[Sexually transmitted diseases: comparative study between the years 1960 and 1990 in the].
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Adolescent↗
Biomedical subjects
H Lakhdar
Publications and source records attributed to H Lakhdar.
[Sunscreens: use in pregnant women at Casablanca].
BACKGROUND: Photoprotection with sunscreens is recommended in hyperpigmentation, particularly in pregnancy-related form. The aim of this study was to evaluate the use of sunscreens in pregnant women and to determine whether melasma was correlated to sunscreen use. PATIENTS AND METHODS: Four hundred and fifteen pregnant women were surveyed by a standardized questionnaire on the presence of melasma and their use of sunscreens. The study took place in Casablanca, Morocco, in two public maternity hospitals and two private gynaecologic centres, from April 1999 to July 1999, during antenatal consultations. RESULTS: The mean age of the 415 pregnant women was 29 +/- 6 years. One hundred and fifty-one women (37 p. 100) had a melasma and 72 (17 p. 100) used a sunscreen at their inclusion in the study. Women with melasma used sunscreens in 18.8 p. 100 and the others in 16.6 p. 100. Users of sunscreens differed from non-users in their higher level of instruction, professional activity, presentation in private practice and of urban origin. DISCUSSION: This study showed a low incidence of sunscreen use in our population of pregnant women. Melasma wasn't a reason for using sunscreens. However, access to medical information, especially to photoprotection advice, and the financial possibility of buying the product led to larger use of sunscreens. Up to now no study has confirmed that sunscreens can prevent melasma in dark phototype women with pregnancy. It would therefore be difficult to recommend them to pregnant women.
Adolescent↗
[Male breast cancer in Morocco].
INTRODUCTION: Male breast cancer is a rare disease, often of late presentation and poor prognosis. The aim of this work was to analyze the different clinical and therapeutic features for this disease in men. PATIENTS AND METHODS: This was retrospective study including 12 cases of male breast cancer seen in the Dermatology department of Casablanca between 1988 and 1999. RESULTS: The mean age of the patients was 60 years and the mean delay to consultation was 27 months. The skin was involved by tumor in 11 cases. Ulceration of the skin by tumor was seen in eight patients, and direct extension of the tumor into the nipple without ulceration was seen in three patients. Axillary lymph node involvement was seen in eight patients. Seven patients with invasive disease had metastases at distance. Treatment was usually surgical. Complementary treatment included radiotherapy, chemotherapy and/or hormonotherapy. DISCUSSION: Although breast cancer in men is far less common than breast cancer in women, it is associated with less favorable prognosis because diagnosis is usually made at an advanced stage. Concerted efforts must be made to educate both the public and health professionals, in order to make earlier diagnosis and thereby improve prognosis.
Aged↗
[Vulvar pathology].
We evaluated the main causes of vulvar dermatoses; in 785 patients with a vulvar diseases who visited Ibn Rochd Hospital Centre, Casablanca between January 1991 and December 2002. The average age was 31 years (range 2 months to 81 years); 362 patients (41.52%) had vulvar pruritus, 273 patients (34.77%) had warts and 157 (20%) had one or more vulva ulcers. The most common infectious pathology was papillomavirus infection, found in 273 cases (34.77%), followed by vulvovaginal candidiases in 102 cases (12.99 %), syphilitic chancre in 18 cases (2.29%) and herpes genitalis in 17 cases (2.16%). The most common non-infectious pathology was vulvar dermatosis: 259 cases (32.99%); idiopathic pruritus vulvae: 61 cases (7.7%); and tumour-related conditions: 45 cases (5.6%). The frequency of infectious conditions was over 50% and these were generally sexually transmitted infections.
Academic Medical Centers↗
Mutations in CGI-58, the gene encoding a new protein of the esterase/lipase/thioesterase subfamily, in Chanarin-Dorfman syndrome.
Chanarin-Dorfman syndrome (CDS) is a rare autosomal recessive form of nonbullous congenital ichthyosiform erythroderma (NCIE) that is characterized by the presence of intracellular lipid droplets in most tissues. We previously localized a gene for a subset of NCIE to chromosome 3 (designated "the NCIE2 locus"), in six families. Lipid droplets were found in five of these six families, suggesting a diagnosis of CDS. Four additional families selected on the basis of a confirmed diagnosis of CDS also showed linkage to the NCIE2 locus. Linkage-disequilibrium analysis of these families, all from the Mediterranean basin, allowed us to refine the NCIE2 locus to an approximately 1.3-Mb region. Candidate genes from the interval were screened, and eight distinct mutations in the recently identified CGI-58 gene were found in 13 patients from these nine families. The spectrum of gene variants included insertion, deletion, splice-site, and point mutations. The CGI-58 protein belongs to a large family of proteins characterized by an alpha/beta hydrolase fold. CGI-58 contains three sequence motifs that correspond to a catalytic triad found in the esterase/lipase/thioesterase subfamily. Interestingly, CGI-58 differs from other members of the esterase/lipase/thioesterase subfamily in that its putative catalytic triad contains an asparagine in place of the usual serine residue.
1-Acylglycerol-3-Phosphate O-Acyltransferase↗
[Loose anagen hair syndrome: a familial case with fetal hair in meconium].
INTRODUCTION: Loose anagen hair syndrome is a recently described hair disorder. We report a familial case. CASE REPORT: Two sisters were examined, 4 years-old and 8 months old, from a consanguineous marriage. At birth, they presented diffuse alopecia of the scalp; the meconium contained black and silky hair. Clinical examination noted the short, fine hair with easy and painless uprooting. No clinical associated symptoms were observed. The trichogram showed 70 p. 100 of anagen hair and 30 p. 100 of telogen hairs. DISCUSSION: Our familial case is original by this clinical symptom: discharge of probably dystrophic foetal hair in the meconium. Loose anagen hair is characterised by easily pluckable hair. The trichogram confirms the diagnostic. It is composed of anagen hairs (80 to 100 p. 100) devoid of sheaths. The loose anagen hair is usually isolated, but associations have been reported. It may occur in families. The precise pathogenesis and treatment of this hair disorder are not known.
Alopecia↗
[Allgrove's syndrome].
INTRODUCTION: Triple A syndrome is an autosomal recessive disease causing achalasia, alacrima and adrenal involvement with isolated glucocorticoid deficiency. Less than 70 cases have been reported worldwide. We report a case of familial adrenal insufficiency with hyperpigmentation diagnosed in a 14 month-old child. CASE REPORT: A 2 year-old boy, a product of consanguineous parents, was referred to our institution for evaluation of melanoderma. Since birth the boy had suffered from vomiting and diarrhea. Aged 6 months, mucosal erosive lesions had appeared associated with fever and further complicated at the age of one year by alacrima. At the age of 14 months, hyperpigmentation of all the skin had occurred. The family history is significant: two siblings (a girl and a boy) died of hypoglycemia with melanoderma and alacrima at the age of 5 and 3 respectively. Physical examination showed generalized hyperpigmentation particularly marked on the lips and genitalia. The genitalia of our patient were normal. Cortisol was 23.7 microns/l (normal: 193-772); ACTH was 11,722 pg/ml (normal: 10-60) and computed tomography of the abdomen confirmed adrenal gland hypoplasia. Treatment was initiated with hydrocortisone at the dose of 10 mg/day. At the age of 3, the boy developed plantar hyperkeratosis. When solid food was introduced, vomiting and regurgitation increased. An oesophagogram with fibroscopy revealed achalasia of the cardia. This was successfully corrected by surgery. Schimer's test confirmed alacrima. DISCUSSION: Our case report, characteristic of triple A syndrome, is unusual in view of the presence of plantar keratoderma and absence of any neurological abnormalities.
Adrenal Insufficiency↗
[Icthyosiform erythroderma: atypical manifestation of sarcoidosis].
BACKGROUND: Specific cutaneous manifestations of sarcoidosis can be extremely varied. We report one observation of cutaneous sarcoidosis with an exceptional clinical presentation: icthyosiform erythroderma. CASE REPORT: A 33-year-old woman had a dry icthyosiform erythroderma associated with arthralgia and muscular deficit of the pelvic belt in a context of fever and weight loss. Skin biopsy revealed the presence of an epithelioid giant-cell granuloma. This granuloma was also found in the salivary glands and in the bronchial mucosa. The chest X ray showed diffuse reticularis opacities. The diagnosis of sarcoidosis was retained. The patient was treated with oral prednisone (0.75 mg/kg/day). Total skin clearing was obtained after 15 days. DISCUSSION: This observation points out the heterogeneous nature of clinical manifestations of cutaneous sarcoidosis. The icthyosis erythroderma form is exceptional. Correct diagnosis is difficult and must be based on clinical, histological, radiological and biological criteria of sarcoidosis.
Adult↗
[Vogt-Koyanagi-Harada's disease: 3 cases].
BACKGROUND: Vogt-Koyanagi-Harada's disease is a bilateral chronic panuveitis sometimes associated with signs of meningo-encephalic, auditory and skin and nail involvement. We report 3 cases. CASE REPORTS: The first case was a 30-year-old woman who consulted for a red eye, low visual acuity, poliosis, and diffuse alopecia which had developed over 9 months. The ophthalmology examination disclosed anterior uveitis with retinal detachment. The second patient was a 9-year-old child who developed poliosis, canities, and achromic lesions over a 2 month period. The ophthalmology examination disclosed low visual acuity, irido-corneal synechiae and pigmented deposits on the anterior lens. The third patient was a 20-year-old man who consulted for alopecia, diffuse canities, and white body hair. The ophthalmology examination disclosed low visual acuity, anterior uveitis, and a serous chorio-retinal detachment. All three patients were given general corticosteroid therapy (1 mg/kg/d). The clinical course was favorable in all cases with however one case of recurrent ocular involvement and one case of pigmentation disorders. DISCUSSION: The diagnosis of Vogt-Koyanagi-Harada's disease was established in these three cases on the basis of the ocular and skin and nail signs. This rare disease usually occurs in young, often female, patients. The pathogenesis remains unknown. Among the three signs observed, ocular involvement is the most serious. Skin and nail signs are seen in two-thirds of cases. For rapid diagnosis and early treatment, this disease requires a multidisiplinary management associating the dermatologist and the ophthalmologist.
Adult↗
[Comparison of diagnostic criteria in Moroccan patients with Behçet's disease].
BACKGROUND: Behçet's disease is a vasculitis with diverse systemic manifestations usually involving the skin, eyes, joints and nerves. Clinical diagnosis is based on several signs, generally using the criteria established by the International Group for the Study of Behçet's disease, by O'Duffy, by Hamza, by Mason and Barnes and by the Japanese Committee. The purpose of this retrospective study was to assess the frequency of clinical manifestations in Behçet's disease and to apply the five diagnostic criteria scorings cited above to our patients to determine their sensitivity. PATIENTS AND METHODS: Between January 1988 and December 1997, 110 cases of Behçet's disease were seen at our Dermatology Unit in Casablanca as inpatients or outpatients. Complete history and clinical data including results of the ophthalmology exam and skin tests were available for 70 patients (29 men and 41 women, mean age 31 years). RESULTS: All 70 patients had buccal aphtosis and 88 p. 100 had genital aphtosis. Ocular signs were found in 36 p. 100 of the patients and the skin test was positive in 57 p. 100. According to the criteria of the International Group for the Study of Behçet's disease, 65 patients (93 p. 100) had Behçet's disease. For the Japanese Committee criteria there were 63 (90 p. 100), for the O'Duffy criteria 48 (68 p. 100), for Hamza criteria 45 (64 p. 100) and for Mason and Barnes criteria 35 (50 p. 100). DISCUSSION: Our series is comparable to other Mediterranean series observed in dermatology units, but our patients had fewer systemic manifestations compared with another Moroccan series reported from an internal medicine ward. This could be due to recruitment bias and different medical education. For positive diagnosis of Behçet's disease, the criteria established by the International Group for the Study of Behçet's disease, the Japanese Committee, O'Duffy and Hamza enabled the diagnosis in the majority of the cases. Fifty percent of the patients had all the criteria described in these scores. These criteria would appear to be both overly descriptive, with the risk of confounding other disease states such as enterocolopathy, or underly descriptive, making it impossible to classify certain patients as having Behçet's disease.
Adolescent↗
Lamellar ichthyosis: further narrowing, physical and expression mapping of the chromosome 2 candidate locus.
Lamellar ichthyosis (LI) is an autosomal recessive genodermatosis which has been shown to be both clinically and genetically heterogeneous. Keratinocyte transglutaminase (or transglutaminase 1: TGM1) has been demonstrated to be the disease-causing gene in some families, whilst in others, a second unidentified LI gene was mapped to chromosome 2q33-35 (ICR2B locus). In this study, we present a physical map that encompasses the ICR2B locus, including the mapping of new microsatellite markers. Based on this new map, genotyping additional families highly suggests a reduction in size of the candidate interval. The final interval is covered by a single yeast artificial chromosome (937-H-3) which is 2.2Mb in length. Fine mapping of potential candidate transcripts was also focused on this region.
Chromosomes, Artificial, Yeast↗
[Herpetic superinfection of pemphigus: 6 cases].
BACKGROUND: Herpes simplex infection should be searched for in patients who experience unexplained aggravation of acantholytic dermatosis. Epidermal alterations appear to play an important favoring role. PATIENTS AND METHODS: We report 6 cases of Herpes simplex infections in patients with pemphigus observed at the Ibn Rochd hospital dermatology unit, Casablanca, over a 3-year period (1995-1998). RESULTS: There were 4 men and 2 women, age range 27-76 years. Superficial pemphigus was observed in 5 cases and pemphigus vulgaris in 1. In all cases, histology showed cytopathogenic Herpes simplex infection. The Herpes simplex infection appeared when the patients were given general corticosteroid therapy at doses from 1 to 1.5 mg/kg/d for 15 days to 1 month. In two cases, the corticosteroids were associated with an immunosuppressor (azathioprine, cyclophosphamide). Cure was rapid with systemic aciclovir, achieved in 5 to 20 days. Recurrence was observed in 2 cases. DISCUSSION: Herpes simplex superinfection in patients with pemphigus is classically described but uncommonly observed. In our experience, it has become more frequent over recent years since, before 1990, we observed no cases. Its prevalence may be underestimated. The diagnosis of Herpes simplex superinfection is sometimes difficult and should be suggested in case of relapse or resistance to treatment. The Herpes virus simulates most cases of pemphigus relapse, with a preferential localization on the bullous lesions. The painful nature of the eruption is highly suggestive of Herpes simplex infection as is the presence of vesicles grouped in bouquets. Both type 1 and type 2 viruses are observed. Cure is rapidly achieved with aciclovir in 5 to 15 days. Spontaneous cure has been reported in the literature as well as recurrent herpetic infection. In two cases in the literature, the Herpes eruption was fatal due to severe herpetic hepatitis and disseminated intravascular coagulation. Herpes simplex infection must be rapidly recognized in pemphigus patients so curative aciclovir treatment can be given early.
Acyclovir↗
Novel recurrent nonsense mutation causing neurofibromatosis type 1 (NF1) in a family segregating both NF1 and Noonan syndrome.
Neurofibromatosis type 1 (NF1), a genetic disorder with neuroectodermal involvement, demonstrates phenotypic overlap in some patients with Noonan syndrome (NS), ultimately resulting in the so-called neurofibromatosis-Noonan syndrome (NF-NS). A strong association of the two phenotypic traits was recently illustrated by a four-generation family, although NF1 and NS were eventually demonstrated to segregate independently on the basis of polymorphic DNA markers [Bahuau et al., 1996: Am J Med Genet 66:347-355]. Identification of the causal NF1 mutation seemed a prerequisite to further dissecting this singular familial association. Using the protein truncation assay, a nonsense mutation (C2446T-->R816X) of the neurofibromin gene was evidenced. This mutation occurred on a CpG dinucleotide within exon 16 and 5' to the GAP domain-specifying region of the gene. R816X creates a recognition site for endonuclease HphI, absent in 2 individuals with NS only. Screening 184 unrelated NF1 patients, three novel occurrences of the mutation were found in individuals diagnosed with classical NF1. Based on the assumption of genotype-phenotype correlation in these individuals, clinical and molecular analyses of this four-generation family demonstrated that the NF-NS phenotype was additive, being the result of both classical NF1 and NS. This particular observation also suggests the presence of an NS locus on 17q, which might be of interest for further linkage studies.
DNA Mutational Analysis↗
Pharmacokinetics of terbinafine and five known metabolites in children, after oral administration.
As an extensive study, the pharmacokinetics of terbinafine and five known metabolites have been investigated after single and repeated oral administration to 12 pediatric patients. After single administration of 125 mg terbinafine, four compounds were unconjugated and the hydroxymetabolites appeared in trace amounts as glucuronides. The main metabolites in plasma were unconjugated carboxy compounds. Kinetics of terbinafine and N-desmethylterbinafine metabolite were comparable. The interindividual AUCt variability was similar for terbinafine, N-desmethylterbinafine and carboxyterbinafine. In urine, the major fraction was the hydrophilic unconjugated N-desmethyl-carboxyterbinafine (15%). After repeated administration of 125 mg day(-1), mean trough levels of terbinafine, N-desmethylterbinafine, carboxyterbinafine and N-desmethylhydroxy-terbinafine, and also that of hydroxyterbinafine metabolite were similar, for each compound, on days 21, 42 and 56 denoting that steady state was reached at least on day 21 and no accumulation occurred between days 21 and 56.
Administration, Oral↗
[Cardiovascular manifestations of pseudoxanthoma elasticum. Apropos of 2 cases].
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Adult↗
[Male predominance of leg ulcer in Morocco].
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Adult↗
[Skin manifestations of sarcoidosis: diagnostic problems].
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Adult↗
[Repair of substance losses of the nose: apropos of 45 cases].
The authors report 45 cases of tissue loss in the nose, in most cases secondary to cancer ablation, and in a few (2 cases) to trauma. The reconstructive techniques were mainly the classical ones using local flaps, usually at the same time as the tumour removal. Where the reconstruction was a total one, this involved the use of a compound extended Converse flap, which has the advantage of bringing in sufficient tissue associated with the bone graft, and also avoids the risks of thinning or exposure of the graft. The indications depend on anatomical factors, and on the extent and depth of the tissue loss. The cospetic results are very satisfactory, but it is necessary to revise the flaps in order to obtain the best final results.
Adolescent↗