Papers by Gilles Lestringant
Dermatology, 2001
We report an unusual case of primary cutaneous nocardiosis due to Nocardia otitidiscaviarum prese... more We report an unusual case of primary cutaneous nocardiosis due to Nocardia otitidiscaviarum presenting first as a mycetoma of the right hand and wrist. The patient refused treatment and was lost to follow-up until he showed up 10 years later with numerous discharging large sinuses and abscesses on the upper right quadrant of the chest wall and in the right armpit. Roentgenograms revealed pleural masses. Histology was in keeping with the diagnosis of mycetoma. Treatment with amikacin, rifampicin and co-trimaxole proved to be successful.
International Journal of Dermatology, Sep 1, 1991
Correspondence 675 other restricted beverages such as coffee), and the bullae rapidly returned. H... more Correspondence 675 other restricted beverages such as coffee), and the bullae rapidly returned. He was once again asked to modify his beverage consumption along the previous guidelines, and the condition resolved. Comment This is the first reported case of pseudoporphyria related to diet. It was not possible to ascertain which ingredient in the Coca Cola® precipitated his blistering. The clinical differential diagnoses include porphyria, epidermolysis bullosa acquisita, and pemphigoid. Both epidermolysis bullosa acquisita and pemphigoid usually have positive basement membrane zone immunofluorescence, and in any case the electron microscopic study is inconsistent with these disorders but typical of porphyria or pseudoporphyria. The nonnal porphyrin metabolism leaves one with a diagnosis of pseudoporphyria, and the dietary cause is suspected in the absence of other provoking factors and is verified by rechallenging with the suspect dietary item.
International Journal of Dermatology, 1988
A 16-year-old appearing man BEDU (bone age 19) born in a remote village of the United Arab Emirat... more A 16-year-old appearing man BEDU (bone age 19) born in a remote village of the United Arab Emirates presented with a painful, patchy keratoderma plantaris. He could barely walk and was crying out with pain. The hyperkeratosis was limited to weight-bearing areas, involving also the tips of the toes. Strong pressure applied on free skin area was without response, whereas a light touch of a patch triggered a defense reflex of the whole limb and screams. The lesions had started approximately 5 years before with a single patch on the right sole, then progressively involved both sides. There was a permanent tenderness with paroxysms, and the pain was more acute the last 6 months. Hyperhidrosis was present, giving a maceration of the keratosis jn places. It is interesting to note that this young man has a patch of hyperkeratosis on the outer region of both ankles. This is a callosity widely found in this country, due to Muslim prayer and sitting on one's feet. These patches were not tender. The palms were unaffected, but there was a painful hyperkeratotic patch on the distal region of the nail beds of both thumbs. There was no evidence of ocular lesions, even after specialized examination, and apparently no history of red eyes, tearing, or photophobia. The patient is not frankly mentally retarded. He cannot read or write but has been attending a school for adults the last 3 months. It seems, however, that he was overreacting to pain. Both his hands are bizarre, with shortened phalanges and swollen joints. Roentgenograms showed no bone lesions. The patient was put on etretinate 50 mg daily (patient weight is 55 kg). Three weeks later, he was much improved and walking normally. The hyperkeratotic skin was peeling off. At that time, tyrosinemia type II was suspected. Tyrosine blood level, obtained while the patient was on etretinate, was 127 Mmol/dl. (N'' up to 8.7). (Bioscientia, Mains, West Germany). The patient failed subsequent appointments but came back 2 months later in acute crisis. On admission, tyrosine blood level was 146 fimo\/d\. Urine showed massive excretion of parahydroxyphenyllactic acid and of parahydroxybenzaldehyde, a degradation product of parahydroxyphenylpyruvic acid. There was also an elevated amount of parahydroxyphenylacetic acid. All
PubMed, Feb 1, 1999
The aim of this study is to evaluate the prevalence of dermo-respiratory symptoms from lung-funct... more The aim of this study is to evaluate the prevalence of dermo-respiratory symptoms from lung-function spirometer values and serum IgE levels in farmers who have been exposed to pesticides in the United Arab Emirates (E.A.U.). The study was a comparison of 98 farmers with 98 non-farmers, the two groups were equivalent in age, sex and selected regions--the town of Al-Ain and in the Emirates of Dubai, Sharjah and Fujairah. Most of the farmers were illiterate or poorly educated (p < 0.0001). Most lived in prefabricated houses (50.5%). Use of chemical substances (70.9%) and a mixture of pesticides by the farmers (60.2%) was very high. The results revealed that the farmers had a very high prevalence of chronic dermo-respiratory symptoms. These differences are statistically significant when compared with the reference group, particularly for the following symptoms: cough (p < 0.003), pharyngitis (p < 0.0003), bronchitis (p < 0.02), asthma (p < 0.008), respiratory insufficiency (p < 0.006), pneumonia (p < 0.003), dyspnea (p < 0.006), nasal catarrh (p < 0.001), sinusitis (p < 0.05), pharyngeal irritation (p < 0.01), nasal irritation (dryness, sneezing and secretions) (p < 0.005), ocular irritation (p < 0.05), cutaneous pruritus (p < 0.002), and contact dermatitis (p < 0.02). The spirometer tests (all parameters together) measured in the farmers were significantly reduced by comparison with the reference group. In conclusion, the authors suggest that a high prevalence of dermo-respiratory symptoms, supported by reduced spirometer tests and high serum IgE, is associated with exposure to pesticides. These results are clearly useful for establishment of work-related reduced exposure in the future.
Clinical Genetics, Jun 28, 2008
With the aim of identifying putative quantitative trait loci (QTLs) involved in the regulation of... more With the aim of identifying putative quantitative trait loci (QTLs) involved in the regulation of blood pressure, we have carried out association studies at a candidate genetic locus-a human pancreatic phospholipase A2 (PLA2) gene localized on chromosome 12. Positive associations were found between the presence of a Taq I dimorphic site localized in the first intron of this gene and hypertension in three sample populations (two from USA and one from Germany). These results indicate that a QTL implicated in determining an individual&#39;s genetic susceptibility to hypertension could be present within up to 30 cM of this human PLA2 gene.
PubMed, Oct 1, 1999
Background: In the past 25 years the United Arab Emirates (UAE) have experienced a socioeconomic ... more Background: In the past 25 years the United Arab Emirates (UAE) have experienced a socioeconomic boom. The once nomadic Bedouin population of Al Ain, in the emirate of Abu Dhabi, now lives in modern air-conditioned accommodation, and huge desalination plants have allowed afforestation and farming. Objective: To evidence responsible airborne allergens in an UAE population. Patients and methods: 263 UAE Nationals who attended Tawam Hospital (Al Ain, UAE) with a respiratory disease suspected of being of allergic origin, were submitted to SPT and RAST. The choice of pollinic allergens was made in accordance with the local flora and market availability. All patients were SPTed with at least the same battery of 15 pollinic and indoor allergens. Most patients were submitted to at least 4 RAST, viz Cynodon dactylon, Salsola kali, Prosopis juliflora and Dermatophagoides pteronyssinus. Results: 71.8% patients were positive for at least one allergen. Pollen accounted for 61.6% of positive patients, with 45.2% positive to chenopodiaceae, 33% to gramineae and 23.5% to P. juliflora. Indoor allergens were positive in 30.4% of patients with 17.9% positive to D. pteronyssinus and D. farinae, 11% to Blatella germanica, 8.3% to Cat fur, 4.9% to Goat hair, 0.7% to Rat hair and Mouse hair and 1.5% to Candida albicans. Conclusion: Pollen was the prominent allergen. There is room, however, for further epidemiological studies possibly with new extracts and RAST specifically designed after the species of the Gulf region.
Clinical Genetics, Jun 28, 2008
We have studied an insertion/deletion dimorphism in the human angiotensin‐converting enzyme gene ... more We have studied an insertion/deletion dimorphism in the human angiotensin‐converting enzyme gene amongst UAE nationals from the Abu Dhabi Emirate. Our findings show lack of association between the I/D allele marker system and clinical diagnosis of essential hypertension, suggesting that variations of the angiotensin‐converting enzyme gene do not play a major role in the determination of elevated blood pressure in this Arab population. This agrees with results reported on other ethnic groups.
Hypertension, Apr 1, 1999
We designed an association (retrospective, case control) study aimed at evaluating associations b... more We designed an association (retrospective, case control) study aimed at evaluating associations between genetic variations of the human apolipoprotein B (apoB) gene and clinical diagnosis of essential hypertension. Our approach was to compare the distribution of the alleles of a highly polymorphic variable number of tandem repeats localized 3Ј to the human apoB gene, the apoB 3Ј hypervariable region (HVR), in a group of normotensive and a group of hypertensive individuals. We collected DNA samples from 437 unrelated nationals (215 normotensives and 222 hypertensives) from the United Arab Emirates (UAEs), and we determined their apoB 3Ј HVR allele and genotype status with a polymerase chain reaction-based assay. In the UAE population, we found 18 alleles underlying a total of 51 genotypes. The distribution of these alleles was significantly different between normotensive and hypertensive UAE nationals. The main peak of the distributions occurred at 35 repeats among hypertensives (with a relative frequency of 25.7% versus 19.6% in normotensives) and at 37 repeats among normotensives (28.8% versus 20.3% in hypertensives). Alleles with 21, 23, 25, 49, and 55 repeats were found in hypertensives only (with a combined relative frequency of 7.6%). We conclude that variations of the apoB gene, or of a nearby gene, that may be in linkage disequilibrium with these alleles play a role in the development of essential hypertension in the UAEs.
American journal of medical genetics, Jan 13, 1998
Follicular atrophoderma is a rare anomaly observed mainly in the X-dominant form of chondrodyspla... more Follicular atrophoderma is a rare anomaly observed mainly in the X-dominant form of chondrodysplasia punctata (Conradi-Hünermann-Happle syndrome) and in the X-linked dominant Bazex syndrome. We report on five Emirati sibs (three girls and two boys), 4-18 years old, with normal stature, diffuse congenital ichthyosis, patchy follicular atrophoderma, generalized and diffuse non-scarring hypotrichosis, and marked hypohidrosis. Steroid sulfatase activity, assessed in the two boys, was found to be normal. Electron microscopic studies of ichthyotic skin did not show any specific abnormality. The association of congenital diffuse ichthyosis with follicular atrophoderma and hypotrichosis has not been reported before. The patients were reminiscent of Bazex syndrome; however, ichthyosis is not a component of Bazex syndrome. We conclude that this syndrome of congenital ichthyosis with follicular atrophoderma represents a new autosomal recessive genodermatosis.
Archives of Dermatology, Aug 1, 1992
... Gilles G. Lestringant, MD; Samir R. Akel, MD, FRCSE; Khalil I. Qayed, MD ... From the Departm... more ... Gilles G. Lestringant, MD; Samir R. Akel, MD, FRCSE; Khalil I. Qayed, MD ... From the Departments of Dermatology (Drs Lestringant and Qayed) and Pediatric Surgery (Dr Akel), Tawam Hospital, Ministry of Health, Al Ain, Abu Dhabi, United Arab Emirates. ...
Clinical Genetics, Dec 1, 1999
The renin (REN) gene is a good candidate that could underlie an individual's genetic susceptibili... more The renin (REN) gene is a good candidate that could underlie an individual's genetic susceptibility to human essential hypertension (EHT). We describe here a polymerase chain reaction-based assay for detection of a BglI dimorphic site located in the first intron of the REN gene. In this retrospective, case-control, association study, we investigated BglI allele and genotype distributions in 554 subjects (280 hypertensives and 274 normotensives) from the United Arab Emirates (UAE)-a genetically homogeneous ethnic population with no history of smoking or alcohol consumption-and in 485 hypercholesterolemic, US Caucasian subjects (250 hypertensives and 235 normotensives). A statistically significant association was found between alleles on which the BglI site is present [BglI(+)] and clinical diagnosis of EHT in the UAE sample group (odds ratio = 2.69, p = 0.0006), and a similar trend was observed in the US group (odds ratio = 1.97, p = 0.01). BglI(+) homozygous status was also investigated in the US group and found to be associated with elevated systolic and diastolic blood pressure values (respectively, 144.8 9 26.1 vs. 134.1 9 23.0 mmHg, p = 0.04; and 91.0 9 12.5 vs. 82.2 9 12.7 mmHg, p = 0.009). In conclusion, variations of the REN (or of a nearby) gene that may be in linkage disequilibrium with the REN BglI(+) marker could play a role in contributing to an increased individual's genetic susceptibility to EHT in the UAE population and amongst US hypercholesterolemic Caucasians. Such a genetic influence, which seems to show a recessive mode of inheritance, could also be implicated in raising both systolic and diastolic blood pressures.
American Journal of Human Genetics, Jul 1, 1997
Journal of Investigative Dermatology, 1997
Archives of Dermatology, Jun 1, 1989
Seven members from three generations of a Saudi family presented with porokeratosis punctata palm... more Seven members from three generations of a Saudi family presented with porokeratosis punctata palmaris et plantaris (PPPP). In our series of patients, the disorder began in their early 20s, seemed to be transmitted as a dominant trait, and affected males only. The elementary lesion was a tiny keratotic spine, and the involvement was strictly limited to the volar aspects of the hands and to the soles of the feet. Histological studies showed a columnar parakeratosis that resembled the cornoid lamella of porokeratosis, but other clinical and histological traits tended to make PPPP an entity that was distinct from true porokeratosis. There have been only six reports in the literature on genuine PPPP before this series.
International journal of gynaecology and obstetrics, Nov 30, 2000
Objective: To determine the association of Acanthosis nigricans, hyperinsulinemia, and hormonal l... more Objective: To determine the association of Acanthosis nigricans, hyperinsulinemia, and hormonal levels in female subjects from the United Arab Emirates (UAE). Design: Prospective study. Setting: Tawam Teaching Hospital of Faculty of Medicine &amp;amp;amp;amp; Health Sciences. Subjects: Ninety-two females (age range 16–65 years) were recruited. Methods: Height, weight, and sitting blood pressure were recorded on 92 female subjects with A. nigricans.
Journal of Pediatric Hematology Oncology, Aug 1, 2004
Xeroderma pigmentosum (XP) is a rare autosomal recessive disease characterized by marked sensitiv... more Xeroderma pigmentosum (XP) is a rare autosomal recessive disease characterized by marked sensitivity to ultraviolet radiation that leads to the development of multiple skin malignancies. The authors describe four XP siblings in a consanguineous Pakistani family. The first patient was a boy who died at age 2 years. The second and third siblings were girls who died at age 2 and 7 years, respectively. The fourth sibling, the propositus, was a boy diagnosed with XP at age 7 years. He developed three different types of malignancies simultaneously and died at age 13. The authors conclude that it is important to be aware of multiple malignancies of different types in the same patient with XP.
British Journal of Dermatology, Mar 1, 2003
Background Congenital atrichias represent a complex and heterogeneous group of genodermatoses, wh... more Background Congenital atrichias represent a complex and heterogeneous group of genodermatoses, which have been shown in several consanguineous families to result from homozygous mutations in the hairless gene (HR). Objectives To identify the molecular basis of congenital atrichia in a non-consanguineous family. Methods Genetic analysis was carried out in a two-generation family with two children with congenital atrichia and one healthy child. Results We established a diagnosis of atrichia with papular lesions based on clinical and histopathological data. We identified a heterozygous 11-bp deletion (189-199del) in the two affected children and their mother. In addition, the two affected children and their father were shown to carry a non sense mutation (Q478X), which has previously been described in a Pakistani family. Haplotype analysis revealed that mutation Q478X occurred independently in the two families. Conclusions We have identified the first case of compound heterozygosity for mutations in HR as well as the first instance of a recurrent mutation in this gene. These data further expand our understanding of the molecular pathomechanisms underlying congenital atrichias.
Dermatology, 1997
The spectrum of Dowling-Degos disease-reticulate acropigmentation of Kitamura (DDD-RAK) is a grou... more The spectrum of Dowling-Degos disease-reticulate acropigmentation of Kitamura (DDD-RAK) is a group of rare autosomal dominant disorders that have in common a unique histological picture of hyperpigmented digitate epidermal &#39;downgrowths&#39;. Patients with the DDD-RAK spectrum may show hyperpigmented macules and papules, facial pits, breaks in dermatoglyphics and epidermoid cysts. We examined 5 unrelated patients, 3 females and 2 males (age range 22-35 years), who presented with clinical and histological features of the DDD-RAK spectrum. In addition, the patients presented with hypo- or depigmented macules and papules. Histopathology of the lesions revealed features that were identical to DDD-RAK; there were, however, diminution or absence of pigmentation. Family histories for pigmented lesions and leukoderma were positive in all patients and consistent with autosomal dominant modes of inheritance. These 5 cases, together with isolated reports in the literature of achromic lesions with histological features of DDD-RAK, point to the hypothesis that achromic macules and papules may be a feature of the DDD-RAK spectrum.
Archives of Dermatology, Jun 1, 1989
Seven members from three generations of a Saudi family presented with porokeratosis punctata palm... more Seven members from three generations of a Saudi family presented with porokeratosis punctata palmaris et plantaris (PPPP). In our series of patients, the disorder began in their early 20s, seemed to be transmitted as a dominant trait, and affected males only. The elementary lesion was a tiny keratotic spine, and the involvement was strictly limited to the volar aspects of the hands and to the soles of the feet. Histological studies showed a columnar parakeratosis that resembled the cornoid lamella of porokeratosis, but other clinical and histological traits tended to make PPPP an entity that was distinct from true porokeratosis. There have been only six reports in the literature on genuine PPPP before this series.
Archives of Dermatology, Aug 1, 1992
... Gilles G. Lestringant, MD; Samir R. Akel, MD, FRCSE; Khalil I. Qayed, MD ... From the Departm... more ... Gilles G. Lestringant, MD; Samir R. Akel, MD, FRCSE; Khalil I. Qayed, MD ... From the Departments of Dermatology (Drs Lestringant and Qayed) and Pediatric Surgery (Dr Akel), Tawam Hospital, Ministry of Health, Al Ain, Abu Dhabi, United Arab Emirates. ...
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Papers by Gilles Lestringant