Psoriasis after cord blood stem cell transplantation.
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Biomedical subjects
Publications and source records attributed to A Taïeb.
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Reactive oxygen species (ROS) generated by ultraviolet (UV) irradiation are counterbalanced by endogenous antioxidant systems. To test the hypothesis of a novel photoprotective approach, we irradiated epidermis reconstructed with normal human keratinocytes overexpressing sustainably lentivirus-mediated catalase (CAT), copper/zinc superoxide dismutase (CuZnSOD) or manganese superoxide dismutase (MnSOD) enzymes. We found that following UVB irradiation there was a marked decrease in sunburn cell formation, caspase-3 activation and p53 accumulation in human reconstructed epidermis overexpressing CAT. Moreover, UVA-induced hypertrophy and DNA oxidation (8-oxodeoxyguanosine) were decreased by CAT overexpression. These effects were not achieved by overexpression of CuZnSOD or MnSOD. In conclusion, vector-mediated CAT overexpression could be a promising photoprotective tool against deleterious effects of UV irradiation such skin cancer especially in monogenic/polygenic photosensitive disorders characterized by ROS accumulation.
Senile lentigo is a common component of photoaged skin. It is characterized by hyperpigmented macules which affect chronically irradiated skin mostly after the age of 50. This study was undertaken to assess the morphology of senile lentigo on the dorsum of the hands. A systematic comparison between lesional and perilesional skin using histology and transmission electron microscopy was done to determine whether melanocytes or keratinocytes are affected in the evolution of lesions and which tissue structure is modified. The histology study showed that lesional skin is characterized by a hyperpigmented basal layer and an elongation of the rete ridges, which seem to drive deeply into the dermis. The epidermis contained clusters of keratinocytes, which retained and accumulated the melanin pigment. Electron microscopy studies showed important modifications in the lesional skin ultrastructure in comparison with perilesional skin. In melanocytes from perilesional and lesional skin, we observed normal size melanosomes at all stages of maturation in the cytoplasm and in migration within dendrites. No pigment accumulation was observed. However, the morphology of melanocytes in lesional skin revealed an activated status with numerous mitochondria and a well-developed endoplasmic reticulum, which could reflect intense protein synthesis. In basal keratinocytes from lesional skin, we observed numerous melanosome complexes called polymelanosomes, which formed massive caps on the nuclei. Observations in colored semi-thin sections also revealed perturbed structures in the basal layer region, which could explain the skin perturbation. Indeed, we observed keratinocytes that presented important microinvaginations and pendulum melanocytes, which sank into the dermis, beneath the basal layer of keratinocytes. These cell modifications seemed to be due to a perturbation of the dermal-epidermal junction, which appeared disorganized and disrupted and could directly disturb the basal support of the cells.
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The differential diagnosis of a midfacial mass in a child includes a great variety of tumours. Odontogenic myxoma is a benign tumour arising from the mesenchymal portion of the odontogenic apparatus that is usually seen in adolescents and adults.
BACKGROUND: We describe three neonates who presented around the tenth day of life with multiple bilateral fluctuating cold abscesses situated in the lymph node areas, with secondary fistulation but without associated fever or general symptoms. RESULTS: In all cases, Staphylococcus aureus was found in the abscesses and the umbilicus with similar antibiograms. The evolution was good and the children remained in good health afterwards. CONCLUSION: Disseminated neonatal staphylococcal infections with a benign evolution are not commonly described in the medical literature. Clinically, cold inflammatory abscesses of the large folds associated with omphalitis may correspond to either transient bacteriemia well controlled in an immunocompetent host or more likely a local contamination process.
Topical steroids are still used suboptimally, but remain the mainstay of atopic dermatitis treatment. Topical steroid phobia is rampant in many countries, a real advantage for the entry on the market of topical immunomodulators (TIMs), which inhibit both antigen specific and non-specific T cell activation in the skin, by blockade of gene transcription of proinflammatory cytokines such as IL2 and TNF alpha. Topical tacrolimus and pimecrolimus have the most advanced clinical development. Tacrolimus, already used orally in transplantation medicine, is already available in France since 2003 as a 0.03% ointment for children (Protopic, Fujisawa). Its introduction on the market has substantially changed prescription habits in atopic dermatitis. Recalcitrant adolescent and adult head and neck lesions are the major target, but the drug is is also widely used in children, with a good safety profile. The risk of herpes virus superinfections did not increase significantly in clinical trials but needs further monitoring. Long-term prescription will need a closer look at a still much debated increased skin cancer risk. The marked efficacy on thin skin sites and absence of atrophogenic properties of the drug balance its side effects at the first applications on inflamed skin (pruritus, burning sensation). Clinical studies using pimecrolimus (Elidel, Novartis), marketed as a 1% cream, show a satisfactorily efficacy profile in adults and children including infants. The drug is better tolerated and is already widely introduced on the international market since 2002 with a pediatric positioning, but is nor available yet in 2004 in France. Besides phototherapy, systemic immunosuppressants remain useful drugs in severe disease especially in older children and adolescents, cyclosporin remaining the leading drug. Preventive immunomodulation modifying the intestinal microflora is very promising approach which deserves a large-scale assessment.
INTRODUCTION: Hereditary angioedema is characterized by episodes of subcutaneous, digestive or laryngeal edema. In some cases, non-pruritic reticular erythema may precede the episodes of edema. OBSERVATION: Every 4 to 6 weeks since infancy, a girl presented non-pruritic widespread reticular erythema, sparing the face. Two or three times every year, abdominal pain or edema of the lower limb joints followed the skin eruption. At 12 years of age, she was hospitalized because of an edema of the face associated with the eruption. Exploration of the complement confirmed the diagnosis of type I hereditary angioedema. DISCUSSION: The mean delay before diagnosis of hereditary angioedema is of 7 years. Reticular erythema in hereditary angioedema is frequent (40p. 100 of cases) and it usually occurs early in childhood, even in the absence of any episode of angioedema. When present, these eruptions usually precede an episode of angioedema. Recognition of this eruption as a symptom of hereditary angioedema would shorten the delay before diagnosis and anticipate appropriate management of the episodes.
BACKGROUND: Dermatological surgery is a relatively new and expanding subspecialty within dermatology. Little information is available about complications in this kind of surgery in the European setting. OBJECTIVES: The aim of this study was to assess the incidence of anaesthetic, haemorrhagic and infectious complications in dermatological surgery and to highlight the factors associated with these complications. METHODS: Data were collected prospectively over a 3-month period for all surgical procedures performed by a network of dermatologists (n = 84 dermatologists) in France, including the excision of all benign or malignant tumours but excluding sebaceous cysts and pyodermas. Information was collected regarding dermatologists, patients, procedures and complications. RESULTS: A total of 3788 surgical procedures were available for review; 236 complications, mostly minor, occurred in a total of 213 surgical procedures (6%), bleeding being the most common (3%). Vaso-vagal syncope was the main anaesthetic complication (51 of 54). Infectious complications occurred in 79 patients (2%). Superficial suppuration accounted for 92% of surgical site infections. Only one patient had a systemic infection. Complications requiring additional antibiotic treatment or repeat surgery accounted for only 22 cases of 3788 (1%). No statistically significant correlation was found with the characteristics of the dermatologists, especially with respect to their training or amount of surgical experience. Similarly, no link could be established between complications and surgical conditions. Multivariate analysis showed that anaesthetic or haemorrhagic complications were independent factors for infectious complications. Sex, administration of an anticoagulant or immunosuppressant, type of procedure performed and duration exceeding 24 min were independent factors for haemorrhagic complications. CONCLUSIONS: This study shows a low rate of complications associated with dermatological surgery performed by dermatologists under local anaesthesia on an outpatient basis.
The diagnosis of atopic dermatitis (AD) is made using evaluated clinical criteria. Management of AD must consider the symptomatic variability of the disease. It is based on hydrating topical treatment, and avoidance of specific and unspecific provocation factors. Anti-inflammatory treatment is used for exacerbation management. Topical corticosteroids remain the first choice. Systemic anti-inflammatory treatment should be kept to a minimum, but may be necessary in rare refractory cases. The new topical calcineurin inhibitors (tacrolimus and pimecrolimus) expand the available choices of topical anti-inflammatory treatment. Microbial colonization and superinfection (e.g. with Staphylococcus aureus, Malassezia furfur) can have a role in disease exacerbation and can justify the use of antimicrobials in addition to the anti-inflammatory treatment. Evidence for the efficacy of systemic antihistamines in relieving pruritus is still insufficient, but some patients seem to benefit. Adjuvant therapy includes ultraviolet (UV) irradiation preferably of UVA wavelength; UVB 311 nm has also been used successfully. Dietary recommendations should be specific and only given in diagnosed individual food allergy. Stress-induced exacerbations may make psychosomatic counselling recommendable. 'Eczema school' educational programmes have proved to be helpful.
BACKGROUND: Glomuvenous malformation (GVM) ("familial glomangioma") is a localised cutaneous vascular lesion histologically characterised by abnormal smooth muscle-like "glomus cells" in the walls of distended endothelium lined channels. Inheritable GVM has been linked to chromosome 1p21-22 and is caused by truncating mutations in glomulin. A double hit mutation was identified in one lesion. This finding suggests that GVM results from complete localised loss of function and explains the paradominant mode of inheritance. OBJECTIVE: To report on the identification of a mutation in glomulin in 23 additional families with GVM. RESULTS: Three mutations are new; the others have been described previously. Among the 17 different inherited mutations in glomulin known up to now in 43 families, the 157delAAGAA mutation is the most common and was present in 21 families (48.8%). Mutation 108C-->A was found in five families (11.8%), and the mutations 554delA+556delCCT and 1179delCAA were present together in two families (4.7% each). Polymorphic markers suggested a founder effect for all four mutations. CONCLUSIONS: Screening for these mutations should lead to a genetic diagnosis in about 70% of patients with inherited GVM. So far, a mutation in glomulin has been found in all GVM families tested, thus demonstrating locus homogeneity.
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Erosive oral lichen planus (OLP) is a painful chronic inflammatory disease that is sometimes resistant to systemic or topical therapies. Topical steroids remain the mainstay of therapy, but topical tacrolimus has recently been used to treat OLP resistant to topical corticosteroids. Topical tacrolimus appears as an effective and safe treatment of symptomatic OLP. We report the first histopathologically documented case of oral mucosa pigmentation after OLP treatment with topical tacrolimus. The relation between tacrolimus treatment and staining was suggested by the appearance of pigmentation during topical tacrolimus treatment and its clinical disappearance when treatment was stopped. Histopathology showed an increase in melanocyte numbers and melanogenesis.
This paper is aimed at defining atopic dermatitis (AD) in a pragmatic and historical perspective (diagnostic criteria, severity scores, quality of life scales) and to summarise its natural history, complications included. The signification of atopy is still not clearly defined. Immuno-allergologists tend to limit the term to a hereditary predisposition of the immune system. The definition of atopic dermatitis in infants has not been validated. Atopic dermatitis in France has a prevalence of 2 to 5 p. 100 at any age (6 to 9 p. 100 before the age of 15), depending on the strictness of the criteria applied. For infants suffering from AD with a first degree parent exhibiting AD, asthma or rhinitis, the risk of developing asthma is of around 40 p. 100. This data suggests the interest of preventive measures targeting the skin in the hypothesis of an atopic "march". There is no consensus to define the terms used in the description of the maintenance phase of treatment of this chronic and recurring affection. Proposals have been made to define the terms of flares, remission, intolerance and resistance.
Necrosis was induced by cell-cell contacts of human dermal fibroblasts in three-dimensional culture. Dramatic induction of cyclooxygenase-2 (COX-2) expression was found throughout these necrotizing cell clusters, whereas no increase in expression of apoptosis markers was seen. The cells were rapidly committed to necrosis, and the process could not be reversed by allowing them to spread and adhere on a solid substrate. Induction of COX-2 expression was accompanied by greatly enhanced production of the prostaglandins E(2), I(2), and F(2alpha). When applied exogenously on necrotizing clusters, these prostaglandins delayed cell clustering and further enhanced COX-2 expression. Abolishing prostaglandin production by NS-398 or indomethacin reduced cell membrane damage (as measured by lactate dehydrogenase release into the culture medium). We also identified alpha-enolase-mediated plasminogen activation as the major extracellular proteolytic executor of necrotic cell death. In contrast to inhibition of COX-2, inhibition of plasminogen activation failed to inhibit membrane damage associated with necrosis. Intracellular proteolysis, by caspases, was shown to take part in COX-2 induction. Taken together, our results indicate that cell-cell contacts induce an actively programmed necrotic process that functionally involves COX-2, a known hallmark of inflammation and cancer.
INTRODUCTION: clinical presentation which seems to be more specific of this infection. EXEGESIS: A 35-year-old woman, 12 weeks pregnant, presented with a primary infection of parvovirus B19. The clinical presentation was characterized by pseudo-cellulitis plaques of the buttocks and the vulva, buccal enanthema with ulcerations and Koplick spot. CONCLUSION: This is the second observation which describes such cutaneous and mucosal manifestations associated with parvovirus B19 infection. This kind of clinical presentation should be systematically reported to become well known by the physicians as erythema infectiosum of fifth disease or "gloves and socks" syndrome.
BACKGROUND: Eccrine squamous syringometaplasia has been reported in some cases as an Herpeviridae complication. We report a case of eccrine squamous syringometaplasia associated with a severe cytomegalovirus infection in an immunocompromised patient, and we discuss about potential viral or drug triggering factors. METHODS: A 22 years-old man was hospitalized in an intensive care unit for rejection of a renal graft associated with a disseminated cytomegalovirus infection. A papular and papulopustular eruption appeared on the trunk and the limbs. RESULTS: Histological examination of a skin sample showed eccrine squamous syringometaplasia, with evidence of cytomegalovirus genomic sequences using PCR. Two weeks later, the patient developed toxic epidermal necrolysis, with fatal issue. CONCLUSIONS: Eccrine squamous syringometaplasia is a rare condition, without specific clinical features. Numerous local affections have been reported to induce eccrine syringometaplasia (ulcer, scar, pyoderma gangrenosum, drug injection.), drugs (cytotoxic agents, non steroidal anti inflammatory therapies) and in cases of infection due to cytomegalovirus or herpes simplex virus. The potential implication of cytomegalovirus or foscarnet as triggering factors in our case is discussed. This observation and other similar reported cases lead to the conclusion that eccrine squamous syringometaplasia may be an underestimated complication of cytomegalovirus infections in immunocompromised patients.