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Biomedical subjects

B Ilie

Publications and source records attributed to B Ilie.

At least 19 recordsLinked to original sources

Dorfman-Chanarin syndrome: morphologic studies and presentation of new cases.

Dorfman-Chanarin syndrome, or neutral lipid storage disease with ichthyosis, is a rare inherited metabolic disorder characterized by accumulation of neutral lipids in different tissues. Variability in dermatologic severity is not understood. We report two new cases, compare their features with other reported cases, and examine the possible relationship between the severity of the dermatologic condition and lipid accumulation in various types of skin cells. The localization of lipid droplets was determined in various dermal and epidermal cells by light microscopy of epoxy resin semithin sections and electron microscopy in four cases of Dorfman-Chanarin syndrome. Lipid droplets in the dermis were detected in fibroblasts, Schwann cells in both myelinated and unmyelinated nerves, smooth muscle cells, and sweat gland cells, but not in endothelial cells of blood capillaries. The droplets were not surrounded by a membrane. Findings indicate that there is no correlation between the clinical severity of the disease and the microscopic findings.

Adolescent↗

Small cell carcinoma of the gallbladder: clinical course and response to chemotherapy.

Small cell carcinoma of the gall bladder is a rare tumor. The neoplasm is highly lethal, metastasizes early, and may cause death shortly after diagnosis. An oat cell carcinoma of the gallbladder metastatic to the liver and adjacent lymph nodes is described in a 60-year-old male. Partial cholecystectomy was performed followed by aggressive chemotherapy with etoposide and cisplatinum. An 80% reduction in the size of the unexcised tumor was noted over a period of 6 months. The partial response and the relatively long survival of the patient suggest the use of the above protocol for these rare cases.

Antineoplastic Combined Chemotherapy Protocols↗

Malignant melanoma of the mucous membranes.

Primary malignant melanoma occurring in mucous membranes is uncommon. Of 410 patients with malignant melanoma, 14 (3.4%) had their primary tumour located in mucosal membranes. Five of the tumours were in the mucosal lining of the head and neck, five in the gastrointestinal tract mucosa and four in the female genitalia. Eight of the tumours were ulcerated, necrotic and deeply invasive. The most common histological subtype was acral lentiginous melanoma. Surgical resection of the primary tumour was performed in 10 patients. In the other four, surgery was not feasible, and they were treated by irradiation and immunotherapy. Six patients underwent regional lymph node dissection. In four of them, regional metastases were found. Chemotherapy was given to seven patients for widespread metastases. Nine patients (64%) died of metastatic melanoma within 2 years. The remaining five patients are alive after 3 to 8 years. It appears that mucosal membrane melanoma is a distinct variant of the disease due to both its aggressive biological behaviour and its relative inaccessibility for surgical removal, and may therefore be classified separately.

Adult↗

Vaginal ulcerations due to lithium carbonate therapy.

A twenty-nine-year-old woman who had been taking lithium carbonate for seven years experienced ulcerated lesions of the vaginal mucosa and acneiform eruption on both thighs. Histopathologic appearance of the skin biopsy specimens was consistent with lichenoid cutaneous changes. The cutaneous lesions cleared when use of the drug was discontinued.

Acne Vulgaris↗

Spinal epidural compression in chronic lymphocytic leukemia.

Spinal epidural compression is a rare neurologic complication in patients with lymphoma. It occurs mostly in those with intermediate-grade to high-grade malignancy disease. This type of neurologic involvement has not been described in chronic lymphocytic leukemia (CLL). A patient with a long, stable CLL course developed spinal epidural compression and consequently died. The frequency of spinal epidural compression in lymphoma, according to the histologic subtypes and the considerations in making the right choice of therapy are discussed in light of the presented case.

Aged↗

Epitheloid hemangiomatosis mistaken for carcinomatosis.

We present a unique case of intra and extra-peritoneal epitheloid hemangioma which was mistaken for diffused carcinomatosis, representing a clinical-pathological problem, and emphasizing the unpredictable behaviour of this tumour. We are dealing with epitheloid vascular tumours, and briefly touch on the differential diagnosis, the principle of biological, pathological and clinical behaviour. Knowledge of this entity would provide an accurate diagnosis and would hence improve dealing with the problem. The conclusion is that this group of Histiocytoid-epitheloid vascular lesions have varying biological potential and this unusual functional state represents a large spectrum of cells, from normal to neoplastic endothelium.

Abdominal Neoplasms↗

Dorfman-Chanarin syndrome. A case report and a review.

Dorfman-Chanarin syndrome in two sisters of Jewish Iraqi origin is reported. This heritable disorder of the metabolism of neutral lipids was manifested by congenital ichthyosis, vacuoles in the leukocytes, and variable involvement of liver, muscle, central nervous system, and the auditory system. In two asymptomatic members of the family leukocyte vacuoles were found as the only sign of the syndrome. Clinical, pathologic, ultrastructural, and biochemical findings are described. Previous reported cases are reviewed.

Adult↗

[Kaposi's sarcoma localized on the shaft of the penis].

The importance of Kaposi's Sarcoma, known for over a century, resides in its relation to AIDS. The localisation on the shaft of the penis is very rare even in the generalised forms, more so when the lesion is primary and localized. The basic lesion appears as a reddish-violet nodule. The authors report a case of a primary Kaposi's Sarcoma in a 58 year-old patient where the tumor appeared as a pedunculated 2 X 2 cm lesion with a necrotic surface. The tumor was excised. A search for internal foci of KS was negative. The complete cure was proven by decreased lymphocyte proliferative responses and defective natural-killer cell activity.

Humans↗

Neoplasms in skin and subcutis over the breast, simulating breast neoplasms: case reports and literature review.

Twenty cases of neoplasms in skin and subcutaneous tissue over the breast were reviewed. There were 17 women, from 15 to 70 years of age, and three men, from 25 to 66 years of age. Among the benign skin neoplasms, superficial leiomyoma, granular cell tumor, and eccrine acrospiroma were misdiagnosed clinically as primary breast carcinoma. Among the malignant neoplasms in subcutaneous tissue, there were three metastatic malignant melanomas, one metastatic epidermoid bronchogenic carcinoma, and two malignant lymphomas. It is interesting that four of these six patients had no prior history of malignant lesion, the subcutaneous nodule presenting as the first manifestation of an occult primary. It is concluded that histological diagnosis of such tumors may lead to avoidance of unnecessary radical surgery.

Adenoma, Sweat Gland↗

Propylthiouracil-induced cholestatic jaundice.

The typical propylthiouracil (PTU)-linked hepatotoxicity, is known to manifest itself by hepatocellular injury with greatly increased serum transaminase values and evidence of hepatic necrosis on liver biopsy. Herewith presented is a 33-year old, thyrotoxic woman who developed cholestatic jaundice two weeks after initiation of PTU therapy. The diagnosis was confirmed by liver biopsy. A causal link between PTU treatment and the cholestatic jaundice was suggested by: the time of onset, typical skin rash and a positive migration inhibition factor (MIF) test to PTU. Awareness of this rare complication of PTU treatment may prevent the performance of unnecessary, expensive and possibly harmful diagnostic procedures.

Adult↗

Mucoid milia.

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Aged↗

Scleredema adultorum associated with localized myositis.

A 70-year-old man presented with large areas of brawny edema of the skin and neck and weakness of the muscles of the forearm. Biopsy revealed scleredema adultorum with inflammatory changes in the forearm muscle. An electromyogram showed a mixed pattern compatible with myositis.

Aged↗

Renal cell carcinoma presenting as acute pancreatitis and GI bleeding.

A 75-year-old woman was admitted for acute pancreatitis, jaundice, GI bleeding, and a tender mass in the right hypochondrium. Upper gastrointestinal series demonstrated a diverticulum-like structure on the lateral aspect of the second part of the duodenum. This structure was excised and turned out to be a renal cell carcinoma invading the duodenum by direct extension from the right kidney. Although renal cell carcinoma may present in numerous and often perplexing manifestations, this is a unique presentation.

Acute Disease↗

Granulomatous peritonitis caused by glove starch.

Corn starch particles are used as a surgical glove lubricant. At present there is no better alternative for this lubricant. Implantation of corn starch particles into the peritoneal cavity can induce foreign body reactions, starch peritonitis and starch granulomata, and may cause adhesions and intestinal obstruction. Starch peritonitis should be treated conservatively.

Gloves, Surgical↗

Perforating cutaneous mucinosis.

A case is described in which a papulonodular eruption developed all over the body. Histologic examination of one of the lesions revealed the dermis to contain mucin (hyaluronic acid). In some areas there was perforation through the epidermis of the mucinous material.

Adult↗