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Biomedical subjects

C Perniciaro

Publications and source records attributed to C Perniciaro.

At least 37 records · Page 2Linked to original sources
PubMed1996

Familial Kaposi's sarcoma.

Familial cases of Kaposi's sarcoma are exceedingly rare. We identified an 85-year-old man with Kaposi's sarcoma. Multiple lesions were present on the feet. Previously, the man's 84-year-old sister presented with similar lesions on the lower extremity. Both siblings were lifelong residents of the United States and were of English/German descent. Neither had risk factors for infection with human immunodeficiency virus (HIV). On histologic examination, lesions in both patients demonstrated vascular slits, hemorrhage, and dense aggregates of spindle cells within the dermis.

Aged↗
PubMed1995

POEMS syndrome.

The principal features of POEMS syndrome are osteosclerotic bone lesions, peripheral sensorimotor neuropathy, and elevated levels of a monoclonal protein in the serum or urine. Skin lesions are present in the majority of patients with POEMS syndrome. Diffuse hyperpigmentation is the most common cutaneous finding. Hemangiomas with histopathological features resembling renal glomeruli are the most specific skin lesions.

Humans↗
PubMed1995

Malignant angioendotheliomatosis is an angiotropic intravascular lymphoma. Immunohistochemical, ultrastructural, and molecular genetics studies.

Malignant angioendotheliomatosis is a rare intravascular (angiotropic) lymphoma. Patients most often present with cutaneous or central nervous system findings. We describe three patients with malignant angioendotheliomatosis involving the skin. The initial lesions in each were tender, indurated nodules on the lower extremities, resembling inflammatory panniculitis. Skin biopsies and immunohistochemical studies from all patients confirmed intravascular B-cell lymphoma. Two patients had visceral involvement, and molecular genetics studies showed clonal immunoglobulin gene rearrangement in one. Electron microscopy in this case showed increased fibrin and atypical lymphocytes within blood vessels. Malignant angioendotheliomatosis is a monoclonal intravascular lymphoma, usually of B-cell phenotype. Occlusion of small blood vessels with lymphoid cells, fibrin, and degenerating cellular debris causes the cutaneous lesions. An excisional biopsy through the depth of subcutaneous tissue may be necessary to confirm the diagnosis of malignant angioendotheliomatosis.

Aged↗
PubMed1995

Hypertrophic discoid lupus erythematosus resembling squamous cell carcinoma.

BACKGROUND: Hypertrophic discoid lupus erythematosus (HDLE) may resemble cutaneous squamous cell carcinoma (SCC) histologically. OBJECTIVE: The histologic features that help to differentiate HDLE from SCC are reviewed. METHODS: Two patients are described with HDLE. RESULTS: Both patients were treated initially with Mohs surgery for "biopsy-proven" SCC. Subsequent biopsies confirmed the diagnosis of HDLE and the patients responded well to appropriate therapy. No significant complications occurred in either patient. CONCLUSION: Lesions of HDLE may imitate SCC. Strategies are suggested to avoid surgical procedures based on an incorrect biopsy report.

Adult↗
PubMed1995

Gardner's syndrome.

Gardner's syndrome is an autosomal dominant genodermatosis. Familial polyposis of the colon, osteomas, and cutaneous epidermoid cysts are characteristic features. Colon cancer will develop in all affected individuals unless prophylactic colectomy is performed. The follow-up and management of patients with Gardner's syndrome require a coordinated effort by physicians with expertise in gastroenterology, general surgery, oral surgery, radiology, endocrinology, neurology, ophthalmology, and dermatology. Genetic and psychological counseling should also be available for these patients.

Adenomatous Polyposis Coli↗
PubMed1994

Paraneoplastic pemphigus: a case of prolonged survival.

OBJECTIVE: To describe a patient with severe, recalcitrant mucous membrane erosions and chronic lymphocytic leukemia. DESIGN: We present a case report and a literature review of paraneoplastic pemphigus. MATERIAL AND METHODS: Immunofluorescence studies and immunoprecipitation confirmed the presence of autoantibodies characteristic of paraneoplastic pemphigus in the patient's serum. RESULTS: Our patient lived almost 8 years after the onset of paraneoplastic pemphigus, the longest time that anyone with this disease is known to have survived. CONCLUSION: The clinical course of paraneoplastic pemphigus tends to be rapid and fatal despite immunosuppressive therapy. Long-term survival is uncommon.

Humans↗
PubMed1994

Bullous morphea: clinical, pathologic, and immunopathologic evaluation of thirteen cases.

BACKGROUND: Bullous morphea is a rare disease. Its pathogenesis is unknown. OBJECTIVE: We evaluated bullous morphea clinically, pathologically, and immunopathologically and investigated the role of spirochetes and eosinophils in its pathogenesis. METHODS: The clinical and pathologic findings from 13 patients with bullous morphea were reviewed. Tissue sections were studied with the Elias-Bosma stain for spirochetes and indirect immunofluorescence for eosinophil granule major basic protein. RESULTS: Bullae were found in all forms of morphea; the lower extremities were the most common sites of involvement. Lymphatic dilatation was found in 77% of the patients. Deposition of major basic protein was found in 60% of cases studied. There was no evidence of spirochetes in any of the specimens examined with the Elias-Bosma stain. CONCLUSION: Our results suggest that the pathogenesis of bullous morphea is related to lymphatic dilatation as well as release of major basic protein from eosinophils in some patients. We found no association between spirochetes and bullous morphea.

Adult↗
PubMed1994

Granuloma annulare in patients with malignant lymphoma: clinicopathologic study of thirteen new cases.

BACKGROUND: Reports of necrobiotic granulomas or granuloma annulare in patients with malignant lymphoma are rare. OBJECTIVE: Our intent was to determine any unique clinical or histopathologic features in patients with granuloma annulare and lymphoma. METHODS: We reviewed the medical records and biopsy material from 13 patients with granuloma annulare and lymphoma. RESULTS: Three patients had Hodgkin's disease and 10 had non-Hodgkin's lymphoma. The granuloma annulare lesions showed typical histopathologic features. However, the clinical pattern was frequently atypical, with painful lesions in unusual locations including the palms and soles. Three patients displayed granulomatous inflammation in noncutaneous sites, either within the malignant lymphoma or in uninvolved tissues, and all three had atypical clinical presentations of granuloma annulare. CONCLUSION: Granuloma annulare with atypical clinical presentations may be associated with an underlying hematopoietic malignancy and may be part of a generalized granulomatous reaction to malignant lymphoma.

Adult↗
PubMed1994

Fatal systemic cytophagic histiocytic panniculitis: a histopathologic and immunohistochemical study of multiple organ sites.

The presence of hemocytophagic histiocytosis within subcutaneous tissue has been termed "cytophagic histiocytic panniculitis" (CHP). CHP may occur as a feature of hematopoietic malignancies or infections, particularly viral. In some patients with CHP, an underlying illness cannot be identified. We describe a young man with a rapidly fatal systemic illness characterized by CHP. Lymphoma was not present, and an infectious agent could not be identified. Immunohistochemical stains of tissue obtained at autopsy from multiple organ sites confirmed the presence of histiocytes and T lymphocytes within adipose tissue.

Adipose Tissue↗
PubMed1994

Hidroacanthoma simplex: an ultrastructural and immunohistochemical study.

A representative case of hidroacanthoma simplex was studied with routine light microscopy, immunohistochemistry, and electron microscopy. Staining with the periodic acid-Schiff reagent and immunostaining with anti-keratin antibodies were useful in demarcating the tumor cells from adjacent normal epithelium. However, antibodies to carcinoembryonic antigen and epithelial membrane antigen did not help us to segregate or identify the neoplastic cells. Electron microscopy revealed tumor cells markedly different in appearance from luminal cells of the acrosyringium. Hidroacanthoma simplex does not appear to be derived from luminal cells of the acrosyringium. We propose criteria for the histologic diagnosis of this benign neoplasm.

Aged↗
PubMed1994

Chilblain lupus erythematosus (lupus pernio): clinical review of the Mayo Clinic experience and proposal of diagnostic criteria.

Five cases of chilblain lupus erythematosus were retrospectively reviewed regarding their clinical, histopathologic, serologic, and immunofluorescence findings. Ages at onset of chilblain lupus erythematosus varied from 26 to 73 years, with a female-to-male ratio of 3:2. Since other entities can be confused with this disorder, we propose the following diagnostic criteria. The two major criteria are skin lesions in acral locations induced by exposure to cold or a drop in temperature, and evidence of lupus erythematosus in the skin lesions by results of histopathologic examination or direct immunofluorescence study. The three minor criteria are coexistence of systemic lupus erythematosus or other skin lesions of discoid lupus erythematosus, response to anti-lupus erythematosus therapy, and negative results of cryoglobulin and cold agglutinin studies. We conclude that chilblain lupus erythematosus can be diagnosed and treated. Discoid lupus erythematosus lesions respond more quickly to treatment than chilblain lupus erythematosus lesions. Treatment with antimalarial agents, prednisone, pentoxifylline, or dapsone was of benefit to our patients.

Adult↗
PubMed1993

Subcutaneous T-cell lymphoma. Report of two additional cases and further observations.

BACKGROUND: Peripheral T-cell lymphomas involving subcutaneous tissue are rare. With modern immunohistochemical stains, subcutaneous T-cell lymphomas may be demonstrated in patients previously thought to have fatal Weber-Christian disease, malignant histiocytosis, and histiocytic cytophagic panniculitis. OBSERVATIONS: We describe two young women with subcutaneous lymphomas. They presented with erythematous nodular lesions, which suggested benign panniculitis. Skin biopsy specimens in both patients demonstrated large atypical lymphocytes in the subcutaneous tissue, karyorrhexis, and cytophagia. Immunohistochemical studies confirmed a T-cell phenotype. Molecular genetics studies performed in one patient showed a major T-cell clonal population. CONCLUSIONS: Subcutaneous T-cell lymphoma is a rare, distinct subtype of postthymic peripheral T-cell lymphoma. Cytophagic "histiocytic" panniculitis is frequently a prominent histologic feature. Early systemic chemotherapy may offer therapeutic advantages in patients with this disease.

Adult↗
PubMed1993

Two cases of vulvodynia with unusual causes.

Two women with vulvodynia are described. In one patient, severe chronic vulvodynia developed secondary to contact dermatitis. Patch-testing confirmed the offending allergens. A second patient with vulvodynia was severely dermatographic. Evaluation of patients with vulvodynia should include an appropriate medical history and diagnostic studies to exclude contact dermatitis and dermatographism. Effective treatment for these disorders may lead to dramatic relief of symptoms.

Adult↗