Sphenoid sinus metastasis displacing the cavernous sinus.
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Biomedical subjects
Publications and source records attributed to J A Kenney.
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Vitiligo is a disease that affects from 1 to 3 per cent of the population. It is characterized by cutaneous white macules that often develop in cosmetically important areas such as the face, the dorsal hands, and the arms. It may be accompanied by ocular abnormalities and a number of associated disorders such as thyroid disease, diabetes, pernicious anemia, and alopecia areata. There is increasing evidence that vitiligo is an autoimmune disease. Although there is as yet no definitive cure, many patients have obtained respectable repigmentation by the use of topical or oral psoralen plus ultraviolet light. When large areas of skin are involved or when the patient is unresponsive to therapy, serious consideration should be given to depigmentation with monobenzone (Benoquin).
Eighty-two children (ages 6 months to 12 years) with clinical and/or histopathologic diagnoses of vitiligo were evaluated; 35 were male and 47, female. Fifty-six were black, 25 white, and 3 classified as "other." Children were compared with control groups of children with other skin diseases and with adults with vitiligo. Children had an increased incidence of segmental vitiligo (p less than 0.01). Children had an increased incidence of autoimmune and/or endocrine disease and also of premature graying in their immediate and extended family members (p less than 0.001). Six of 33 children with vitiligo tested had positive organ-specific serum autoantibodies, which was a higher incidence than in the control group of children (p less than 0.05). Eighteen percent of children treated with topical psoralens and long-wave ultraviolet light (PUVA) therapy had an acceptable response, which was less than an adult group similarly treated. We have found childhood vitiligo to be a distinct subset of vitiligo, showing increased segmental presentation; strong autoimmune and/or endocrine disease background and high incidence of premature graying in the families of affected children; the presence of organ-specific serum autoantibodies and a poor response to topical PUVA therapy.
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Twenty-five patients with vitiligo and twenty-five healthy control subjects were evaluated with the use of flow cytometry to compare percentages of peripheral T lymphocytes and natural killer cells. The percentages of total T lymphocytes, helper T cells, suppressor T cells, and natural killer cells were evaluated with the use of OKT3, OKT4, OKT8, and Leu-7 monoclonal antibodies, respectively. Mean total T lymphocytes and helper T cells were markedly depressed; mean natural killer cells were markedly elevated and mean suppressor T cells were moderately elevated in patients with vitiligo in comparison with control subjects. These results indicate that cell-mediated immunity is subject to some defect in regulation in patients with vitiligo. It remains to be determined whether these abnormalities are a direct cause or a result of vitiligo. Antibody-dependent cytotoxicity, utilizing killer cells with recently reported antimelanocyte antibodies found in patients, may be responsible for pigment cell destruction in vitiligo. Helper T cells may be reduced because of low levels or faulty production of T lymphocyte-stimulating factors in patients or because of a serum factor in patients that is toxic to helper T cells. The presence or absence of autoimmune and/or endocrine disease in patients with vitiligo had no effect on lymphocyte populations. There seemed to be a trend toward lower levels of helper T cells in patients having vitiligo for the shortest amount of time. In summary, the data indicate immunologic abnormalities in patients with vitiligo.
One hundred fifty-six patients with vitiligo were examined for ocular abnormalities. A 2:3 ratio of white to black patients allowed us to evaluate the role of race in the occurrence of ocular disturbances. A large percentage (40%) of all patients showed some degree of fundal pigment disturbance including pigment clumps, focal hypopigmented spots, and choroidal nevi. Although racial variations were found in the incidence of choroidal nevi (p = 0.001) and iris transillumination (p = 0.0012), these variations were believed to reflect normal differences found in patients without vitiligo.
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8-Methoxypsoralen (8-MOP) levels in the blood of vitiligo patients were determined through the use of a reverse-phase high-performance liquid chromatographic method. The overall recovery of the internal standards was 85-94%, with the lower detection limit of 8-MOP at 2 ng. Peak blood levels as low as 130 ng/ml and as high as 3892 ng/ml were obtained in patients at 1-3 h following the oral administration of 0.6 mg/kg body weight of Oxsoralen capsules (Elder Pharmaceuticals Co.). These results are consistent with the clinical observation that maximum response in phototherapy is obtained at about 2 h after oral administration of the drug. Two hours after oral administration of 0.6 mg/kg of Oxsoralen, 8-MOP levels in the epidermis, dermis, and whole skin of the guinea pig (in ng/g) were: epidermis, 330 +/- 20; dermis, 89 +/- 16; whole skin, 379 +/- 19. Also detected were 8-MOP levels of 441 +/- 22 ng/ml in aqueous humor, 166 +/- 18 ng/ml in vitreous gel, 355 +/- 15 ng/g in lens, and 410 +/- 26 ng/g in retina. These results point to the fact that the eyes of the patient must be protected from exposure to sunlight after psoralen UV treatment, and that 8-MOP is absorbed in blood unevenly and varies from patient to patient. The fact that only 50-60% of the patients responded to psoralen photochemotherapy for vitiligo may be related to the variation of absorption of the drug in individual patients.
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A rapid and sensitive method is described for determining 8-methoxypsoralen in plasma. Plasma samples are acidified with 6 mol/liter and heated in a boiling water bath to release the plasma-bound drug nondestructively. It then is extracted into a solvent mixture consisting of benzene/ethyl acetate (9/1 by vol). The solvent phase is separated, evaporated, and an aliquot of the dissolved residue is thin-layer chromatographed, with benzene/ethyl acetete (9/1) as developing solvent. The plate is dried and the spots, made visible under ultraviolet light (320-400 nm), are scanned. The smallest amount detectable is 20 ng; the overall analytical recovery from plasma is 84%. We used the method to determine the drug in the plasma of rabbits after intravenous and oral administration of 10 mg, and in one patient after an oral dose of 30 mg.
Some skin disorders are more common in blacks than in whites or are so rare in whites as to be almost peculiar to blacks. Pigmentary abnormalities, either dark or light spots, often occur in association with inflammatory skin diseases. Curvature of the hair follicle is thought to be at the root of two other painful conditions, pseudofolliculitis of the beard and keloidal folliculitis. Certain hairstyles and treatments are damaging and can even cause alopecia if continued.
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