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Biomedical subjects

R K Winkelmann

Publications and source records attributed to R K Winkelmann.

At least 235 records · Page 13Linked to original sources
PubMed1982

The involuting lichenoid plaque.

Progressive involution and subsequent regression of a plaque that showed the characteristic histologic findings of solitary lichen planus-like keratosis were observed in five patients. In each lesion, involution took place during a four- to eight-week period. In four patients, the lesion arose in a preexisting pigmented macule. Evolution into a plaque-like lesion coincided with the development of the inflammatory process. Origin from a preexisting lesion suggests that the lichenoid plaque is not an independent entity but a stage in the involution of the preexisting pigmented macule. The lichenoid inflammatory process resembles a delayed hypersensitivity reaction and seems to represent a cell-mediated rejection reaction.

Female↗
PubMed1982

Cutaneous and subcutaneous inflammatory sclerosis syndromes.

Systemic scleroderma and localized scleroderma (morphea) show comparable changes on skin biopsy specimens, and a distinction has often been made on the basis of Raynaud's phenomenon, organ involvement, and laboratory abnormalities characteristically seen in systemic scleroderma. Critical evaluation not only of patients with localized scleroderma but also of those with eosinophilic fasciitis, morphea profunda, and acral pansclerotic morphea has disclosed Raynaud's phenomenon, organ involvement, and laboratory abnormalities typical of systemic scleroderma in a small percentage of patients. Histologically, all five conditions show similar inflammation and sclerosis of the skin, the primary difference being the depth at which these changes occur. These conditions may possibly be related, and the clinical and laboratory differences observed may result from variations in depth, nature, and intensity of the cutaneous and subcutaneous inflammatory sclerosis.

Child↗
PubMed1982

High-dose vitamin A therapy for Darier's disease.

Three patients with Darier's disease were treated with 1 X 10(6) IU of orally administered vitamin A daily for 14 days. In all patients, 50% to 80% improvement in the skin lesions was noted. Desquamation was minimal, and side effects consisted of drowsiness, mild frontal headache, dry lips and dry nose. During therapy, all patients had a transient, mild increase in the serum triglyceride level, and two patients had a minimal increase in the serum cholesterol concentration.

Absorption↗
PubMed1982

Response of nodular non-X histiocytosis to vinblastine.

Progressive cutaneous papulonodular non-X histiocytosis of the face and proximal parts of the extremities developed in a 28-year-old woman. Histologic study showed masses of benign histiocytes with foci of lymphocytes. Occasional cells contained diastase-resistant cytoplasmic polysaccharide that stained positive with PAS stain. No iron, fat, or Touton or other giant cells were present. Ultrastructural study disclosed laminar bodies but no Langerhans' cells or Langerhans' granules. The process responded to low-dose intravenous vinblastine sulfate treatment.

Adult↗
PubMed1981

Cutaneous immunofluorescence in primary biliary cirrhosis.

Biopsy specimens of normal skin of 11 patients with primary biliary cirrhosis were studied by immunofluorescence for immunoglobulin and complement deposition. Of the 11 patients, seven had positive immunofluorescent staining, primarily in a granular pattern either at the basement membrane zone or around the blood vessels or in both locations. The principal immunoglobulin noted was IgM, which, in six of the seven patients, was seen at the basement membrane zone or around the vessels (or both). Deposition with C3, fibrin, IgA, and IgG was seen less frequently. In this series of patients, cutaneous lesions included discoid lupus erythematosus, lichen planus, vitiligo, chronic bullous lesions of the legs, and scleroderma. Non-organ-specific immunologic markers in this group included elevated levels of IgG, IgM, and IgA, antinuclear antibodies, anti-nDNA, rheumatoid factor, antimitochondrial antibodies, smooth-muscle antibodies, and circulating immune complexes. We wish to add cutaneous immunofluorescence as an additional immunologic marker for primary biliary cirrhosis.

Adult↗
PubMed1981

Cytophagic histiocytic panniculitis with fever, cytopenia, liver failure, and terminal hemorrhagic diathesis.

We have seen five adult patients with a clinical picture of recurrent histiocytic, cytophagic panniculitis, cytopenia, abnormal liver function tests, and a terminal, febrile bleeding diathesis. Originally thought to have Weber-Christian disease, these patients, we believe, represent a unique syndrome: lobular, histiocytic, cytophagic panniculitis. Erythrophagocytosis and cytophagocytosis are readily observed, but the cells do not show malignant features. Histiocytosis can be found at times in the bone marrow, lymph nodes, liver, spleen, and serosal tissues, as well as in the skin and subcutaneous tissues. The terminal hemorrhage in these patients is characterized by features of pancytopenia, liver failure, and intravascular coagulation. This disease may be separated from malignant histiocytosis by the chronic course, the primary involvement of the adipose tissue, and the benign histiocytes in the infiltrate. It has some similarities to other regional histiocytoses such as sinus histiocytosis, intestinal histiocytosis, and splenic histiocytosis.

Adult↗
PubMed1981

Lymphoma-associated papulosis: lymphomatoid papulosis associated with lymphoma.

Lymphomatoid papulosis is a condition characterized by the continuing appearance of self-healing inflammatory papulonodules which histologically show atypical cells simulating a malignant process. We describe two patients who had features typical of lymphomatoid papulosis in whom a lymphoreticular malignancy developed. The first patient developed Hodgkin's disease 30 years after the onset of lesions that resembled lymphomatoid papulosis, and the second patient developed a lymphocytic lymphoma 11 months after the appearance of the self-healing papules. While most patients with lymphomatoid papulosis have a benign protracted course, "lymphoma-associated papulosis" represents the malignant end of this spectrum, in which lymphomatoid papulosis is associated with a lymphoma. All patients with lymphomatoid papulosis require close, long-term follow-up.

Biopsy↗
PubMed1981

Leukapheresis in the treatment of Sézary syndrome.

Five patients received leukapheresis therapy for Sézary syndrome. All patients had erythroderma, lymphocytic band infiltration of the skin, and a normal white blood cell count with an absolute Sézary cell count of more than 1,000/mm3. Improvement in 3 to 6 months occurred, usually with the addition of a combined regimen of prednisone and low-dose chlorambucil therapy. One patient had sustained improvement with repeated leukapheresis for more than 2 years. The two patients who showed a major response were the youngest patients, who had normal karyotypes, normal delayed-hypersensitivity skin tests, and a quantitative response of the Sézary cells and the T and B cells to leukapheresis. These preliminary results suggest that there may be selected patients for whom leukapheresis is useful therapy and that it is possible to perform the procedure safely in patients with normal white blood cell counts.

Adult↗
PubMed1981

Cutaneous clinicopathologic correlation of allergic granulomatosis.

Allergic granulomatosis is a distinct clinical syndrome occurring in adults with asthma, eosinophilia, and multisystem vasculitis. Atopy and drug sensitivity are other important features. The skin reactions are most commonly nodular and inflammatory lesions. A unique feature is the deep papulonodules, which may occur singly on the scalp or symmetrically on the extremities. Cutaneous findings may range from purpura to urticaria and ulceration. The most common histologic finding is the extravascular granuloma; however, necrotizing vasculitis of small vessels is seen, as well as periarteritis nodosa involving larger vessels of the skin. This varied histologic and clinical spectrum seen in patients with systemic allergic granulomatosis is a reflection of a unique host response to multiple antigens. The cutaneous findings imply that allergic granulomatosis probably represents a unique host response to the same causative and pathogenetic factors as are usually found in periarteritis nodosa and necrotizing vasculitis.

Adult↗
PubMed1981

The histopathology of lupus erythematosus panniculitis.

Lupus erythematosus panniculitis is a clinical variant of lupus erythematosus in which the main pathologic process involves the deep corium and subcutaneous tissue. We reviewed twenty-nine cases of lupus panniculitis, as well as the cases previously reported in the literature. The histopathologic changes in lupus panniculitis are characterized by a lymphocytic panniculitis, hyaline degeneration of the fat, hyaline papillary bodies, and lymphoid nodular structures in the lower dermis and subcutaneous tissue. Direct immunofluorescence can be important in supplementing the histopathologic study of lupus panniculitis. Lesions of discoid lupus erythematosus are seen in 21% of cases. When this disorder exists in the absence of other typical cutaneous or systemic lesions, the diagnosis of lupus erythematosus has been questioned. We believe that the histopathologic findings of this entity are alone sufficient for a diagnosis of lupus panniculitis, even in the absence of cutaneous or systemic lesions.

Adult↗
PubMed1981

Lymphomatoid contact dermatitis.

Five elderly patients with recurrent and progressive erythematous patchy dermatitis had temporary flares of generalized erythema and associated positive patch tests. The histopathologic findings were of a distinct pattern of lymphocytic dermal reticulosis and chronic epithelial response. We believe this represents a potential pre-Sézary state that can be recognized by the course and the histologic pattern.

Aged↗
PubMed1981

Direct immunofluorescent findings in scleroderma syndromes.

Cutaneous direct immunofluorescent findings were examined in 78 patients who had either vascular scleroderma (group 1, 52 patients) or scleroderma with features of myositis or lupus erythematosus (group 2, 26 patients). Group 2 had higher antinuclear antibody levels, erythrocyte sedimentation rates, serum IgG concentrations, frequency of positive LE clot test, and rheumatoid factor activity. Ninety-two percent of group 1 (48 patients) had negative direct immunofluorescent findings, whereas 77% of group 2 (20 patients) had positive findings at the basement membrane or in the blood vessels (or both). The 6 patients in group 2 who had negative immunofluorescent findings were all on systemic steroid therapy. Of the 17 patients in group 2 who had tests for antibody to extractable nuclear antigen, only 3 had high-titer antibody to ribonucleoprotein--a pattern characteristic of mixed connective tissue disease. Direct cutaneous immunofluorescence is proposed as a means of identifying those patients with scleroderma who may be steroid-responsive.

Adolescent↗
PubMed1981

Cutaneous syndromes of non-X histiocytosis. A review of the macrophage-histiocyte diseases of the skin.

The bone marrow and blood monocytes are precursor cells to the macrophage-histiocyte, the Langerhans cell, the indeterminate cell, the interdigitating cell, and the dendritic cell in the thymus, lymph node, and skin. While there are many histologic and functional similarities in these cell types, some differences do occur. A notable difference is the minimal phagocytosis by the Langerhans cell and the histiocytosis X cell. Non-X histiocytosis is represented by a series of cutaneous disease patterns characterized by localized, nonaggressive, histioxanthomatous skin and mucous membrane lesions, with a tendency to self-heal, Eruptive histiocytoma, juvenile xanthogranuloma, reticulohistiocytoma cutis, papular xanthoma, necrobiotic xanthogranuloma with paraproteinemia, and xanthoma disseminatum are cutaneous patterns of non-X histiocytosis that suggest the possibility of at least two patterns of peripheral macrophage differentiation and pathologic change.

Adult↗
PubMed1981

Treatment of vasculitis.

Several clinical and pathologic types of vasculitis have been identified, but basically there are two categories: large and small vessel vasculitis; and type III immune complex and type IV delayed hypersensitivity lymphocytic vasculitis. Rapid advances in therapy of several forms of vasculitis indicate that more use should be made of active agents across the spectrum of vasculitis syndromes. This review should make this possible.

Humans↗
PubMed1981

Staging laparotomy in cutaneous T-cell disease.

Staging laparotomy performed in 13 patients with various forms of cutaneous T-cell disease (mycosis fungoides, Sézary syndrome, cutaneous lymphoma, atrophic parapsoriasis, and alopecia mucinosa) showed evidence of visceral or lymph node involvement in three (23%) patients; in two of these patients, noninvasive investigations and staging procedures did not disclose any abnormalities. All three patients were alive after a mean follow-up period of 3 1/2 years. In the ten patients with normal findings at laparotomy, the presence of intra-abdominal lymphoma was suggested in six by noninvasive staging procedures (lymphangiogram, spleen or liver-spleen scan, and computerized tomographic scan of the abdomen). Five of these ten patients died; four of the five patients died after relentless progression of the disease and visceral involvement. Staging laparotomy may have a role in the management of cutaneous T-cell disease because noninvasive investigations may be poor indicators of the presence or absence of intra-abdominal disease. The course of these patients, even those in whom laparotomies did not show any abnormalities, emphasizes the progressive potential of cutaneous T-cell disease.

Adult↗
PubMed1981

Surgical treatment of familial benign chronic pemphigus.

Familial benign chronic pemphigus (Hailey and Hailey disease) is a rare, recalcitrant, often disabling genodermatosis that may not respond to conservative dermatologic therapy. We describe herein five patients with intertriginous familial benign chronic pemphigus who underwent excision and split-thickness skin grafting. All were men whose duration of disease ranged from one to 38 years. Follow-up evaluations ranging from ten months to nine years revealed no recurrence in graft sites in three patients, mild recurrence in one patient after eight years, and one death from pulmonary embolus in the postoperative period. The occurrence of familial benign chronic pemphigus around graft edges was a universal, but relatively minor, problem. Surgical excision provided definite relief from an otherwise disabling disease in four of our patients and a satisfactory improvement in life-style.

Adult↗