The management of Darier's disease

Darier's disease (keratosis follicularis) is a rare, dominantly inherited condition which is characterised by the presence of warty papules and plaques on the trunk, scalp and flexures. Diagnostic nail changes are present in most patients. Suprabasal clefting, acantholysis and dyskeratosis are present in involved skin and the diagnosis may be confirmed by a skin biopsy. The disease never remits. Exacerbating factors such as heat, sweating and ultraviolet light should be avoided. Topical therapy with emollients, moderately potent corticosteroids and antimicrobials may provide some symptomatic relief, but have no effect on the progress of the disease. Oral retinoids are effective in most patients. Complications such as cutaneous infections, blistering and salivary gland obstruction may occur in some patients.     

Humanized monoclonal antibody treatment in rheumatoid arthritis.

A 41-year-old woman with active, seropositive erosive rheumatoid arthritis was treated with the humanized monoclonal antibody Campath 1H. She had not responded or developed side effects to myocrisin, sulfasalazine and penicillamine, and had not responded to inpatient bedrest and physiotherapy. There was a rapid clinical improvement within 24 hours of infusion, which was maintained for about 12-14 weeks after the infusion. The lymphocyte count was suppressed for 7 months after treatment. There were no significant side effects during or after treatment. No anti-Campath 1H response was detected. This preliminary study suggests humanized monoclonal antibody therapy may be of value in the treatment of rheumatoid arthritis.     

Ampullary carcinoma: Demonstration by current MR techniques

The objective of this study was to demonstrate the appearance of ampullary carcinoma using current MR techniques, including fat suppression, gadolinium enhancement, and MR cholangiography. Nine patients with ampullary carcinoma were examined by MRI at 1.5 T. MR examinations included T1-weighted spoiled gradient echo, T1-weighted fat-suppressed, and immediate postgadolinium spoiled gradient echo images for all patients and MR cholangiography for three patients. The imaging features of ampullary carcinomas, including tumor size and morphology, signal intensity, and enhancement characteristics, were determined. Ampullary carcinomas shown on MR images ranged in size from 1.5 to 5.5 cm. Tumors were low in signal intensity on precontrast T1-weighted spoiled gradient echo and T1-weighted fat-suppressed images relative to normal pancreatic tissue and enhanced less than normal pancreas on immediate postgadolinium spoiled gradient echo images. Tumor conspicuity was greatest on immediate postgadolinium spoiled gradient echo images. MR cholangiography demonstrated high grade obstruction of the common bile duct and mild dilatation of the pancreatic duct at the level of the ampulla with abrupt termination of the ducts in two untreated patients and moderate dilatation of the common bile duct in one patient who had a biliary stent. Ampullary carcinomas can be demonstrated on MR images as small masses arising at the ampulla. Tumors are well defined on immediate postgadolinium spoiled gradient echo images.     

Mortality studies of machining fluid exposure in the automobile industry III: A case-control study of larynx cancer

A case-control study of larynx cancer was conducted within a cohort of automobile workers exposed to metal working fluids, commonly referred to as machining fluids (MF). Results are based on 108 cases of larynx cancer and 5:1 matched controls. Risks associated with specific types of MF, as well as specific components of the fluids were evaluated. Based on a retrospective exposure assessment, lifetime exposures to straight and soluble fluids, grinding particulate, biocides, selected metals, sulfur, and chlorine were examined. Exposure to asbestos and acid mists at two of the three study sites was also characterized. Results suggest that straight mineral oils are associated with almost a two-fold excess in larynx cancer risk. There was also evidence of an association with elemental sulfur, commonly added to straight MF to improve the integrity of the materials under extreme pressure and heat. It is not clear whether sulfur is causally related to an excess relative risk of larynx cancer or whether the observed association is the result of unmeasured confounding by another contaminant or process feature. For example, the high stress operations that require MF enriched with sulfur are also more likely to produce polycyclic aromatic hydrocarbons (PAHs) during the process. Thus, the observed association with sulfur may be due to an association with PAH. The finding of excess risk of laryngeal cancer associated with MF is consistent with several previous reports in the literature. This is the first study, however, to distinguish straight mineral oils from other types of MF. Based on these findings, a general reduction in concentrations of straight mineral oil particulate in occupational environments would be prudent.     

Transmission of methicillin-resistant Staphylococcus aureus in the neonatal intensive care unit from a patient with community-acquired disease.

Methicillin-resistant Staphylococcus aureus (MRSA) has traditionally been a nosocomial pathogen. However, several recent studies have noted community-acquired MRSA among young, healthy patients with no risk factors or healthcare system exposure. We report the transmission of a strain of community-acquired MRSA in our neonatal intensive care unit.     

Identity formation in adolescence: Case study of gender identity disorder and treatment through an intermediate-care day hospital

A review of the literature on gender identity disorders is integrated with a case study presentation of a psychiatrically disturbed nineteen-year-old transvestite youth. Accommodations and interventions made both with this patient and in the day care program for psychiatrically disturbed youths that allowed him to live at home and be maintained despite severe pathology are discussed. Amaro Reyes, M.D. was formerly an attending Psychiatrist in the Community Division of Bronx Children's Psychiatric Center