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Biomedical subjects

R K Winkelmann

Publications and source records attributed to R K Winkelmann.

At least 217 records · Page 12Linked to original sources

Cutaneous extravascular necrotizing granuloma and lymphocytic lymphoma.

A patient had clinical findings of asthma, peripheral eosinophilia, multisystem abnormalities, and cutaneous extravascular granulomas, consistent with a diagnosis of allergic granulomatosis. In addition, a well-differentiated lymphocytic lymphoma was diagnosed on the basis of bone marrow findings, and a monoclonal gammopathy was demonstrated by serum immunoelectrophoresis. Clinically, the skin lesions appeared as erythematous papules or nodules on the extremities, and some of them showed central ulceration. Histologically, there were dermal extravascular necrotizing granulomas embedded in a granulomatous and inflammatory cell infiltrate. These lesions bore a striking resemblance to those described by Churg and Strauss in patients with allergic granulomatosis, although tissue eosinophilia was significantly less prominent.

Aged↗

[Normolipemic xanthoma planum and its associated syndromes].

The clinical feature of xanthoma is related to syndromes with quite different dignity. The clinical picture of diffuse plane normolipemic xanthoma represents a benign skin disease while the form of so-called plane xanthoma is frequently associated with lymphoproliferative processes (paraproteinemia, myeloma, lymphoma, leukemia). In this paper these principal points are stressed on the base of own cases and by survey of literature.

Diagnosis, Differential↗

Seborrheic keratoses: appearance in course of exfoliative erythroderma and regression associated with histologic mononuclear cell inflammation.

We observed a patient in whom large numbers of seborrheic keratoses developed in the course of a generalized dermatitis that progressed into an exfoliative erythroderma. A renal mass was finally discovered and was surgically excised. On microscopic examination, the kidney tumor proved to be a benign fibrous-walled, multilocular cyst. After removal of the renal cyst, the erythroderma and exfoliation subsided. A few months later, the seborrheic keratoses were noted to have become smaller, flatter, and fewer in number. Microscopic examination of five such involuting lesions showed, in each instance, mononuclear cell infiltration of the seborrheic keratoses. Our findings confirm earlier reports that seborrheic keratosis-like lesions developing during the course of widespread inflammatory cutaneous disorders may regress after control of the dermatosis and suggest that mononuclear cell inflammation may be the mechanism by which regression of these lesions is accomplished.

Aged↗

Basal cell tumor with eccrine differentiation (eccrine epithelioma).

In 1969, Freeman and Winkelmann reported two cases of basal cell tumors with features of eccrine differentiation. On the basis of subsequent personal cases and isolated literature reports, we wish to emphasize the characteristics of this tumor. It is an infiltrating basaloid growth, usually on the scalp or head, that frequently recurs, apparently because of inadequate excision. Basaloid, alveolar, and cystic epithelial masses, which constitute the tumor, may be seen individually or together. Areas that appear to be morpheaform epithelioma or similar to syringoma can be observed. Enzyme studies reveal phosphorylase and focal lysosomal enzymes such as acid phosphatase. Acid mucopolysaccharides are present in foci. Electron microscopy shows epithelial masses with eccrine duct features. Eccrine epithelioma is a rare tumor that may be mistaken for syringoma, basal cell carcinoma, adenocystic carcinoma, sweat gland carcinoma, or metastatic tumor. Its differentiation toward eccrine structure and function predicts the chronic, recurrent, locally infiltrative clinical course observed in patients to date.

Acid Phosphatase↗

Hematologic malignancies occurring in patients with urticaria pigmentosa.

Six patients had hematologic malignancies and coincident urticaria pigmentosa, five with the disseminated maculopapular form and one with the plaque form. Two patients had the juvenile-onset variety; the remainder had the adult eruptive variety. None of the patients complained of symptoms that could be attributed to liberation of histamine. In the two patients with juvenile-onset urticaria pigmentosa, the hematologic malignancies developed at the age of 17 years; one had Hodgkin's disease, and the other had acute myelomonocytic leukemia. In three patients with adult eruptive urticaria pigmentosa, the cutaneous lesions developed within 12 months of the diagnoses of lymphocytic lymphoma (two patients) and evolving myelomonocytic leukemia (one patient). In the remaining patient, cutaneous lesions developed many years before chronic lymphocytic leukemia was diagnosed. None of the patients had systemic mastocytosis. Skin biopsy specimens from all six patients showed an increase in dermal and perivascular round cells, and mast cells were seen in specimens from five of the six patients. In patients who received cytotoxic drugs for the hematologic malignancy, there was no change in the urticaria pigmentosa.

Adolescent↗

Tilorone hydrochloride in the treatment of T cell lymphoproliferative cutaneous disease.

Tilorone hydrochloride was used to treat eleven patients with T cell cutaneous disease ranging from pre-Sézary syndrome to tumor-stage mycosis fungoides. Cutaneous histologic study, Sézary counts, delayed skin tests, patch tests, and quantitative T cell counts were monitored. The study revealed that the response to tilorone depends in part on the type and stage of disease, as well as the characteristics and responsiveness of the individual lymphocyte population. Patients with pre-Sézary syndrome are most likely to benefit from tilorone. The effect of tilorone on the T cell population is manifested by changes in responses to patch and skin tests, as well as histologic improvement. Intact immune responses and elevated levels of IgE may be important prognostic clinical parameters in these patients. Tilorone is ineffective in patients with mycosis fungoides and advanced Sézary syndrome. Keratopathy can be a limiting but reversible complication of therapy. The drug may provide an effective therapeutic step in the treatment of early T cell cutaneous disease or an adjunctive therapy to leukapheresis and chemotherapy.

Adult↗

The clinical and histopathologic spectrums of urticarial vasculitis: study of forty cases.

Urticarial skin lesions may occur in patients as a manifestation of necrotizing vasculitis. We describe a series of forty patients with idiopathic chronic urticaria and histologic features of necrotizing vasculitis. On the basis of clinical evaluation, we have classified urticarial vasculitis into two major groups: (1) hypocomplementemic (sixteen patients, ten of whom had evidence of renal disease) and (2) normocomplementemic (twelve patients with systemic disease and twelve with only cutaneous involvement). Most patients with hypocomplementemia presented with arthritis, and some had abdominal pain or airway compromise. Although patients with normocomplementemia and systemic disease had a less severe clinical course, four exhibited renal disease that was characterized by microhematuria and proteinuria. Direct immunofluorescence microscopy of the skin aids in assessing renal involvement in some cases of hypocomplementemic urticarial vasculitis, particularly when IgG and IgM are deposited at the basement membrane. There seems to be a spectrum of disease in urticarial vasculitis, ranging from benign cutaneous lesions to systemic disease.

Adolescent↗

Carpal tunnel syndrome in cutaneous connective tissue disease: generalized morphea, lichen sclerosus, fasciitis, discoid lupus erythematosus, and lupus panniculitis.

Carpal tunnel syndrome developed concurrently with cutaneous connective tissue disease in five patients. The skin lesions varied from morphea, lichen sclerosus, fasciitis, and discoid lupus erythematosus to lupus panniculitis. Variable and transitory serologic and direct immunofluorescent findings were noted. In two cases, surgical specimens from carpal tunnel operations had lymphoid nodules. Treatment of the cutaneous connective tissue syndrome (antimalarials, four cases; corticosteroids, two cases) brought healing of the carpal tunnel syndrome as well as improvement of the skin lesions.

Adult↗

Lipid synthesis in cutaneous xanthoma.

In vitro lipogenesis was studied on the xanthoma tissue from 6 patients with normal plasma lipids and 4 patients with hyperlipidemia. Xanthoma tissue was incubated at 37 degrees C for 6 hr in Krebs-Ringer phosphate buffer containing sodium [14C]acetate. The radioactivity of each lipid class was determined after extraction and separation of lipids. The incorporation of acetate into all major lipid groups was much greater in xanthoma tissue than in control normal-appearing skin. There was no difference in the incorporation pattern of 14C between xanthomas of patients with normal plasma lipids and those of hyperlipidemic patients. The data exemplify considerable in situ lipid synthesis of xanthoma tissue. Although the lipids in xanthomas of hyperlipidemic persons may be derived from plasma, the plasma origin of xanthoma lipids in normolipidemic persons remains to be confirmed, and the contribution of local lipogenesis cannot be ignored. The lipids in cutaneous xanthomas are most likely derived from a multiple input system.

Acetates↗

Cutaneous neuropathology in multiple endocrine neoplasia, type 2b.

Increase in masses of mixed (myelinated and nonmyelinated) nerve are observed in the dermis of clinically normal skin of patients with multiple endocrine neoplasia, type 2b (MEN 2b). Preliminary histochemical studies revealed nucleoside triphosphatase and nonspecific acid esterase. Electron microscopy showed axonal organelles, mild degeneration of axons, and numerous axons unassociated with Schwann cells. The normal-appearing skin in MEN 2b seems to contain abnormal nerve tissue development equally with the mucosal and the gastrointestinal tract.

Axons↗

Epidermoid hidradenoma. A clinicopathologic study.

Clinical and histopathologic data in 8 cases of epidermoid hidradenoma are presented. Most of the tumors were in the head and neck, and most of the patients were middle-aged and older adults. Generally, the lesions were asymptomatic nodules that sometimes showed ulceration or rapid growth. Despite having some histologic atypia or focal mitotic activity, the tumors were found to be benign on long-term evaluation. This epidermoid variant should not be otherwise differentiated from the benign group of solid-cystic hydradenomas, because cytologic variability did not predict a significant change in prognosis.

Adenoma, Sweat Gland↗

The histochemistry of the Merkel cell neuroepithelial endings in cat and rabbit skin and oral mucosa.

The tactile hair disks in the oronasal area of the rabbit registered positive for specific and nonspecific cholinesterase. In the cat, the tactile hair disks were positive for alkaline phosphatase in the same region. The hederiform Merkel-Ranvier ending in the epidermis of the palate was positive for cholinesterase in the rabbit and positive for alkaline phosphatase in the cat. In the rabbit, 4-methoxyleucine aminopeptidase activity was also demonstrated in the focal palate epithelium associated with nerves of the Merkel-Ranvier ending. These histochemical reactions of the Merkel neuroepithelial complexes are associated with the nerve ending and may be a specific indicator of the species' innervation pattern.

Alkaline Phosphatase↗

Dermatomyositis in childhood.

Childhood dermatomyositis may be a serious and even lethal disease of childhood. Two types are recognized: the rare Banker type, based on vasculopathy and infarction commonly proceeding to death, and the more common Brunsting type, which is a steroid-responsive inflammatory myopathy comparable to the usual adult form. The diagnosis is suggested by the finding of skin changes (present in most patients) and by the results of clinical, enzymatic and electromyographic evaluation of muscle function. A muscle biopsy specimen should be taken in all cases to observe muscle and vessel changes and to rule out unusual myopathy. Direct immunofluorescence of skin and muscle may be helpful. Steroid therapy has reduced inflammation, morbidity and mortality, but remissions may occur without treatment. Methotrexate may be used when there is no response to steroids. Nutrition and physical therapy must be applied properly to be effective. Corticosteroid therapy of the disease has improved the prognosis for musculoskeletal function and for life.

Adolescent↗

Verruciform xanthoma in an epithelial nevus.

Verruciform xanthoma is an unusual lesion that was initially reported in 1971. Clinically, it is a verrucous, vascular, epithelial plaque or papillomatous growth commonly found on oral or mucosal tissues. Histologically, it is characterized by a verruciform epithelium with abundant parakeratosis and by foam cells occupying the vascularized dermal papillae. This is the second extramucosal verruciform xanthoma reported. Our patient had several unique features. She is the youngest patient described to date, her lesion is the largest reported, and the lesion occurred in an epithelial nevus-type eruption of a phocomelic extremity. Light and electron microscopic studies disclosed abnormal keratinization, mast cell proliferation, and the previously described foam cells. We believe that the verruciform xanthoma in our patient represents part of a reaction to the epithelial nevus occurring in association with a developmental anomaly.

Abnormalities, Multiple↗

Plantar warts recently turned black. Clinical and histopathologic findings.

The inflammatory component associated with blackening and subsequent regression of plantar warts has been little appreciated in the literature. Two patients with plantar warts in whom one of the warts showed prominent, clinically evident inflammation were observed. Blackening and subsequent regression of all plantar warts then occurred. In one patient, microscopic examination of biopsy specimens of two lesions that were taken within 24 and 72 hours, respectively, after they had turned black demonstrated the following histologic findings: blood clots and hemorrhage in the stratum corneum, degeneration and necrosis of epidermal cells, eosinophilic cytoplasmic masses within degenerating epidermal cells, thrombosis of superficial and deeper dermal blood vessels, a mononuclear cell infiltrate in and around dermal blood vessels, and a mixed polymorphonuclear and lymphocytic infiltration in the areas of hemorrhage and degenerating epidermis. This constellation of histopathologic changes suggests that involution was in progress long before blackening of the warts occurred.

Administration, Topical↗