11/59. The glucagonoma syndrome. A distinctive cutaneous marker of systemic disease. The glucagonoma syndrome is a rare clinical condition characterized by a distinctive cutaneous eruption associated with a glucagon-secreting islet cell neoplasm of the pancreas. A 19-year-old woman manifested typical features of this condition: a polymorphous skin eruption with characteristic distribution of lesions in perioral and paragenital regions; lesions in sites of cutaneous trauma; a skin biopsy that showed epidermal cleavage; glossitis; weight loss; mild anemia; abnormal glucose tolerance test results. plasma glucagon levels, determined by radioimmunoassay, were approximately five times normal. angiography indicated a pancreatic tumor with liver metastases. Islet cell origin was confirmed histologically. It is hoped that wider recognition of the distinctive clinical features of this syndrome will result in earlier detection and possible surgical cure of the underlying malignancy. ( info) |
12/59. Cutaneous infarction. Manifestation of disseminated mucormycosis. We describe the second reported case of cutaneous infarction as a manifestation of disseminated mucormycosis. The lesion, which closely resembled ecthyma gangrenosum, occurred in a leukemic patient who was on a regimen of broad-spectrum antibiotics. In this case as in the only other reported case, Mucor pusillus was the cause. ( info) |
13/59. Cutaneous necrotizing vasculitis complicating Takayasu's arteritis with a review of cutaneous manifestations. We report a case of a 32-year-old man with chronic active Takayasu's arteritis complicated by cutaneous necrotizing vasculitis. Symptoms of the early phase of his disease coexisted with complications of the late phase. ( info) |
14/59. antibodies to cytoplasmic antigens in lupus erythematosus. Serologic marker for systemic disease. Seven patients with classic cutaneous lupus erythematosus are described. Three of these patients had features satisfying four of the American Rheumatism association (ARA) preliminary criteria for the diagnosis of systemic lupus erythematosus (SLE). Their sera, however, lacked antinuclear antibodies but demonstrated precipitating antibodies reactive against cytoplasmic RNP (La) and non-nucleic acid (Ro) antigens. Four additional ANA-negative patients lacking significant skin disease but having a lupus-like multisystem disease were found to have antibodies to soluble cytoplasmic antigens. Thirty-three of 130 ANA-positive SLE patients, but none of 16 discoid lupus patients, possessed these anticytoplasmic antibodies. These findings suggest that antibodies to Ro and La may be a marker for systemic disease in ANA-negative patients with 1) cutaneous lupus and 2) a distinct subpopulation of patients with a lupus-like syndrome without skin disease. ( info) |
15/59. Report of a case resembling the 'fleck retina of Kandori' with ectodermal peculiarities and macula degeneration. This report involves the case of a 36-year-old woman followed-up for nine years. The symptoms include unique, sharply-defined, irregular, yellow, large flecks of the retina combined with bilateral macula degeneration. The patient's rusty-red hair, enamel dysplasia, and ashen-gray skin color were also noted. It is argued that this case is very likely identical with the 'fleck retina of Kandori' and as such, the first case reported outside japan. The literature is reviewed. ( info) |
16/59. Macular cherry-red spots and beta-galactosidase deficiency in an adult. An autopsy case with progressive cerebellar ataxia, myoclonus, thrombocytopathy, and accumulation of polysaccharide in liver. An adult patient with macular cherry-red spots, a gargoyle-like physical appearance, cerebellar ataxia, myoclonus, convulsive seizures, and pyramidal tract signs showed a profound deficiency of beta-galactosidase in liver and brain. Thrombocytopathy of undetermined etiology was evident since childhood, and the patient died of intracranial bleeding at age 22. Cerebral ganglioside pattern was normal. Hepatic mucopolysaccharides were not increased. GM1-gangliosidosis and mucopolysaccharidosis were ruled out by those analytical data. However, a large amount of amylopectin-like polysaccharide was found to be accumulated in liver. Hepatocyte contained numerous inclusion bodies with granulofibrillary structure similar to Lafora bodies, corpora amylacea, and inclusion bodies in glycogenosis type IV. This case seems to represent a new inborn metabolic disease closely related to GM1-gangliosidosis and mucopolysaccharidosis. The primary metabolic defect is not known at present. ( info) |
17/59. A cutaneous marker in the Hunter syndrome a report of four cases. A report of four patients and review of the literature suggest that a cutaneous marker exists for the Hunter syndrome. All previously described patients with this eruption have been males with clear corneas. The distinctive lesions consist of firm ivory-white papules and nodules that may coalesce to form ridges or a reticular pattern. The papules are usually seen in symmetrical areas between the angles of the scapulas and posterior axillary lines, the pectoral regions, the nape of the neck and/or on the lateral aspects of the upper arms and thighs. These lesions are seen in both allelic forms and cannot be used to separate a benign from a rapid course. ( info) |
| An infant with short-bowel syndrome developed flexural desquamation and "enamel paint" skin in pressure-bearing areas. Hyperalimentation with amino acid and lipid solution was unsuccessful in correcting the malnutrition and skin changes. ( info) |
19/59. Human essential fatty acid deficiency: treatment by topical application of linoleic acid. An essential fatty acid (EFA) deficiency developed in a 19-year-old man who was being maintained on a long-term regimen of fat-free, intravenous hyperalimentation fluids. The EFA deficiency was reversed after 21 days by daily, topical application of linoleic acid to the patient's skin. The ratio of eicosatrienoic acid (20:3, n-9) to eicosatetraenoic acid (20:4, n-6) decreased to normal levels in the skin and serum with clinical improvement of the EFA deficiency syndrome. The cutaneous manifestations (scalp dermatitis, alopecia, and depigmentation of hair) were reversed with continued, topical application of safflower oil, which contains 60% to 70% linoleic acid. ( info) |
20/59. The diagonal earlobe crease: a cutaneous manifestation of coronary artery disease. skin signs of systemic disease are of great interest to dermatologists. Reports based on coronary angiographic studies and postmortem examinations have shown that there is a higher prevalence of earlobe creases in patients with coronary heart disease. In this report seven patients with documented coronary heart disease are presented. Five of the seven patients had bilateral diagonal ear creases. The evidence to date indicates that this ear crease may be a cutaneous sign of coronary heart disease, and can therefore be used to identify patients at an increased risk for coronary heart disease. ( info) |