1/4. Protean manifestations of lipoid proteinosis in a 16-year-old boy.We report a 16-year-old Japanese male with lipoid proteinosis showing various skin manifestations. The patient was born to nonconsanguineous parents and none of his relatives was similarly affected. The patient suffered from a hoarse voice and refractory temporal epilepsy from early childhood. Computed tomography scanning of the brain showed bilateral calcification in the temporal lobes, a characteristic feature of lipoid proteinosis. On physical examination, various skin manifestations, including papules and haemorrhagic blisters, acne-like scars at sites of minor trauma or friction, and beads of small papules along the free margins of the eyelids were noted. A skin biopsy showed deposits of homogeneous hyaline-like material, positive on periodic acid-Schiff staining, throughout the dermis, particularly around small blood vessels. It is noteworthy that a range of characteristic skin lesions can be present in a patient with lipoid proteinosis even with mild systemic involvement.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
2/4. Hyalinosis cutis et mucosae.Hyalinosis cutis et mucosae is an uncommon genetic disease characterized by accumulation of glycoproteinaceous material not only at mucocutaneous sites but also in salivary glands, central and peripheral nervous systems, eyes, nd other organs and tissues. The course of the disease is protracted, and it is not likely to regress, either spontaneously or with therapy. Although it does not usually result in decreased longevity, the disorder may produce considerable disfigurement and functional impairment. This article documents the case of a 5-year-old girl who presented with vocal weakness, misarticulations, and hoarseness; hypopigmented scarring of the arms and pubis; fibrotic thickenings of the oral and laryngeal mucosa; and serous otitis media. A tongue biopsy revealed diffuse hyaline deposits throughout the connective tissue and in a lamellar pattern about blood vessels. A diagnosis of hyalinosis cutis et mucosae was made.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
3/4. Traumatic retropharyngeal hematoma: a case report.We describe a case of retropharyngeal hematoma after a cervical hyperextension injury in an elderly man. Progressive hoarseness, dysphagia, and dyspnea were the early signs that necessitated oral endotracheal intubation and, ultimately, tracheostomy. The hematoma was explored and drained through a lateral cervical approach, and a bleeding vessel in a small tear in the anterior spinous ligament was noted and cauterized. The patient recovered uneventfully.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
4/4. Clinically probable brainstem stroke presenting primarily as dysphagia and nonvisualized by MRI.Ten patients with clinically probable brainstem stroke presenting primarily as acute dysphagia but without visible brainstem abnormality by MRI are described. The patients were evaluated with neurologic examinations, cinepharyngoesophagography, and brain MRI studies. Each patient solely or predominately experienced sudden pharyngeal dysphagia, and additional symptoms or signs other than dysphonia or dysarthria were scarce. Small vessel disease or cardiac embolism were the apparent causes of what appear to have been very discrete brainstem strokes in these patients. Acute pharyngeal dysphagia can be the sole or primary manifestation of brainstem stroke. A negative MRI study should not preclude consideration of this diagnosis, if brainstem stroke is otherwise clinically probable.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |