Pediatric Dermatology Unit

Bordeaux, France

Pediatric Dermatology Unit

Bordeaux, France
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Zvulunov A.,Pediatric Dermatology Unit | Zvulunov A.,Ben - Gurion University of the Negev | Shkalim V.,Tel Aviv University | Ben-Amitai D.,Pediatric Dermatology Unit | And 3 more authors.
Journal of the American Academy of Dermatology | Year: 2012

Background: Some authorities consider alopecia mucinosa (AM)/follicular mucinosis (FM) to invariably represent mycosis fungoides (MF). This understanding of AM/FM derives from observations in adults. Objectives: We sought to explore the clinicopathologic features and natural history of pediatric AM/FM. Methods: Medical records were searched for children given the diagnosis of AM/FM from 1998 through 2009. Diagnosis of AM/FM was defined as the presence of well-demarcated hairless plaques with follicular prominence plus an abundance of mucin on histopathologic examination. Results: Forty children with a clinical diagnosis of AM/FM were identified. Nine did not meet the inclusion criteria. In the 31 remaining cases (16 boys, 15 girls) the mean age at onset was 9 ± 3.5 years. Histopathologic examination showed folliculotropism in 28 patients (90%) and epidermotropism in 15 (48%). Twelve cases fulfilled the International Society of Cutaneous Lymphomas (ISCL) diagnostic criteria for early MF. The histopathologic findings were typical of MF in only in two of these cases. T-cell receptor gene rearrangement was positive in 3 of 6 (50%) of tested samples, one in a patient who fulfilled the ISCL criteria for early MF. Mean duration of follow-up was 6.2 ± 3.7 years. All skin lesions resolved and none persisted or recurred. Hodgkin lymphoma was diagnosed 6 months after diagnosis of AM/FM in one patient. Limitations: This was a retrospective study. Conclusions: Although some pediatric cases meet the diagnostic criteria for MF, AM/FM cannot be regarded unequivocally as early follicular MF in this age group. We suggest the current diagnostic criteria for early MF should exclude children with AM/FM. Long-term follow-up of children with AM/FM is nevertheless warranted.

Blaydon D.C.,Queen Mary, University of London | Nitoiu D.,Queen Mary, University of London | Eckl K.-M.,University of Cologne | Cabral R.M.,Queen Mary, University of London | And 12 more authors.
American Journal of Human Genetics | Year: 2011

Autosomal-recessive exfoliative ichthyosis presents shortly after birth as dry, scaly skin over most of the body with coarse peeling of nonerythematous skin on the palms and soles, which is exacerbated by excessive moisture and minor trauma. Using whole-genome homozygosity mapping, candidate-gene analysis and deep sequencing, we have identified loss-of-function mutations in the gene for protease inhibitor cystatin A (CSTA) as the underlying genetic cause of exfoliative ichthyosis. We found two homozygous mutations, a splice-site and a nonsense mutation, in two consanguineous families of Bedouin and Turkish origin. Electron microscopy of skin biopsies from affected individuals revealed that the level of detachment occurs in the basal and lower suprabasal layers. In addition, in vitro modeling suggests that in the absence of cystatin A protein, there is a cell-cell adhesion defect in human keratinocytes that is particularly prominent when cells are subject to mechanical stress. We show here evidence of a key role for a protease inhibitor in epidermal adhesion within the lower layers of the human epidermis. © 2011 The American Society of Human Genetics.

Choi J.-S.,Yale University | Boralevi F.,Pediatric Dermatology Unit | Brissaud O.,Paediatric Care Unit | Sanchez-Martin J.,University of Oviedo | And 4 more authors.
Nature Reviews Neurology | Year: 2011

Background A 3-month-old male infant presented, beginning on the second day of life, with paroxysmal painful events that started with tonic contraction of the whole body followed by erythematous harlequin-type color changes.Investigations Screening of the SCN9A gene, which encodes the voltage-gated sodium channel Na V 1.7, identified a new mutation, Gly1607Arg, located within the domain IV S4 voltage sensor. Whole-cell patch-clamp analysis demonstrated functional effects of the mutant channel that included impaired inactivationg-a hallmark of paroxysmal extreme pain disorder (PEPD).Diagnosis The patient was diagnosed as having PEPD, an autosomal dominant disorder characterized by severe rectal pain triggered by defecation or perineal stimulation, usually followed by ocular or submaxillary pain. Erythematous flushing, sometimes in a harlequin pattern, can be a prominent feature of this condition.Management Treatment with carbamazepine (10 mg/kg/day) for g-3 months was ineffective in this case, and the parents made a decision to discontinue the drug. The mother was instructed to avoid painful stimuli that could trigger an episode. © 2011 Macmillan Publishers Limited. All rights reserved.

Lapidoth M.,Laser Unit | Lapidoth M.,Tel Aviv University | Israeli H.,Sheba Medical Center | Ben Amitai D.,Pediatric Dermatology Unit | And 2 more authors.
Dermatology | Year: 2013

Objective: The aim of this study was to evaluate the clinical results of 71 cases with verrucous epidermal nevus (VEN) treated by cryotherapy or CO 2 laser. Methods: The files and photographs of patients with VEN who were treated in the laser unit of a tertiary hospital between January 2005 and December 2011 were evaluated. Pretreatment and 12-month follow-up photographs were evaluated using a 5-point scale: 'excellent' (75-100% clearance), 'good' (50-75% clearance), 'fair' (25-50% clearance), 'poor' (<25% clearance) or 'worse'. Results: Of 71 patients, 62 responded well to cryotherapy alone, and 9 facial VEN required CO2 laser treatment. Small VEN required relatively few treatments (mean 3.4) with 90% scoring 'excellent' and 10% scoring 'good'. Larger VEN required more treatments (mean 7.4) and did not respond as well, with 71% scoring 'excellent', 14% scoring 'good' and 14% scoring 'fair'. Conclusion: Our experience suggests that cryotherapy is the ideal treatment for small facial VEN, while ablation by CO2 laser should be considered an option for resistant facial lesions. © 2013 S. Karger AG, Basel.

Halachmi S.,Herzelia Dermatology and Laser Center | Israeli H.,Sheba Medical Center | Ben-Amitai D.,Pediatric Dermatology Unit | Ben-Amitai D.,Tel Aviv University | And 2 more authors.
Lasers in Medical Science | Year: 2014

Hereditary hemorrhagic telangiectasia (HHT) is a familial disorder, in which variably sized arteriovenous malformations develop in the skin, respiratory tract, central nervous system, and gastrointestinal and urogenital tracts. Bleeding is a major problem in the abnormal vessels, primarily in the nose and internal lesions. Skin lesions, in contrast, do not generally bleed spontaneously but pose a significant cosmetic concern. The lesions are characteristically small caliber vessels located in the dermis. As such, they are ideal targets for vascular lesions. Eight patients with cutaneous facial lesions of HHT were treated by pulsed dye laser. All patients exhibited excellent (75-100 %) clearance after a mean of 2.6 treatments (range 1-8). In comparison, a control group of patients with non-HHT facial telangiectasia required a mean of 1.9 (range 1-5; p < 0.05) treatments. No adverse events were reported in either group. The study confirms the safety and efficacy of PDL in the management of the cutaneous lesions of HHT. © 2013 Springer-Verlag London.

Sagi L.,Tel Aviv Sourasky Medical Center | Sagi L.,Tel Aviv University | Zvulunov A.,Pediatric Dermatology Unit | Zvulunov A.,Ben - Gurion University of the Negev | And 4 more authors.
Dermatology | Year: 2014

Background: Propranolol is highly effective in the treatment of infantile hemangioma (IH), but important clinical and pharmacological data are lacking. Objective: The aims of the present study were to evaluate the efficacy of propranolol for the treatment of IH, to identify favorable prognostic factors in propranolol-treated IH, and to evaluate the safety of propranolol for the treatment of IH. Methods: Clinical data were recorded from the electronic files and digital photographs of 99 patients with IH attending a tertiary pediatric medical center (2008-2011). Findings were evaluated by regression in volume and color changes. Results: The male-to-female ratio was 1:4. Age at treatment initiation was 9.4 ± 10.1 months; 15% of the treated hemangiomas were beyond the proliferative phase (17-54 months). The propranolol starting dose was 2 mg/kg/day. Duration of the treatment was 8.5 ± 3.2 months. All but 1 patient responded to treatment. A longer treatment course was required for segmental and deep hemangiomas. Mild side effects occurred in 32% of patients. Recurrence occurred in 13% of patients. Conclusion: Lesions located on the face are better responders when treatment is started early. Treatment should continue up to age 12-15 months, with a longer course for segmental or deep hemangiomas. © 2014 S. Karger AG, Basel.

Halachmi S.,Herzelia Skin and Laser Center | Ben Amitai D.,Pediatric Dermatology Unit | Ben Amitai D.,Tel Aviv University | Lapidoth M.,Laser Unit | Lapidoth M.,Tel Aviv University
Journal of Drugs in Dermatology | Year: 2013

Background: Acne scarring is a prevalent and challenging cosmetic issue, which is often addressed by multiple modalities. A low-viscosity non-animal stabilized hyaluronic acid (NASHA) dermal filler, injected in microdoses into the mid-to-superficial dermis, may provide a useful new approach to improving the appearance of depressed acne scars. Materials and Methods: Twelve consecutive patients with moderate to severe acne scarring, who had completed a series of fractional laser resurfacing, underwent microinjections of 20 mg/mL hyaluronic acid (HA) gel into discrete depressed acne scars on the face. Results: Immediate visual improvement was observed in all lesions. The procedure was well tolerated. Adverse events were limited to transient pinpoint bleeding at the injection site. Conclusion: Microinjection of low viscosity HA offers a valuable technique for the treatment of discrete depressed acne scars. Copyright © 2013 Journal of Drugs in Dermatology.

Pavlotsky F.,Phototherapy and Day Care Center | Pavlotsky F.,Tel Aviv University | Hodak E.,Rabin Medical Center | Hodak E.,Tel Aviv University | And 4 more authors.
Journal of the American Academy of Dermatology | Year: 2014

Background Psoralen plus ultraviolet (UV) A (PUVA) radiation is the preferred treatment for folliculotropic mycosis fungoides (MF) and MF refractory to narrowband (NB) UVB radiation. However, systemic PUVA has many unfavorable side effects and contraindications. Bath PUVA has been found to be a suitable alternative in patients with psoriasis, but data on MF are sparse. Objective The purpose of the study was to evaluate the effectiveness of bath PUVA in the treatment of folliculotropic MF and NB-UVB-refractory early-stage MF. Methods The study group included 26 patients of average age 44 years attending a tertiary medical center in 2004 through 2012, 14 with folliculotropic type and 12 with NB-UVB-refractory early-stage MF who were not amenable for oral PUVA. Treatment consisted of 0.2 mg/L 8-methoxypsoralen bath 3 times weekly followed by UVA irradiation at 0.3 J/cm2 with fixed increments every second session. Results A complete clinical response was achieved in 62% of patients after an average of 33 weeks and a cumulative radiation dose of 158 J/cm 2. Limitations This was a relatively small series. Conclusion Bath PUVA is a good treatment option for superficial folliculotropic MF and NB-UVB-refractory early-stage MF. © 2014 by the American Academy of Dermatology, Inc.

Valdman-Grinshpoun Y.,Pediatric Dermatology Unit | Valdman-Grinshpoun Y.,Szold Health Center | Ben-Amitai D.,Pediatric Dermatology Unit | Ben-Amitai D.,Tel Aviv University | And 2 more authors.
Dermatology Research and Practice | Year: 2012

Atopic dermatitis is a multifactorial, chronic relapsing, inflammatory disease, characterized by xerosis, eczematous lesions, and pruritus. The latter usually leads to an "itch-scratch" cycle that may compromise the epidermal barrier. Skin barrier abnormalities in atopic dermatitis may result from mutations in the gene encoding for filaggrin, which plays an important role in the formation of cornified cytosol. Barrier abnormalities render the skin more permeable to irritants, allergens, and microorganisms. Treatment of atopic dermatitis must be directed to control the itching, suppress the inflammation, and restore the skin barrier. Emollients, both creams and ointments, improve the barrier function of stratum corneum by providing it with water and lipids. Studies on atopic dermatitis and barrier repair treatment show that adequate lipid replacement therapy reduces the inflammation and restores epidermal function. Efforts directed to develop immunomodulators that interfere with cytokine-induced skin barrier dysfunction, provide a promising strategy for treatment of atopic dermatitis. Moreover, an impressive proliferation of more than 80 clinical studies focusing on topical treatments in atopic dermatitis led to growing expectations for better therapies. © 2012 Y. Valdman-Grinshpoun et al.

Defrin R.,Tel Aviv University | Lurie R.,Pediatric Dermatology Unit
Clinical Journal of Pain | Year: 2013

OBJECTIVES: The underlying mechanism of trichodynia (scalp/hair pain, is unknown). The aim of this study was to characterize chronic trichodynia and to conduct, for the first time, sensory testing in patients with trichodynia to learn about possible underlying mechanisms. METHODS: Participants were 16 trichodynia patients and 19 healthy controls. Participants underwent testing of touch and pressure-pain threshold as well as allodynia in painful and pain-free scalp sites and in the hands (intact remote region). A trichogram (hair test) was conducted on painful and pain-free scalp sites to evaluate hair cycle abnormalities. The chronic pain was characterized as well. RESULTS: Painful sites were characterized by decreased thresholds for light touch (P<0.01) and pressure pain (P<0.01) and high rates of static allodynia (94%) compared with adjacent pain-free sites and controls. A significant negative correlation was found between chronic pain intensity and scalp thresholds. Spontaneous and evoked pain existed only in scalp sites with hair cycle abnormalities. In addition, pressure-pain threshold in the hands was significantly lower in trichodynia patients compared with controls. DISCUSSION: The cranial hyperalgesia and allodynia, the generalized hyperalgesia, and the correlation between hyperalgesia and chronic pain suggest that trichodynia is related with both peripheral and central sensitization, respectively. The coexistence of hair cycle abnormalities and chronic pain might suggest a common denominator for both phenomena, possibly mediated by proinflammatory agents. Clinical implications are discussed. Copyright © 2012 by Lippincott Williams & Wilkins.

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