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Biomedical subjects

E E Sahn

Publications and source records attributed to E E Sahn.

17 recordsLinked to original sources
PubMed1992

Annular epidermolytic ichthyosis: a unique phenotype.

A 30-year-old white woman developed bullous and ichthyosiform skin lesions at the age of 8 months. Blistering ceased at puberty, but the hyperkeratotic plaques persisted. She subsequently delivered five children, two of whom were affected with a bullous type of ichthyosis. At the age of 27 years, she developed numerous annular and polycyclic, erythematous, hyperkeratotic plaques on the trunk and extremities that were pruritic, enlarged slowly, and then resolved. Histopathologic examination of these annular plaques revealed epidermolytic hyperkeratosis. Electron microscopic examination of the annular plaques showed interrupted perinuclear tonofilament rings consistent with either bullous ichthyotic erythroderma of Brocq or ichthyosis bullosa of Siemens. This kindred may represent a new phenotype of epidermolytic (acanthokeratolytic) ichthyosis.

Adult↗
PubMed1992

Demodicidosis in a child with leukemia.

A 2-year-old girl developed acute lymphoblastic leukemia at the age of 9 months. She was treated successfully with chemotherapy but developed a pruritic, papulopustular facial eruption that was caused by Demodex folliculorum. The eruption cleared after treatment with one overnight application of 5% permethrin cream. Demodicidosis should be included in the differential diagnosis of facial eruptions in children who undergo chemotherapy and in those with congenital or acquired immunodeficiency.

Child, Preschool↗
PubMed1991

Follicular accentuation of leukocytoclastic vasculitis in an HIV-seropositive man. Report of a case and review of the literature.

Palpable purpura in a follicular localization developed in association with acute epididymitis in a white man who was seropositive for the human immunodeficiency virus (HIV). Biopsy specimens revealed a leukocytoclastic vasculitis with follicular accentuation. With antibiotic therapy the vasculitis resolved, but it recurred during repeated episodes of the epididymitis. Follicular accentuation of skin disease is often seen in HIV-seropositive patients. Leukocytoclastic vasculitis with a follicular localization may be the presenting skin manifestation of HIV infection.

Adult↗
PubMed1990

Scleroderma following augmentation mammoplasty. Report of a case and review of the literature.

A 46-year-old woman developed localized scleroderma after surgical manipulation of her silicone gel-filled breast prostheses. She developed firm, shiny plaques on her legs that progressed to involve the thighs. Histopathologic examination of a deep-skin biopsy specimen confirmed the diagnosis of scleroderma. On surgical removal of the silicone implants, and their replacement with saline-filled implants, the scleroderma gradually resolved. Histopathologic examination of the removed implant capsules revealed evidence of silicone leakage. All new female patients with scleroderma should be questioned and examined regarding augmentation mammoplasty. Until prospective studies are completed on the possible association between scleroderma and silicone breast implants, it would seem prudent to use the saline-filled, elastomeric envelope-type breast implant for augmentation mammoplasty rather than the silicone gel-filled implant.

Breast↗
PubMed1990

Pyoderma gangrenosum in pediatric acquired immunodeficiency syndrome.

We describe two children with human immunodeficiency virus infection in whom pyoderma gangrenosum developed. Although pyoderma gangrenosum most commonly occurs in children with inflammatory bowel disease, it has also been described in patients with a variety of immunodeficiencies. In such patients a vigorous search to exclude a treatable infection should be made before the lesions are treated as pyoderma gangrenosum.

Acquired Immunodeficiency Syndrome↗
PubMed1990

Multiple rhabdomyomatous mesenchymal hamartomas of skin.

A case of multiple rhabdomyomatous mesenchymal hamartomas is presented. The patient is a black male infant, the product of an uncomplicated term gestation and delivery. At birth, there were numerous polyps distributed over the periorbital and periauricular areas bilaterally. Some appeared fingerlike with constrictions below their tips. Others were branched or globular in shape. These projections showed spontaneous and independent movement, particularly during feedings. On histopathologic examination, the polyps were covered by squamous epithelium and contained normal follicular units. Bundles of skeletal muscle were present in the reticular dermis, extending into the subcutis. Regular cross-striations were seen in these muscle fibers. In some specimens, the muscle bundles formed a solid, central core. Skeletal muscle histochemical stains confirmed the presence of both types 1 and 2 muscle fibers. Electron microscopy revealed a normal skeletal muscle banding pattern. This case is the first report of multiple rhabdomyomatous mesenchymal hamartomas of skin. Functional skeletal muscle with spontaneous movement is part of the clinical picture.

Diagnosis, Differential↗
PubMed1990

Preschool sarcoidosis masquerading as juvenile rheumatoid arthritis: two case reports and a review of the literature.

Preschool sarcoidosis is characterized by the triad of skin, joint, and eye disease without pulmonary involvement. Arthritis and uveitis are also frequently seen together in juvenile rheumatoid arthritis. We report two patients with preschool sarcoidosis, both of whom were initially diagnosed and treated as having juvenile rheumatoid arthritis. Skin biopsy is an important early diagnostic procedure to differentiate the conditions. The uveitis in both diseases can have serious sequelae.

Arthritis, Juvenile↗
PubMed1989

D-penicillamine-induced elastosis perforans serpiginosa in a child with juvenile rheumatoid arthritis. Report of a case and review of the literature.

Elastosis perforans serpiginosa is a rare complication of D-penicillamine therapy. It has been reported to occur in Wilson's disease and cystinuria, usually after many years of high-dose therapy. We report a case of D-penicillamine-induced elastosis perforans serpiginosa with unique clinical features occurring in a 10-year-old child with juvenile rheumatoid arthritis who received only 71 gm of the drug over 9 months. The case is also unusual because of the short course and low cumulative dose of drug received and because of the calcification of elastic fibers. The calcification of elastic fibers suggests that this case may represent an unusual variant of elastosis perforans serpiginosa or an overlap with pseudoxanthoma elasticum. All reported cases of D-penicillamine-induced elastosis perforans serpiginosa are reviewed, and histopathologic and electron microscopic findings are presented. The theoretic mechanisms of action of D-penicillamine on elastic tissue synthesis and morphology are discussed.

Arthritis, Juvenile↗
PubMed1988

A new method for the comprehensive automatic morphologic image analysis of Langerhans cells.

Standard techniques used to study Langerhans cell (LC) populations are tedious and time-consuming. We developed a rapid and comprehensive method to evaluate LC using automatic digital image analysis. Human epidermal sheets were stained with OKT6 monoclonal antibody and evaluated on an IBAS 2000 computerized automatic digital image analysis system. The fields were imaged on a Photomicroscope I using a high-resolution monochrome video camera. Each field was digitized, normalized, and filtered. The LC were discriminated by density; each field was interactively edited to separate overlapping cells and to bridge small gaps in the dendrites. The discriminated binary image of the LC was measured for number of cells per field, total area of individual cells per field, and percent of the field occupied by LC. The cell bodies were then separately discriminated and subtracted from the thinned cell image to allow a separate measurement of number and length of dendrites per cell. Finally, the binary cell image in a reference section was dilated to occupy approximately the total field area. This degree of dilation was then applied to all subjects to give the "area of influence." This methodology provides a rapid and comprehensive tool for the evaluation of LC.

Autoanalysis↗
PubMed1976

Wegener granulomatosis with aphasia.

Wegener granulomatosis, a necrotizing granulomatous vasculitis that characteristically involves the respiratory tract and the kidneys, may affect the nervous system. Despite the relative frequency of neurologic manifestations, there has not been a single report of Wegener granulomatosis manifesting as a cerebral vascular accident. Our patient had limited Wegener granulomatosis with aphasia as the symptom that was observed first. A dramatic recovery occurred after corticosteriod and cytotoxic therapy.

Aphasia↗
PubMedDate not supplied

Spinal cord compression in lymphoma.

A retrospective analysis of 59 patients with diffuse histiocytic and diffuse poorly differentiated lymphocytic lymphoma was performed to determine the incidence and characteristic features of patients developing spinal cord compression. Spinal cord compression occurred in 6 of 59 (10.2%) patients. All were females despite a 1:1 male: female ratio in the entire group. The mean age of females with spinal cord compression was significantly lower than the mean age of females with lymphoma without this complication. These findings suggest that young females with diffuse histiocytic or diffuse poorly differentiated lymphocytic lymphoma represent a subgroup at high risk for spinal cord compression.

Adult↗