Bullous pyoderma gangrenosum.

Pyoderma gangrenosum is a necrotizing, ulcerative process commonly associated with inflammatory bowel disease but also occurring in several other systemic illnesses. When associated with myelodysplastic syndromes, its clinical appearance is often atypical. We describe a patient with severe, refractory bullous pyoderma gangrenosum. At autopsy cardiac involvement was demonstrated, a finding not previously reported. Patients with this unusual variant of pyoderma gangrenosum have a grave prognosis.

Persistent scabies in nursing home patients.

Persistent scabies outbreaks in extended care facilities result from multiple factors: atypical presentations in the elderly, leading to delayed or inaccurate diagnosis; incomplete or ineffective treatment of the patient and exposed population; and failure to coordinate notification, education, treatment, and disinfection. A case report of scabies in an elderly resident of an extended care facility illustrates various types of scabies manifestations, the advisability of obtaining diagnostic scrape preparations, and the efficacy and safety of permethrin. Guidelines for an aggressive and comprehensive effort for eradication are offered.

Acute alopecia: clue to thallium toxicity.

The combination of rapid, diffuse alopecia, and neurologic and gastrointestinal disturbance is pathognomonic for thallium toxicity. The hair mount, showing a tapered or bayonet anagen hair with black pigmentation at the base, may be highly diagnostic before the onset of alopecia. We saw a 10-year-old boy who suffered from thallium poisoning.

Solitary congenital indeterminate cell histiocytoma.

BACKGROUND: Indeterminate cell proliferations are rarely described in the literature. We present a case of a patient who presented at birth with a solitary lesion that spontaneously regressed. OBSERVATIONS: Five cases of indeterminate cell proliferations have been described in the literature. Their clinical patterns display some common features including adult onset, usually with multiple lesions and a relatively benign clinical course. This patient presented at birth with a single lesion and spontaneous regression, as one would expect with congenital self-healing reticulohistiocytosis. Biopsy confirmed a dermal histiocytic nodule that was predominantly CD1 and S100 positive. Unlike patients with congenital self-healing reticulohistiocytosis, however, electron microscopy failed to demonstrate Birbeck granules. CONCLUSION: Indeterminate cell proliferations are a distinct entity. This presentation expands the range of clinical manifestations reported. It also suggests a close relationship between indeterminate cells and Langerhans cells.

Sjögren-Larsson syndrome: case reports.

Sjögren-Larsson syndrome (SLS) is a rare, autosomal recessive disorder with worldwide distribution. It consists of ichthyosis, spastic diplegia, and mental retardation. An enzymatic defect in fatty alcohol oxidation recently was identified and is thought to be responsible for the disorder. We report two siblings with SLS. In addition to the typical features of the syndrome, the sister had marked hyperkeratosis of the palms and soles, which is rarely seen to this degree. The brother had joint hyperextensibility, which has not been reported previously. Both individuals had documented deficient activity of fatty alcohol:NAD+ oxidoreductase.

Afferent connections of the median preoptic nucleus in the rat: anatomical evidence for a cardiovascular integrative mechanism in the anteroventral third ventricular (AV3V) region.

The afferent connections of cell groups in the anteroventral third ventricular (AV3V) region were investigated using wheat germ agglutinin-horseradish peroxidase conjugate injections into the AV3V area. The median preoptic nucleus was found to receive a unique set of inputs from the subfornical organ, the paraventricular nucleus of the hypothalamus, the parabrachial nucleus, the nucleus of the solitary tract and the ventrolateral medulla. As each of these structures has been implicated in various aspects of cardiovascular regulation, it is likely that the cardiovascular effects of AV3V lesions are due at least in part to median preoptic damage.