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Biomedical subjects

R K Winkelmann

Publications and source records attributed to R K Winkelmann.

At least 55 records · Page 3Linked to original sources
PubMed1991

Cutaneous manifestations in patients with essential thrombocythemia.

In a retrospective study of 268 patients with essential thrombocythemia, related cutaneous manifestations were found in 58 (22%). In 27 cases (10%) the related skin lesions were present at the time of the primary diagnosis of essential thrombocythemia. Hematomas, ecchymoses, petechiae, or purpura occurred in 24 (41%) of the 58 patients. Fifteen patients (26%) had erythromelalgia, in 11 of whom it was the initial complaint. Livedo reticularis, recurrent superficial thrombophlebitis, ischemic complications with gangrene, leg ulcers, or ulcers on the toes were other manifestations. Urticaria occurred in two patients, and Raynaud's phenomenon and necrotizing vasculitis were each observed in one patient. Platelet function may be abnormal, and this was found in 13 of 19 patients studied, 9 of whom had spontaneous platelet aggregation. Knowledge of the cutaneous manifestations in essential thrombocythemia may lead to its earlier detection. Prompt diagnosis of essential thrombocythemia is important because treatment may prevent severe hemorrhagic or thrombotic events.

Adult↗
PubMed1991

Histopathologic features of the L-tryptophan-related eosinophilia-myalgia (fasciitis) syndrome.

Study of 18 biopsy specimens in 11 patients with L-tryptophan-related eosinophiliamyalgia (fasciitis) syndrome showed hyaline sclerodermoid changes. Dermal scleroderma was found in eight of nine punch biopsy specimens and eight of nine excisional biopsy specimens. Fascial scleroderma was found in eight excisional biopsy specimens. One specimen obtained by excision had no fascia present. Eleven biopsy specimens showed edema of the dermis, and 13 showed dilated lymphatic structures; thus, the clinical picture of edematous sclerosis was confirmed. Mucinous fasciitis was present in five excisional biopsy specimens, in conjunction with a large number of macrophages in four. Dermal mucinosis was present in 11 biopsy specimens. Lymphocytic and macrophage inflammation was minimal in 14 biopsy specimens and pronounced in only 4. Plasma cells were present in eight cases. Eosinophils were present in substantial numbers in three biopsy specimens and only occasionally in four. Eosinophilic spongiosis was observed in one patient. Lymphocytic inflammation was noted around a single muscle spindle and around large nerve trunks in three patients. No relationship was established between these pathologic features and the duration or dose of tryptophan, prednisone treatment, or duration of symptoms. Pathologic features of the L-tryptophan syndrome consist of hyaline sclerodermoid collagen in the dermis, the septa, and the fascia. Edema, focal mucinosis, and macrophage inflammation may be features that identify this event.

Collagen↗
PubMed1991

The evolution of Hodgkin's disease and necrobiotic xanthogranuloma syndrome.

We describe a woman in whom hypogammaglobulinemia and severe granulomatous cutaneous lesions had developed during childhood; subsequently, Hodgkin's disease and necrobiotic xanthogranuloma were diagnosed. This case illustrates an apparent association with disease activity and raises the question of a direct relationship of necrobiotic xanthogranuloma with lymphoproliferative disease.

Adolescent↗
PubMed1991

Adult lipophagic atrophic panniculitis.

We report two patients with chronic, recurrent, nodular panniculitis lesions that later developed areas of lipoatrophy. Histologically, there was a consistent lobular lipophagic replacement of fat cells with lipophagic giant cells. The clinical appearance was that of tender, erythematous, superficial or subcutaneous, symmetrical nodules and plaques of 1-2 weeks' duration. The lesions could occur with episodes of fever. One patient had hepatomegaly and the other had an increased sedimentation rate and leucocytosis. The histology and the clinical pattern of the panniculitus syndrome resembled those of lipophagic lipoatrophy of childhood. This is a panniculitis of unknown cause in which the principal inflammatory cell response in the subcutaneous tissue is the macrophage.

Adipose Tissue↗
PubMed1991

Diffuse-plane normolipaemic xanthoma with aortic-valve xanthoma.

A patient with diffuse-plane normolipaemic xanthomatosis had mucous-membrane, conjunctival, and aortic-valve xanthomatosis. The presence of a monoclonal protein and hypocomplementaemia suggests that the xanthomatous lesions were probably not metabolic in origin but developed as secondary events in a histiocytosis of involved tissues. No heart-valve xanthomatization in like circumstances has previously been reported.

Aortic Valve↗
PubMed1991

Destructive granuloma annulare of the skin and underlying soft tissues--report of two cases.

Two cases of extensive cutaneous granuloma annulare (GA) associated with severe deformity and impairment of function in the involved extremity are described. In the first patient a mutilating, destructive arthritic condition and infiltrative oedema of upper extremities developed over the years along with florid disseminated GA of the tendons and overlying skin. In the second patient disseminated GA of one lower extremity resulted in diffuse induration and chronic lymphoedema. In both cases, histology showed palisading granulomas typical of GA at all levels of the dermis, subcutaneous tissues, fascia, and synovium. The unique clinical features of GA in both presented cases emphasize the local destructive potential and, as yet, poorly understood progression of GA to a soft-tissue disease.

Aged↗
PubMed1991

Lipophagic panniculitis in re-excision specimens.

Lipophagic panniculitis consists of a macrophage infiltrate in the subcutaneous tissue. The macrophages transform into foam cells within the panniculus; they replace lipocytes and may form giant cells. Although those pathologic features have been described as diagnostic of Weber-Christian disease, we report the occurrence of lipophagic panniculitis in re-excision specimens. Among 252 re-excision specimens from previously biopsied skin tumors, 5 cases in which masses of lipophages were infiltrating and replacing the subcutaneous tissue were found. The infiltrate was localized to the deep dermis and superficial subcutaneous tissue below and beside the initial biopsy site. In 3 cases, suture or hair was detected within the tissue, and granulation tissue with foreign body giant cells was observed along the dermal suture line. In 4 cases there was evidence of phlebitis within or close to areas of infiltration. None of these patients developed symptomatic panniculitis. Lipophagia can be a normal response of wound healing in some patients.

Foam Cells↗
PubMed1990

Septal granulomatous panniculitis: comparison of the pathology of erythema nodosum migrans (migratory panniculitis) and chronic erythema nodosum.

Fifty-eight cases of septal granulomatous panniculitis were reviewed; 14 cases were diagnosed as erythema nodosum migrans (migratory panniculitis) and 36 as chronic erythema nodosum on the basis of clinical and histopathologic features. Erythema nodosum migrans was characterized by markedly thickened and fibrotic septae, marked capillary proliferation (like granulation tissue), and massive granulomatous reaction (with giant cells) along the borders of the widened septa. Hemorrhage was rare, and phlebitis was not seen. Chronic erythema nodosum showed mild septal change, little fibrosis, and lymphohistiocytic perivascular inflammation with only focal granulomatous formation. Phlebitis and hemorrhage were common. The condition termed erythema nodosum migrans has many of the same clinical features as chronic erythema nodosum, and we think this term is preferable to migratory panniculitis. We believe that there are sufficient clinical and histopathologic features to justify considering erythema nodosum migrans as a unique clinicopathologic entity.

Adult↗
PubMed1990

Mycosis fungoides in children and adolescents.

The clinical and histologic findings in five young patients with mycosis fungoides are reviewed. The skin specimen from a 16-year-old boy had an infiltrate predominantly Leu-1+, Leu-4+, Leu-3a + 3b+, OKT6+, Leu-2a-, Leu-8-, and Leu-9-, and rearrangements were noted in T cell receptor gene (beta chain [constant region probe] and gamma chain [J region probe]); no rearrangements were found in a histologically normal lymph node or peripheral blood. The skin specimen from an 11-year-old girl contained predominantly Leu-4+ and Leu-9+ lymphoid cells. No T cell receptor gene rearrangements were found in the skin or in an involved lymph node. The variations in clinical, histologic, and immunopathologic features suggest that mycosis fungoides in young patients represents a heterogeneous subgroup of cutaneous T cell lymphoma.

Adolescent↗
PubMed1990

The heterogeneity of Jessner's lymphocytic infiltration of the skin. Immunohistochemical studies suggesting one form of perivascular lymphocytoma.

Seventeen patients (six women and 11 men) with Jessner's lymphocytic infiltration of the skin were studied. Nineteen biopsy specimens were analyzed with the following monoclonal antibodies: UCHL1 (T cells), 4KB5, L26 (B cells), LN1 (germinal center B cells), and BerH2 (Ki-1+ cells). Routine direct immunofluorescence was performed on 15 specimens. In 10 of 19 specimens both B and T cells were demonstrated; the B cells were in close proximity to small blood vessels. In 50% of these cases the B cell component expressed LN1 positivity. We use the term perivascular lymphocytoma to describe this pattern. Nine specimens showed T cells only. No Ki-1+ cells were detected. Routine immunofluorescence studies were performed on 15 specimens. In six of seven specimens in which there was a mixed cell population of B and T cells, results were completely negative. In contrast, six of the remaining eight specimens with a predominantly T cell population revealed weak, patchy, granular, positive labeling for immunocomponents, particularly IgM and C3 at the basement membrane. These findings suggest that Jessner's lymphocytic infiltration is a heterogeneous disorder with at least two separate immunophenotypes. The finding of perivascular follicular center differentiation in more than half the specimens suggests a possible relationship in these cases to other benign lymphoid hyperplasias (pseudolymphomas).

Adult↗
PubMed1990

Benign lymphangioendothelioma.

We have studied eight cases of an acquired lymphatic endothelial lesion for which we propose the name "benign lymphangioendothelioma." The lesions developed as solitary, slowly extending, erythematous macules and plaques, usually occurring on the extremities or the shoulders in adolescents or adults. The characteristic histopathologic feature is permeation of the dermal collagen by flattened, endothelium-lined channels and spaces. Hemorrhage, iron deposition, and inflammation were not part of the lesion. Ulex europaeus agglutinin I labeled the lesional endothelial cells consistently, but factor VIII-related antigen labeling was negative. This histologic pattern and the special studies suggested a lymphatic lesion. Surgical excision, performed in six patients, was not followed by recurrence.

Adolescent↗
PubMed1990

Scleroderma and L-tryptophan: a possible explanation of the eosinophilia-myalgia syndrome.

Scleroderma developed in six women who were taking L-tryptophan. Fasciitis and morphea were most common, but one patient had pleural effusion, hypertension, and signs of cardiac and kidney failure. In five patients the biopsy findings were characteristic of scleroderma; the sixth patient had Crohn's disease and developed fasciitis; her biopsy specimen showed inflammatory arteritis. All patients' conditions improved after cessation of their L-tryptophan intake, initiation of corticosteroid therapy, or both. These findings confirm previous data that show altered tryptophan-kynurenine metabolism in some patients with scleroderma and fasciitis, particularly with tryptophan loading.

Adult↗
PubMed1990

Cutaneous malignant melanoma in Rochester, Minnesota: trends in incidence and survivorship, 1950 through 1985.

In Rochester, Minnesota, 107 incidence cases of cutaneous malignant melanoma (in 46 male and 61 female patients) were diagnosed during the years 1950 through 1985. Overall crude incidence rates were 6.0 and 6.6 per 100,000 males and females, respectively. Evaluation of trends in 9-year periods showed that the rates increased from 3.2 to 8.9 for males (P = 0.015) and from 4.4 to 11.7 for females (P less than 0.001). Age-specific rates suggested that the highest incidence occurs in the age-groups 50 to 59 years and 70 years or older for males and 40 to 49 years and 70 years or older for females. Lesions were most common in the head and neck area among males (P = 0.044) and on the lower extremities among females (P = 0.018). The most frequent histologic type was superficial spreading melanoma (61%). Five-year survival was diminished overall for patients with cutaneous malignant melanoma--0.72 in comparison with 0.88 expected for the general population. Statistically significant risk factors for survival were depth of invasion of the lesion (Clark level), thickness of the lesion, histologic type, and age of the patient.

Adult↗
PubMed1990

Intravesical condyloma acuminatum with progression to verrucous carcinoma.

Condyloma acuminatum is a common form of venereal disease. Most patients with condylomata acuminata suffer from only the local cosmetic and irritative effects of the lesions. Few patients have progression to aggressive, regionally distributed lesions that can be life-threatening. We describe a forty-three-year-old white woman who had a seventeen-year history of scleroderma with extravesical and intravesical condylomata acuminata. During two years of conservative management with transurethral excision, electrocoagulation, and intravesical chemotherapy, the disease progressed to involve the entire bladder and resulted in obstructive renal insufficiency that required anterior exenteration and urinary diversion. The natural history of the disease is described, and the relevant literature is reviewed.

Adult↗
PubMed1990

Reticulohistiocytoma cutis--review of 15 cases and an association with systemic vasculitis in two cases.

We reviewed 15 cases of reticulohistiocytoma cutis (RHC). Three cases were of solitary lesions. Four cases were associated with a destructive arthritis but no other systemic features. One additional patient had a destructive arthritis and lesions of reticulohistiocytoma in synovium, larynx, and mucosa. Three patients had associated xanthelasma. Two cases were associated with internal malignancy (metastatic malignant melanoma, adenocarcinoma of the bowel). Two cases were sequentially associated with systemic vasculitis (Wegener's granulomatosis, periarteritis nodosa). This occurrence of systemic vasculitis in RHC patients is unique. RHC may have variable cutaneous and systemic manifestations and associations with malignancy and immunoinflammatory diseases, including vasculitis.

Adult↗
PubMed1990

Clinicopathologic study of cutaneous plasmacytoma.

Eight patients with skin tumor lesions composed of dense, predominantly plasma cell infiltrates were studied. Primary cutaneous plasmacytoma can be reactive (polyclonal) or neoplastic (monoclonal). In four of the patients skin lesions were associated with multiple myeloma. Specific skin lesions usually consisted of reddish or purple nodules located on the trunk. In one case the cutaneous lesions developed at the site of previous herpes zoster. Histologically, the cutaneous plasmacytic infiltrate was mainly diffuse and monomorphous. Most of the plasma cells were mature, but in some cases immature immunoblasts and mitoses were observed. Serum immunoelectrophoresis findings correlated with the monoclonality or polyclonality of the plasmacytoma. Presence or absence of systemic involvement cannot be predicted from the appearance of clinical lesions or from maturity of plasma cell infiltration in the skin.

Aged↗
PubMed1990

Acid mucopolysaccharide staining in scleredema.

We studied 26 formalin-fixed biopsy specimens taken from 22 patients with scleredema and found positive staining in 67% of the tissues when both colloidal iron and alcian blue methods were used. Single stains were positive in only half the tissues. Positive findings were noted in both patients with diabetes (11 patients) and in those without it (11 patients). Our experience indicates that multiple biopsies may be necessary to demonstrate mucin in the dermis. Specimens obtained by incisional biopsy are preferred.

Glycosaminoglycans↗