable that monitoring this cell population may aid in anticipating more consequential skin disease.
Inflammation in inflammatory seborrheic keratoses was found to be composed of mononuclear cells, particularly lymphocytes. Accumulations of mononuclear cells in the papillary and subpapillary dermis frequently showed no tendency to infiltrate the epithelium. However, dramatic interaction of the seborrheic keratosis and the inflammatory process also was common. The lymphocytic exocytosis into the epithelium followed two basic patterns: eczematous, characterized by intercellular and intracellular edema, microvesicle formation, and epithelial necrosis; and lichenoid, in which a bandlike infiltrate attacked the dermoepidermal junction and hyaline bodies occurred in the infiltrated epithelium and in the dermis. Epithelial changes consisted of necrosis of epithelial cells and related parakeratosis; squamous eddies were occasionally found. Inflammatory cell infiltration in seborrheic keratoses possibly represents a process other than irritation, and we propose that mononuclear cell infiltration in seborrheic keratoses may be related to an involutionary process.
Acute myelomonocytic leukemia, a type of leukemic conversion that may be a manifestation of mycosis fungoides, developed in two patients with fairly typical courses of mycosis fungoides. The cases of five other patients with myelogenous leukemia as the terminal event of mycosis fungoides have been reported in the literature. A relationship to therapy could not be established, although all patients had had irradiation or chemotherapy or both.
A review of our experience with cicatricial pemphigoid revealed three patients with cicatrical pemphigoid and rheumatoid arthritis and one patient with ankylosing spondylitis who had a high titer of rheumatoid factor. A comparison of these four patients with patients who had bullous pemphigoid and rheumatoid arthritis shows similarities between cicatricial pemphigoid and bullous pemphigoid in relation to the development of rheumatoid arthritis.
Two patients with long-standing discoid lupus erythematosus developed tender, discrete breast nodules that, on the mammogram, were interpreted as carcinomas. The histologic features, direct immunofluorescence findings, and laboratory data confirmed the diagnosis of lupus mastitis. Antimalarial therapy resulted in the complete resolution of the lesions.
The cutaneous, necrotizing, palisading granuloma (Churg-Strauss granuloma) was observed on histopathologic study of skin specimens from seven patients. Two patients had Wegener's granulomatosis and one patient each had allergic granulomatosis, limited Wegener's granulomatosis, bacterial endocarditis, systemic lupus erythematosus, and rheumatoid arthritis. The microscopic picture consists of extravascular, palisading, dermal granuloma. The center of the granuloma consists of basophilic fibrillar necrosis in which linear bands of destroyed tissue are interspersed with masses of polymorphonuclear leukocytes and leukocytoclastic debris. This necrotic leukocytic mass is surrounded by histiocytes and some lymphocytes. The clinical lesions are symmetric, erythematous papules or nodules on the extremities. The histopathologic picture of the Churg-Strauss granuloma is unique and, as demonstrated in these cases, indicates the presence of systemic vasculitis.
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The conditions of three patients who had generalized granuloma annulare responded favorably to treatment with alkylating agents. This mode of therapy in two cases was chosen on the basis of recent evidence that implicates delayed hypersensitivity mechanisms as forming part of the pathogenesis of granuloma annulare. Two patients had rapid clearing of their lesions. The response in one of these patients was observed during therapy for a concomitant plasma cell dyscrasia. The third patient had an unusual form of granuloma annulare that had evolved during a period of 28 years, with hand deformities secondary to fascial and tendon involvement and the production of contractures and lymphedema, which responded partially to therapy. The results lend further support to data implicating cellular immunity to granuloma annulare.