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Biomedical subjects

W F Bergfeld

Publications and source records attributed to W F Bergfeld.

At least 55 records · Page 3Linked to original sources

Generalized non-Langerhans cell histiocytosis: four cases illustrate a spectrum of disease.

BACKGROUND: The proliferation of non-Langerhans cell histiocytes is a poorly understood process of unknown cause. Variation in the clinical features and/or histopathology of histiocytic proliferation has led to subclassification of the general category of non-Langerhans cell histiocytes. Although the current classification may provide some useful generalizations in regard to the anticipated clinical course, wide variability in presentation and outcome make this classification less than optimal when dealing with individual patients. The objectives of the study were to present four cases of generalized non-Langerhans cell histiocytosis. MATERIALS AND METHODS: Medical records and slides of four patients diagnosed with non-Langerhans cell histiocytosis at the Cleveland Clinic are reviewed. RESULTS: The patients exhibit features of more than one subtype of non-Langerhans cell histiocytosis. CONCLUSION: The overlap among the clinical and histologic features of the generalized cutaneous non-Langerhans cell histiocytic disorders suggests that they represent one disease entity with a wide spectrum of presentations rather than many distinct disorders.

Adolescent↗

Lichen planus associated with neoplasia: a cell-mediated immune response to tumor antigens?

BACKGROUND: Individual case reports have suggested an occasional association of lichen planus with internal malignancy. OBJECTIVE: Our purpose was to describe five patients with a neoplastic disease in whom lichen planus developed. METHODS: Serologic and immunopathologic studies were conducted. RESULTS: No evidence of autoantibody production characteristic of paraneoplastic pemphigus was found, and antibodies reactive with basal cell keratinocytes were not detected. CONCLUSION: Lichen planus may be rarely induced by neoplasia. A cell-mediated immune reaction possibly causes this phenomenon.

Adenocarcinoma↗

Necrolytic migratory erythema: a report of three cases.

Necrolytic migratory erythema (NME) is a cutaneous reaction pattern with specific histopathologic features that is typically associated with a functioning pancreatic islet cell neoplasm such as a glucagonoma. Three examples of NME, each associated with a different cause, are presented: glucagonoma, pancreatic insufficiency, and gluten-sensitive enteropathy. All three patients were successfully treated by surgical resection of the pancreatic tumor, total parenteral nutrition and pancreatic enzyme replacement, or a strict gluten-free diet, respectively. All remain free of skin disease more than 2 years later. Any patient with NME should be evaluated for glucagonoma and small bowel disease that may be associated with malabsorption and malnutrition.

Adult↗

Diffuse intraepidermal deposition of immunoreactants on direct immunofluorescence: a clue to the early diagnosis of epidermal necrolysis.

BACKGROUND: Toxic epidermal necrolysis is a distinctive disorder that is readily identified clinically and histologically in advanced cases. Early on, however, toxic epidermal necrolysis may be difficult to identify. Some consider fixed drug eruption a limited form of toxic epidermal necrolysis. METHODS: Direct immunofluorescence was performed on biopsy material of erythematous skin lesions. RESULTS: Diffuse deposition of immunoreactants in the midmalpighian layer was noted. This finding has not been encountered in other disorders studied in our immunopathology laboratory. CONCLUSIONS: Diffuse immunoreactant deposition in the mid-epidermis should suggest a diagnosis of epidermal necrolysis either from toxic epidermal necrolysis or fixed drug eruption. Additional cases will need to be assessed to document the usefulness of this pattern for prospective diagnosis.

Aged↗

Pretibial mucin. Histologic patterns and clinical correlation.

BACKGROUND AND OBJECTIVE: We identified several patients with a histologic diagnosis of pretibial myxedema in whom thyroid disease was not found. The purpose of this study was to investigate if histologic characteristics can distinguish between pretibial mucinosis secondary to Graves' disease and that unassociated with thyroid disease. METHODS: Biopsy specimens interpreted as compatible with pretibial myxedema were reviewed; these included 12 cases of pretibial mucinosis with documented Graves' disease, and six cases of pretibial mucinosis without evidence of Graves' disease. Ten specimens interpreted as compatible with stasis dermatitis were also evaluated for histologic characteristics, including the possible presence of mucin. RESULTS: Features that distinguish between pretibial mucinosis associated with Graves' disease and pretibial mucinosis without Graves' disease included preservation of a zone of normal-appearing collagen in the superficial papillary dermis (12/12 with Graves' disease, 0/6 without), mucin deposition in the reticular dermis (12/12 with Graves' disease, 0/6 without), lack of mucin deposition in the superficial papillary dermis (11/12 with Graves' disease, 1/6 without), angioplasia (2/12 with Graves' disease, 6/6 without), and the presence of hemosiderin (2/12 with Graves' disease, 6/6 without). Mucin deposition in the papillary dermis was found in six of 10 specimens interpreted as stasis dermatitis. CONCLUSIONS: There are patients with pretibial mucinosis in whom there is no thyroid disease. Specimens from patients without Graves' disease have features of stasis dermatitis in addition to mucinosis. We conclude that pretibial mucinosis may result from stasis or Graves' disease and that histologic differences allow for accurate differentiation.

Diagnosis, Differential↗

Indirect immunofluorescence on rat bladder transitional epithelium: a test with high specificity for paraneoplastic pemphigus.

BACKGROUND: Paraneoplastic pemphigus is a blistering disease with specific serum immunoprecipitation findings. Although immunoprecipitation studies allow accurate diagnosis, they are time-consuming, expensive, and not readily available. In contrast, indirect immunofluorescence (IIF) testing of serum on transitional rat bladder epithelium is a simple and inexpensive method available to any immunopathology laboratory. OBJECTIVE: Our purpose was to determine the specificity of positive IIF on rat bladder epithelium for paraneoplastic pemphigus. METHODS: The IIF findings in four index cases of paraneoplastic pemphigus were compared with the findings in 47 patients with a variety of malignant neoplasms and no associated blistering disease as well as 49 patients with vesiculobullous or lichenoid disease but no neoplasia. RESULTS: IIF was negative in all patients with neoplasia and no blistering disease and negative in all but one of the patients with vesiculobullous or lichenoid disease without neoplasia (98.9% specificity). CONCLUSION: IIF on transitional rat bladder epithelium appears to be a highly specific test for paraneoplastic pemphigus. Because of its simplicity and inexpensiveness, we suggest that IIF be performed on transitional epithelium in any suspected case of paraneoplastic pemphigus.

Animals↗

Vascular nodules and plaques resembling chronic mucocutaneous candidiasis in a patient with a low interleukin 2 level.

A 21-year-old woman had a lifelong history of widespread friable vascular nodules and plaques. Her condition had previously been diagnosed as chronic mucocutaneous candidiasis because of her clinical appearance. Routine studies of immune function were performed in addition to a comparison of cytokine secretion in mononuclear leukocytes of our patient and normal controls. Interleukin 2 secretion by phytohemagglutinin-stimulated mononuclear leukocytes was deficient when compared with six healthy donors (< 300 pg/ml vs 2108 +/- 336 pg/ml). Interleukin 2 deficiency may be a causative factor in cases of unusual vascular proliferation. Treatment with methotrexate, which increases interleukin 2 activity and inhibits neovascularization in vitro, was helpful in our patient.

Adult↗

Persistent annular erythema of infancy.

Annular erythema of infancy is an uncommon, nonpruritic, figurate erythema that begins in the first year of life. Biopsy specimens reveal a perivascular and interstitial lymphocytic infiltrate with numerous eosinophils. The cause of the disorder is unknown, but a hypersensitivity response to unrecognized antigens is suspected. The disorder is self-limited, but may last for many months. In our patient the eruption continued 19 months after its onset.

Biopsy↗

Schwannoma with sweat duct differentiation.

We report a schwannoma with well differentiated ducts resembling cutaneous sweat ducts. The tumor presented as a painless mass near the left knee of a 41-year-old female. The mass had been present for many years. Some increase in size had been noticed over the previous 2 to 3 yr. The bulk of the tumor was composed of spindle cells with the appearance of Antoni A and Antoni B tissue. Rare mitotic figures were noted. In several areas of the tumor, numerous well-differentiated ducts were present. Most resembled normal cutaneous sweat ducts. In some areas, cystic dilatation of the ducts was present. Focal areas demonstrated poorly formed ducts and single cells with prominent nuclei and ample cytoplasm. Well formed ducts, poorly formed ducts, and single cells marked with AE 1/3 (keratin) and epithelial membrane antigen. The spindle cell proliferation marked for S 100 protein and vimentin. Sweat duct differentiation has not been reported previously in either benign or malignant schwannoma. Light and electron microscopic features of this tumor are presented.

Adult↗

Malignant melanoma arising in a blue nevus with features of pilar neurocristic hamartoma.

A 65-year-old man presented with a history of a giant blue plaque of the parietal scalp since childhood. Biopsy revealed a cellular blue nevus. The blue nevus was observed for 10 years and thought to be unchanged until a new adjacent lesion was noted. Biopsy of the new lesion revealed metastatic malignant melanoma. A wide excision was performed of the original lesion which revealed malignant melanoma arising in a blue nevus. Areas within the blue nevus were consistent with a pilar neurocristic hamartoma, whereas other areas were consistent with a common blue nevus. Subsequent satellite metastases developed, with early metastases resembling blue nevi except for the absence of a stromal component and the presence of hyperchromatic nuclei. Later metastases were typical of metastatic melanoma. This case illustrates the uncommon evolution of malignant melanoma from a blue nevus. The histological features and relationship between melanoma, blue nevus, and pilar neurocristic hamartoma are reviewed.

Aged↗