Vulvar melanosis and vitiligo

Clinically it is impossible to make difference between the vulvar melanosis the harmless brown spotty change of the vulva and melanoma malignum, that is one of the most dangerous malignancies. Even the dermatoscopical examination was not efficient to exclude the melanoma surely. A small, representative biopsy was enough for the diagnosis, which has exempted the patient from the unjustified mutilating resections. On the patient's skin they detected also a few relatively small disseminated hypopigmented spots. Electronmicroscopically they could observe plenty of melanosomes and big melanosoma-complexes in the lower keratinocyte layers of the vulvar melanotic macules. Melanocytes could not be recognised in the hypopigmented spots.     

Hypopigmented mycosis fungoides in a white female

A rare case of a young Caucasian female with hypopigmented mycosis fungoides is described. We reviewed and discussed the literature.     

Clear cell papulosis of the skin

Clear cell papulosis is a new entity first described in 1987. To date, six patients have been reported: all were young Taiwanese children. The disease is characterized clinically by multiple small, whitish maculopapules distributed along the milk line and by the presence of large, benign pagetoid cells in the epidermis resembling the clear cell of the nipple. The significance of this entity lies in its potential histogenetic link with Paget's disease of the skin. We report four new Taiwanese patients, three girls and one boy, aged between 21 months and 4 years. Two were sisters. Small hypopigmented macules first appeared on the pubis. They were eventually distributed bilaterally along the milk line but were most numerous in the public area. The disease may easily be overlooked when the macules are tiny or few in number and thus display no clear milk-line distribution, or when they occur in white-skinned individuals. Histologically, solitary large clear cells with large, round pale nuclei were detected in the basal layer of the hypomelaninized epidermis. The numbers of clear cells varied on haematoxylin and eosin staining and were only small in two patients. The cytoplasm of the clear cells was decorated by antikeratin AE1 and anticarcinoembryonic antigen antibodies. AE1 was the best marker of the clear cell. Some of the AE1-positive cells were tadpole-like in shape and were situated well above the basal layer. Ultrastructurally, large clumps of disintegrated or vacuolated mucin granules were present in the cytoplasm of the clear cells. The melanocytes appeared normal; the suprabasal keratinocytes were essentially devoid of melanosomes. The pathological findings in the present study support the hypothesis that these clear cells are an aberrant derivative of sweat gland cells in the epidermis and are potentially the precursor cells giving rise to mammary and extramammary Paget's disease. The differential diagnosis includes chicken pox scars, idiopathic guttate hypomelanosis, hypomelanotic tinea versicolor, anetoderma and early, hypopigmented lesions of Paget's disease.     

Effect of lead on revelation of calcium metabolic disturbance in rats treated with stannous chloride (author''s transl)

Serial observations on cutaneous sensation and on sweat response in hypopigmented flat patches of twenty-nine patients with tuberculoid and five with dimorphous leprosy on dapsone therapy were studied over 2 years. Sensation was determined by routine methods, while sweat stimulation was done by intradermal injection of 0-1 ml (0.025 mg) of carbachol (carbaminoylcholine). There was significant improvement in sensation and considerable augmentation of sweat response in the final as compared to the initial tests. The difference in sweat response was statistically significant, suggesting the value of this simple procedure in assessing the progress of leprosy.     

Increased absolute light sensitivity in Himalayan mice with cold-induced ocular pigmentation

Controversy over the relationship between ocular pigmentation and absolute dark-adapted light sensitivity has persisted for over two decades. Previous electrophysiological experiments in hypopigmented mammals (mice, rats, rabbits) show increased thresholds in the dark-adapted state proportional to the deficit in ocular melanin. Animals with the least amount of ocular melanin have the most elevated thresholds. Dark-adapted thresholds in hypopigmented mice show similar threshold elevations in behavioral tests. The present study extends these findings to show that a specific increase in ocular pigmentation results in the converse effect, lowered absolute dark-adapted thresholds. The increase in ocular melanin was accomplished by keeping Himalayan mice in the cold (4 degrees C) for 6 weeks. Himalayan mice (C57BL/6J cH/cH) were compared to black mice (C57BL/6J (+/+)) and albino mice (C57BL/6J c2J/c2J) after 6 weeks at either 4 degrees C or 20 degrees C in 12-h cycling light (<1 cd/m2). The Himalayan mice that were kept in the cold exhibited a 44% increase in ocular melanin compared to Himalayan mice kept at room temperature. Cold rearing did not effect ocular melanin or visual thresholds in control animals (black mice = 10(-5.9) cd/m2 and albino mice = 10(-4.4) cd/m2). In contrast, the Himalayan mice maintained at 4 degrees C had thresholds of 10(-5.7) cd/m2 compared to 10(-5.1) cd/m2 for Himalayan mice kept at 20 degrees C. This represents compelling evidence of a direct relationship between ocular melanin concentration and absolute dark-adapted light sensitivity.     

Comparative linkage maps for the mosquitoes (Culex pipiens and Aedes aegypti) based on common RFLP loci

OBJECTIVE: To investigate the ocular complications in pediatric bone marrow transplantation (BMT) patients. DESIGN: Cross-sectional study. PARTICIPANTS: A total of 29 pediatric BMT patients were studied. TESTING: Comprehensive ophthalmic check-up, including best-corrected visual acuity, intraocular pressure (IOP), Schirmer's test, tear breakup time, and slit-lamp and fundus examinations, was performed. MAIN OUTCOME MEASURES: Tear film instability and its related complications, IOP, cataract, and fundus lesions were measured. RESULTS: The mean age of patients was 9.1 years (range, 1.5-15 years). The mean post-BMT duration was 20.2 months (range, 3-54 months). Fifteen patients (51.7%) had tear abnormalities. Subconjunctival fibrosis was detected in two patients (6.9%). Dry and scaly skin of the eyelids was seen in one patient (3.4%). Lens opacities were found in 2 (33.3%) of 6 irradiated patients and 2 (8.7%) of 23 nonirradiated patients. Two patients (6.9%) had fundus changes, one with unilateral epiretinal membrane and the other with bilateral multiple discrete chorioretinal hypopigmented lesions in the middle to peripheral part of the retina. The overall complication rates for the anterior and posterior segments were 75.8% and 6.9%, respectively. CONCLUSION: Ocular manifestations of BMT in children are not uncommon. The most common anterior segment problem is tear dysfunction. Posterior segment complications are less common but do exist. High rate of cataract formation is reported, and this probably is the most important long-term "amblyogenic" problem in these immature eyes. Awareness and management of these problems with routine eye examination and early intervention are recommended.     

Detection of occult disease in tissue donors by routine autopsy

A particular pigmentary disorder develops on the skin of Chinese children. The lesions are composed of whitish macules and patches arranged in linear bands on the extremities unilaterally. Histologically these lesions are hypopigmented chronic dermatitis. Although the lesions are arranged in a lichen striatus pattern, they differ clinically from lichen striatus by the absence of papules and plaques. These cases may represent a new entity. The differential diagnosis includes lichen striatus, vitiligo, piebaldism, nevus depigmentosus, and hypomelanosis of Ito. The differences between the disorder presented here and those of different diagnosis are discussed.     

Transplanted and repopulated retinal pigment epithelial cells on damaged Bruch's membrane in rabbits

AIMS: The authors studied how artificially damaged Bruch's membrane influenced growth and differentiation of transplanted embryonic retinal pigment epithelial (RPE) cells and of host RPE cells in rabbits. METHODS: Embryonic RPE cells obtained from pigmented rabbits were transplanted into the subretinal space of adult albino rabbits. The host RPE was removed with a silicone cannula, and Bruch's membrane was damaged by scratching with a microhooked 27 gauge needle under the detached retina in closed vitrectomy. The transplantation sites were examined 3, 7, and 14 days after surgery by light and electron microscopy. RESULTS: Varying degrees of damage in Bruch's membrane were observed. Pigmented and hypopigmented RPE cells showed a normal polarity and tight junctions were seen at the sites of mild to moderate damage 3-7 days after the surgery. In contrast, fibroblast-like cells with no such features of RPE cells formed multiple layers at the sites of severe damage involving the full thickness of Bruch's membrane and the choriocapillaris even 14 days after the surgery. Without transplantation, host RPE cells repopulated the damaged areas in the same way as transplanted RPE cells. CONCLUSIONS: Transplanted embryonic RPE cells as well as host RPE cells grew and differentiated on the moderately damaged Bruch's membrane, while the severely damaged Bruch's membrane did not allow differentiation of RPE cells although these cells could grow and cover the damaged areas.     

Lupus vulgaris complicated by metastatic squamous cell carcinoma

A 47-year-old Bedouin man presented with an ulcerated nodule of several months' duration on the nape of the neck. The nodule developed on an asymptomatic, slowly growing plaque which appeared during childhood. Physical examination revealed two erythematous plaques covering the posterior and right lateral aspects of the neck. The border of the plaques was soft, circinate, with a reddish-brown color, while the center was slightly erythematous and atrophic. An ulcerated nodule measuring 2 cm was seen on one of the plaques. Physical examination was unremarkable with no lymphadenopathy. Laboratory tests, including complete blood cell count, erythrocyte sedimentation rate (ESR), and routine chemistry tests, were all within normal limits. Chest X-ray showed a small calcified perihilar lymph node. The Mantoux test was positive with erythema and induration of 15 mm after 48 h. Biopsy from a plaque showed extensive diffuse granulomatous infiltration throughout the dermis with epithelioid and Langhans giant cells surrounded by mononuclear inflammatory cells. No caseation necrosis was present. Ziehl-Neelsen, periodic acid-Schiff (PAS), and Giemsa stains were negative. Polymerase chain reaction (PCR) for the detection of Mycobacterium tuberculosis and atypical mycobacteria from a skin sample was also negative. Fresh tissue cultures yielded M. tuberculosis after 6 weeks. A biopsy specimen from the ulcerated nodule demonstrated islands of atypical malignant squamous cells invading the dermis, which were compatible with moderately differentiated squamous cell carcinoma (SCC). The ulcerated nodule was completely excised, and treatment for tuberculosis was initiated with a combination of isoniazid, rifampicin, and pyrazinamide. Within 3 months of therapy, the patient's lesions resolved, leaving only slightly atrophic hypopigmented scars. A month after the treatment's initiation, an enlarged cervical lymph node was noted showing metastatic SCC on histologic examination. The patient underwent neck dissection and radiation therapy without evidence of any further metastases.     

Human pigmentation genetics: the difference is only skin deep

There is no doubt that visual impressions of body form and color are important in the interactions within and between human communities. Remarkably, it is the levels of just one chemically inert and stable visual pigment known as melanin that is responsible for producing all shades of humankind. Major human genes involved in its formation have been identified largely using a comparative genomics approach and through the molecular analysis of the pigmentary process that occurs within the melanocyte. Three classes of genes have been examined for their contribution to normal human color variation through the production of hypopigmented phenotypes or by genetic association with skin type and hair color. The MSH cell surface receptor and the melanosomal P-protein are the two most obvious candidate genes influencing variation in pigmentation phenotype, and may do so by regulating the levels and activities of the melanogenic enzymes tyrosinase, TRP-1 and TRP-2.     

Inhibition of fibrinogen binding and surface recruitment of GpIIb/IIIa as dose-dependent effects of the RGD-mimetic MK-852

Mycobacterium simiae was the third most common mycobacterium identified over a 2-year period from a single clinical laboratory in southern Arizona. Thirty-three isolates from 25 patients were identified over 1 year. The isolation of M. simiae was considered clinically significant for only two of 23 evaluable patients. None of five patients with human immunodeficiency virus infection had clinical disease associated with M. simiae. Twenty isolates were available for detailed study. All but one of the 20 isolates were niacin-negative, and 11 were nonphotochromogenic. All 20 isolates had a triple-cluster pattern consistent with M. simiae by high-performance liquid chromatography, and restriction fragment patterns were identical for 16 isolates. Analysis of 16S rDNA confirmed the identity of all the tested isolates as M. simiae. In this study, M. simiae was a frequent clinical isolate but was rarely associated with disease. The organisms isolated were confirmed to be M. simiae but appeared to be phenotypically distinct strains of low virulence.     

Differential effects of preterm birth and small gestational age on cognitive and motor development

AIMS: To determine the differential effects of preterm birth and being small for gestational age on the cognitive and motor ability of the child. METHODS: A longitudinal cohort of all infants of gestational age < or = 32 weeks born to mothers resident in the counties of Cheshire and Merseyside in 1980-1 was studied. The children were assessed at the age of 8 to 9 years using the Wechsler Intelligence Scale for Children, the Neale analysis of reading ability, and the Stott-Moyes-Henderson test of motor impairment. Adequacy of fetal growth was determined by the birthweight ratio--that is, the ratio of the observed birthweight to the expected birthweight for a given gestational age. Children with clinically diagnosed motor, learning or sensory disabilities were excluded. Information on social variables was obtained by a questionnaire completed by the parents. Of the 182 children, 158 were assessed. RESULTS: IQ was positively correlated with birthweight ratio but not with birthweight or gestational age. Motor ability was associated with birthweight, gestational age, and birthweight ratio. Reading comprehension was associated with birthweight ratio, but reading rate and accuracy were best explained by social variables and sex. IQ remained associated with birthweight ratio, after adjusting for maternal education, housing status, and number of social service benefits received. Reading ability was related to these social variables but motor ability was not. CONCLUSIONS: The effects of SGA and preterm birth differed: SGA was associated with cognitive ability, as measured by IQ and reading comprehension; motor ability was additionally associated with preterm birth. Reading rate and accuracy were not associated with SGA or preterm birth but were socially determined.     

Adult T cell leukemia/lymphoma effectively treated with chronic oral etoposide

A 52-year-old man, who complained of tarry stool and systemic lymphadenopathy, was admitted to our hospital on July 2, 1992. Biopsy showed diffuse large cell lymphoma. Leukocytosis with atypical lymphocytes was not shown in the peripheral blood, but there was an elevated serum LDH level. The man was found to have both HTLV-I antibody and the monoclonal integration of proviral DNA in malignant lymph node cells obtained at biopsy. The diagnosis was lymphoma-type adult T-cell leukemia (ATLL). The chemotherapy regimens of MI-FP, CHOP and modified DHAP were used for the treatment, but were not effective. So, he was treated with etoposide 75 mg orally for 25 days (chronic oral etoposide therapy) and achieved partial remission. This chemotherapy induced myelosuppression with neutropenia, but there was no documented infection. Chronic oral etoposide therapy is an effective regimen for patients with relapse or refractory lymphoma.     

Mutation induction by heavy ion beams and X-rays: analysis with cultured cell line

This study was performed to determine the biological effects of heavy ion beams on cultured cells. V79 cells were irradiated with carbon or neon beams or X-rays, and cell survivals was calculated by the colony assay method. The Do values for 150 kVp X-rays, 20 keV/micron and 80 keV/micron carbon beams, and 80 keV/micron neon beams were 2.2Gy, 1.8Gy, 1.0Gy and 1.4Gy, respectively. After 7-10 day expression periods, the mutation frequencies at the hypoxanthine-guanine phosphribosyl transferase (hprt) locus were analyzed from the numbers of colonies formed in media supplemented with 6-thioguanine. An extremely higher frequency of mutation was observed with heavy ion beams compared with X-rays. Both cell killing effect and the mutation induction were enhanced when the LET of carbon beams was increased from 20 to 80 keV/micron. These results mean that carbon beams had a stronger cell-killing effect than X-rays, but also carried a high risk of mutation induction. On the other hand, neon beams yielded cell survival curves similar to those of carbon beams, but they had a smaller mutation induction effect than carbon beams. The effect of fractionated irradiation (3 hr interval) on cell survival and mutation frequency were also examined. When cells were irradiated with X-rays, cell survival was increased by fractionation, but the mutation frequency was not modified. Irradiating cells with fractionated carbon beams, survival curves were not affected, but mutation frequency was reduced.     

Methanogenium frigidum sp. nov., a psychrophilic, H2-using methanogen from Ace Lake, Antarctica

Methanogenium frigidum sp. nov. was isolated from the perennially cold, anoxic hypolimnion of Ace Lake in the Vesfold Hills of Antarctica. The cells were psychrophilic, exhibiting most rapid growth at 15 degrees C and no growth at temperatures above 18 to 20 degrees C. The cells were irregular, nonmotile coccoids (diameter, 1.2 to 2.5 microns) that occurred singly and grew by CO2 reduction by using H2 as a reductant. Formate could replace H2, but growth was slower. Acetate, methanol, and trimethylamine were not catabolized. Cells grew with acetate as the only organic compounds in the culture medium, but growth was much faster in medium also supplemented with peptones and yeast extract. The cells were slightly halophilic; good growth occurred in medium supplemented with 350 to 600 mM Na+, but no growth occurred with 100 or 850 mM Na+. The pH range for growth was 6.5 to 7.9; no growth occurred at pH 6.0 or 8.5. Growth was slow (maximum specific growth rate, 0.24 day-1; doubling time, 2.9 days). This is the first report of a psychrophilic methanogen growing by CO2 reduction.     

The effect of intravenous and oral salbutamol on fetus and mother in premature labour

Salbutamol was administered intravenously to 14 patients in premature labour. Inhibition of uterine contractions occurred on 22 out of 23 occasions. The mean increase in maternal and fetal heart rate and blood pressure were within clinically acceptable limits but there were wide individual variations. The mean delay achieved when the intravenous regime was supplemented with oral salbutamol was greater than that achieved by intravenous salbutamol alone, but the difference was not statistically significant.     

Coping With Distress by Eating or Drinking: Role of Trait Urgency and Expectancies.

The authors propose that trait urgency (the tendency to act rashly when distressed) is a risk factor for both alcohol abuse and bulimic symptoms, that disorder-specific expectancies influence whether one engages in one behavior or the other, and that expectancies moderate urgency's influence on those behaviors. Cross-sectional findings were consistent with the model. Problems from alcohol use were comorbid with binge eating and purging. Trait urgency was associated with both behaviors. Alcohol expectancies were associated with drinking levels and with problem drinking, but not with eating. Eating expectancies were associated with binge eating, but not with alcohol use or problems. Urgency's effect on binge eating was moderated by expectancies, but its effect on alcohol use and problem drinking was not. (PsycINFO Database Record (c) 2010 APA, all rights reserved)     

GDP-L-fucose pyrophosphorylase. Purification, cDNA cloning, and properties of the enzyme

The enzyme that catalyzes the formation of GDP-L-fucose from GTP and beta-L-fucose-1-phosphate (i.e. GDP-beta-L-fucose pyrophosphorylase, GFPP) was purified about 560-fold from the cytosolic fraction of pig kidney. At this stage, there were still a number of protein bands on SDS gels, but only the 61-kDa band became specifically labeled with the photoaffinity substrate, azido-GDP-L-[32P]fucose. Several peptides from this 61-kDa band were sequenced and these sequences were used for cloning the gene. The cDNA clone yielded high levels of GFPP activity when expressed in myeloma cells and in a baculovirus system, demonstrating that the 61-kDa band is the authentic GFPP. The porcine tissue with highest specific activity for GFPP was kidney, with lung, liver, and pancreas being somewhat lower. GFPP was also found in Chinese hamster ovary, but not Madin-Darby canine kidney cells. Northern analysis showed the mRNA in human spleen, prostate, testis, ovary, small intestine, and colon. GFPP was stable at 4 (o)C in buffer containing 50 mM sucrose, with little loss of activity over a 9-day period. GTP was the best nucleoside triphosphate substrate but significant activity was also observed with ITP and to a lesser extent with ATP. The enzyme was reasonably specific for beta-L-fucose-1-P, but could also utilize alpha-D-arabinose-1-P to produce GDP-alpha-D-arabinose. The product of the reaction with GTP and alpha-L-fucose-1-P was characterized as GDP-beta-L-fucose by a variety of chemical and chromatographic methods.     

Cytochalasin B-induced triploidy in mouse oocytes fertilized in vitro

Mouse eggs and spermatozoa were treated in various ways with 5 or 10 mug cytochalasin B/ml. The fertilization rate in vitro was reduced by treatment with the drug but 80-90% of the eggs fertilized were triploid. Many of the experimental eggs were penetrated by one or more spermatozoa but remained unfertilized (75% compared with 9% in control eggs). It is suggested that cytochalasin B weakens the zona reaction and interferes with fusion of gametes but does not prevent the block to polyspermy.