Y de Prost

Université René Descartes - Paris 5, Lutetia Parisorum, Île-de-France, France

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Publications (245)692.15 Total impact

  • [Show abstract] [Hide abstract]
    ABSTRACT: Background Lymphomatoid Papulosis (LyP) is an uncommon cutaneous T-cell lymphoproliferative disorder (CTLPD) rarely encountered in children.Objective: To specify characteristics of paediatric LyP and to describe both diagnostic difficulties and course of the disease with the experience of 10 years follow up.Patients and Methods This was a retrospective, single-center study of twenty-five children diagnosed with LyP according to the 2008 World Health Organization guidelines, and a clinical and pathological correlation by two experts.ResultsThe mean age at the onset was 7y 6m. The lesions were mostly papulonodular with frequent pruritus (40%). A mucosal involvement could be observed. A single ulcerate nodule was initially suggestive of a primary cutaneous anaplastic large cell lymphoma (C-ALCL). Pityriasis lichenoides was associated in 36% of cases, atopic dermatitis in 28% and non specific infections in 28%. Complete remission was observed in 44% of cases. Through the mean follow-up of 10 years,none of our patients have experienced lymphoma occurence. Histopathologic subtype A clearly predominated (82%). A marked eosinophilic infiltrate was present in 44% and a cutaneous T gamma clone in 40%. No correlation was observed between histopathologic subtype, cutaneous clone or LyP clinical course.Limitations: retrospective studyConclusion Paediatric LyP belongs to CD30-positive CTLPD including C-ALCL. Children have to be carefully life-long followed-up even if the prognosis appears good. The high frequency of an associated viral infection, atopic dermatitis, marked eosinophilic infiltrate and a good outcome suggests that paediatric LyP could be considered as a reactional disease rather than a malignant disorder.This article is protected by copyright. All rights reserved.
    British Journal of Dermatology 04/2014; · 3.76 Impact Factor
  • The American Journal of dermatopathology 02/2013; · 1.30 Impact Factor
  • The American Journal of dermatopathology 02/2013; · 1.30 Impact Factor
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    ABSTRACT: Connective tissue nevi (CTN) may be isolated, either sporadic or hereditary, or syndromic as in the Buschke-Ollendorff syndrome. Few publications have addressed the variable clinical and histopathologic expression of these benign hamartomas. We sought to characterize the clinical and histopathologic features of CTN and to highlight a spectrum of clinical disease. We carried out a retrospective study of cases selected after strict clinical and histopathologic confirmation of the diagnosis. A total of 33 patients with CTN were included. The average age of onset was 2 years. Three clinical forms were distinguished: type A with lesions at a single site, with one case presenting as an ulcerated infiltrated plaque; type B with two or more sites of involvement; and type C with unusually severe infiltration with functional impairment of a limb. Histopathologic examination of lesional biopsy specimens showed 10 collagenomas, one elastoma, 18 mixed CTN, and an increased number of fibroblasts in 4 cases. No correlation between clinical type and histopathologic findings was observed. This was a descriptive case series. CTN comprise a clinical spectrum ranging from isolated papules to unusually severe aggressive plaques with monomelic involvement. The histopathologic features are heterogeneous and include a newly described variant, which we name "cellular CTN" because of the increased number of fibroblasts.
    Journal of the American Academy of Dermatology 10/2011; 67(2):233-9. · 4.91 Impact Factor
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    ABSTRACT: Generalized recessive dystrophic epidermolysis bullosa (RDEB) is often complicated by high nutritional difficulties with risks of malnutrition. To provide information regarding the benefits of enteral feeding by gastrostomy (GTF), energy and protein requirements, tolerance, growth and pubertal development in children with RDEB. Twenty-four patients were referred over a 7-year period in a retrospective study. Gastrostomy placement was decided in patients unable to feed orally and/or presenting loss in weight and height of at least 1 SD compared with their best growth level, despite regular nutritional advice. Weight and height were expressed as Z-scores. Catch-up growth following GTF onset was studied. Gastrostomies were performed in 11 children (aged 9·0±5·8years), and one young man aged 18years. The body weight Z-score was -2·3±1·0, height Z-score 1·1±1·1, weight-for-height was 81±11% and height-for-age 95± 4%. At onset, GTF provided 74±21% and 180±81% of the recommended dietary allowance (RDA) for energy and proteins, respectively. At study update (53±20months), GTF provided 91±29% and 205±100% of RDA for energy and proteins, respectively. Weight-for-height reached 92±15% and height-for-age 98±5%. A normal puberty was obtained when GT was performed before the age of 10years. Skin was not improved. Malnutrition was observed in 50% of the children with generalized RDEB. Protein and energy needs are particularly high. GTF is well tolerated and helps with catch-up growth and puberty. It must be considered before malnutrition onset, and, if necessary, before puberty.
    British Journal of Dermatology 09/2011; 166(2):354-61. · 3.76 Impact Factor
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    ABSTRACT: Lymphoblastic lymphoma (LBL) is a rare malignant neoplasm usually occurring in the mediastinum of children and adolescents. The B-cell immunophenotype of LBL (B-LBL) accounts for less than 20% of all cases and may involve extramediastinal areas, such as the skin. Although highly aggressive, LBL is potentially curable if diagnosed early. We sought to describe the clinical and histopathologic features of B-LBL in children presenting with cutaneous lesions, and to highlight the specific features of this rare and serious disease. Seven children with a confirmed diagnosis of cutaneous B-LBL were identified by retrospective chart review. The clinical and histopathologic features were documented, analyzed, and compared with cases previously published in the literature. Six children developed nodules on the head, and one child presented with lesions on the back and abdomen. Histopathology showed a diffuse dermal and subcutaneous monomorphous infiltrate made up of atypical cells with an immature B-cell phenotype. The average duration of the lesions before diagnosis was 3.2 months. A staging workup revealed extracutaneous disease in 5 patients, including bone-marrow involvement in 4 children. This was a retrospective study with a small number of patients. The cutaneous lesions of B-LBL typically manifest as rapidly growing erythematous firm nodules located on the head. Awareness of these clinical features is important for the diagnosis to be reached rapidly and treatment started without delay.
    Journal of the American Academy of Dermatology 07/2011; 66(1):51-7. · 4.91 Impact Factor
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    ABSTRACT: Recent identification of STAT3 mutations in autosomal dominant (AD) hyper-IgE syndrome (HIES) has improved the clinical, genetic, and molecular classification of the HIES. We sought to characterize the cutaneous signs observed in molecularly diagnosed AD-HIES. We conducted a retrospective study of 21 patients with AD-HIES and confirmed STAT3 mutations, treated at Necker-Enfants Malades Hospital, Paris, France. A papulopustular rash on the face and scalp before the age of 2 months was observed in 67% of patients. This "early rash" was distinguished from other neonatal pustular eruptions by crusted papules and pustules, rash intensity, and a continuum with chronic dermatitis. An eczematous dermatitis was almost always present before the age of 18 months (95% of patients) and was mainly confined to the face, scalp, chest, and buttocks. All patients presented with infected dermatitis (Staphylococcus aureus) and 59% had chronic candidiasis of the oral mucosa and nails. Cutaneous herpes virus infections were not unusually severe. Coarse facial skin at puberty, and sometimes at a younger age, with prominent follicular ostia resembling atrophoderma vermiculatum was not related to severe acne or facial abscesses. This was a retrospective study with a small number of patients. When associated with serum IgE levels 10 times the age-appropriate level, a neonatal papulopustular rash progressing to a chronic impetiginized eczematous dermatitis that differs from classic atopic dermatitis is highly suggestive of AD-HIES. Early recognition is important for initiation of prophylactic antistaphylococcal and antifungal treatment.
    Journal of the American Academy of Dermatology 06/2011; 65(6):1167-72. · 4.91 Impact Factor
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    ABSTRACT: Incontinentia pigmenti (IP) is a multisystem disorder, in which cutaneous symptoms can be accompanied by dental, ocular, and central nervous system defects. In adults, the clinical diagnosis of IP is based principally on the late onset of stage 4 lesions and their association with dental, nail, ocular, or central nervous system anomalies. Nevertheless, these lesions are often unrecognized. Our aim was assessment of IP manifestations in adults to clarify diagnostic criteria for mild forms of the disease, to help physicians detect adult IP in the presence of subtle lesions and avoid misdiagnosis. We conducted clinical and histologic examination of 25 adults with IP and nuclear factor-κB essential modulator gene rearrangement or mutations. Linear atrophic, hypopigmented, and hairless lesions (stage 4) are constant in adults. Apoptotic keratinocytes in the epidermis or dermis and atrophic hair follicles, with absence of arrector pili muscles, are frequently observed. In contrast, nipple anomalies are rare. We were unable to determine the age of the onset of IP stage 4 lesions. Skin manifestations are constant in adult patients with IP. Histology is characteristic and could be considered as a minor diagnostic criterion of IP. Nipple anomalies also may be considered as a minor criterion. Detection of such subtle manifestations can evoke IP in patients with repeated miscarriages or unexplained neurologic manifestations.
    Journal of the American Academy of Dermatology 03/2011; 64(3):508-15. · 4.91 Impact Factor
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    ABSTRACT: To determine and list the clinical and pathological features of cutaneous hematologic diseases in childhood. We retrospectively analyzed the data for 51 patients up to 15 years of age, who presented with primary cutaneous hematologic disorders according to the WHO-EORTC classification, at Necker-Enfants Malades Hospital, Paris, France, over a 17-year period. The cases were classified into the following diagnostic categories: CD30+ T-cell lymphoproliferative disorders (24) all consisting of lymphomatoid papulosis (LyP, 24), lymphoblastic lymphoma (LL, 7), acute leukemias (AL, 7), mycosis fungoides (MF, 5), Epstein-Barr virus-related lymphoproliferative disorders (EBV-related LPD, 5), T/NK-cell lymphoma, nasal type (1), γ/δ T-cell lymphoma (1), and panniculitis-like T-cell lymphoma (1). We encountered a majority of LyP. No secondary lymphoma was found in LyP patients with a median follow-up of 8 years. 29% and 80% of LyP and MF patients, respectively, presented with pityriasis lichenoides chronica (PLC) before onset of disease. Owing to a frequently misleading clinicopathological presentation, MF patients were first underdiagnosed. Clinicopathological features of LL and AL were highly stereotypical, leading to the diagnosis being suspected and confirmed more promptly. In the latter patients and in EBV-related LPD patients, skin lesions usually led to the discovery of systemic disease. Distribution of cutaneous hematologic diseases seems to be different in adults and in children aged <15-year old. PLC was strongly correlated with MF and LyP. Physicians must be made aware of the stereotypical clinical presentations of LL and AL to allow prompt diagnosis and treatment.
    Pediatric Blood & Cancer 03/2011; 58(2):226-32. · 2.35 Impact Factor
  • Energy Economics - ENERG ECON. 01/2011; 59.
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    ABSTRACT: Fibrodysplasia ossificans progressiva (FOP) is a rare genetic disorder characterized by progressive ossification of soft tissues. Clinical diagnosis is important because trauma from lesional biopsies can exacerbate the disease. We sought to evaluate the frequency of scalp nodules as the presenting manifestation of FOP. We describe 3 infants with FOP who presented with multiple neonatal scalp nodules. We reviewed all 43 cases of this disorder in the French FOP registry. Scalp nodules were found in 40% of cases and usually represented the first manifestation of the disease. All 43 patients had characteristic skeletal malformations involving the great toes (n = 43), fingers (n = 12), and vertebrae (n = 3). Other abnormalities were cerebral malformations (n = 1) and alopecia (n = 2). Histopathologic analysis did not contribute to the differential diagnosis and was interpreted as cranial fasciitis in two patients. Our study was retrospective, and the presence or absence of scalp nodules was not always recorded. Neonatal scalp nodules associated with a characteristic malformation of the great toes are a common presentation of FOP. Physicians should be aware that lesional biopsies can exacerbate the disease and must therefore be avoided. A diagnosis of classic FOP can be confirmed by molecular genetic studies.
    Journal of the American Academy of Dermatology 11/2010; 64(1):97-101. · 4.91 Impact Factor
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    ABSTRACT: Hypohidrotic and anhidrotic ectodermal dysplasia (HED/EDA) is a rare genodermatosis characterized by abnormal development of sweat glands, teeth, and hair. Three disease-causing genes have been hitherto identified, namely, (1) EDA1 accounting for X-linked forms, (2) EDAR, and (3) EDARADD, causing both autosomal dominant and recessive forms. Recently, WNT10A gene was identified as responsible for various autosomal recessive forms of ectodermal dysplasias, including onycho-odonto-dermal dysplasia (OODD) and Schöpf-Schulz-Passarge syndrome. We systematically studied EDA1, EDAR, EDARADD, and WNT10A genes in a large cohort of 65 unrelated patients, of which 61 presented with HED/EDA. A total of 50 mutations (including 32 novel mutations) accounted for 60/65 cases in our series. These four genes accounted for 92% (56/61 patients) of HED/EDA cases: (1) the EDA1 gene was the most common disease-causing gene (58% of cases), (2)WNT10A and EDAR were each responsible for 16% of cases. Moreover, a novel disease locus for dominant HED/EDA mapped to chromosome 14q12-q13.1. Although no clinical differences between patients carrying EDA1, EDAR, or EDARADD mutations could be identified, patients harboring WNT10A mutations displayed distinctive clinical features (marked dental phenotype, no facial dysmorphism), helping to decide which gene should be first investigated in HED/EDA.
    Human Mutation 10/2010; 32(1):70-2. · 5.21 Impact Factor
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    ABSTRACT: Tufted angioma (TA) is a rare benign vascular tumor that mostly appears during infancy or early childhood. Histologic tufts of capillaries infiltrating the whole dermis in a "cannonball" distribution pattern associated with dilated lymphatic vessels are characteristic of the disease and confirm the diagnosis. Few case series of TA have been published, and the morphologic structure and evolution of TA seem to vary. We describe the largest series to date of childhood TA, comprising 13 cases. All children developed lesions within the first year of life; 7 cases were congenital. We found a clear male predominance (9 of 13 children). Presentation was a nascent or florid tumor, usually a dusky red to violaceous plaque, that was indurated, firm, and sometimes associated with hyperhidrosis or hypertrichosis. Locations of the lesions included limbs, abdomen, and genitalia. Five children had spontaneous regression, 5 children had Kasabach-Merritt syndrome, and 1 child had a lesion that stabilized. Two children with painful TA had chronic coagulopathy without thrombocytopenia that was controlled by ticlopidine hydrochloride and aspirin. The following 3 clinical patterns could be distinguished: TA without complications, TA complicated by Kasabach-Merritt syndrome, and TA without thrombocytopenia but with chronic coagulopathy. To our knowledge, this study is the first to describe the third pattern. Because of the aggressive nature of Kasabach-Merritt syndrome, it is essential to obtain a complete blood cell count when evaluating a child with TA.
    Archives of dermatology 07/2010; 146(7):758-63. · 4.76 Impact Factor
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    ABSTRACT: Keratitis-ichthyosis-deafness (KID) syndrome is an autosomal dominant congenital ectodermal defect characterized by the association of skin lesions, hearing loss and keratitis. Most of the cases appear to be sporadic. KID syndrome is mostly related to mutations of GJB2 gene encoding connexin-26. Recently, a lethal form of the disease during the first year of life has been reported in two unrelated Caucasian patients. This rare lethal form is caused by the G45E mutation of GJB2 gene. We here report the first pre-natal molecular genetic diagnosis of the lethal form of KID syndrome relating to a G45E mutation. In the same family, the occurrence of this condition in three other siblings born to African non-consanguineous healthy parents lead to perform pre-natal diagnosis for this last pregnancy. Molecular analysis confirms the diagnosis of the lethal form of KID for the fetus. These results establish the role of germline mosaicism in KID syndrome and warrant careful genetic counseling. Furthermore, analysis of our cases and the literature allowed us to define a characteristic severe neonatal phenotype including facial dysmorphy, severe cornification with massive focal hyperkeratosis of the skin with erythroderma, dystrophic nails, complete atrichia and absence of foreskin.
    Clinical Genetics 06/2010; 77(6):587-92. · 4.25 Impact Factor
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    ABSTRACT: To describe clinical and immunohistochemical findings in patients with cutaneous Langerhans cell histiocytosis (LCH) beginning in the first 3 months of life and to define predictors of disease evolution. Observational retrospective survey from July 15, 1989, to April 30, 2007. Referral center in pediatric dermatology. Thirty-one patients with a diagnosis of cutaneous LCH in the first 3 months of life and no previous visceral LCH. Cutaneous lesion characteristics, regulatory T-lymphocyte density, and E-cadherin expression were assessed. Data were compared between the patient groups with self-regressive vs non-self-regressive forms of cutaneous LCH. Pathologic analysis was performed blinded to patient group. Self-regressive cutaneous LCH was found in 21 patients and non-self-regressive cutaneous LCH in 10 patients. Monolesional forms, necrotic lesions, hypopigmented macules at presentation, and distal topography of limb lesions were seen only in patients with self-regressive cutaneous LCH. Regulatory T-lymphocyte density correlated with interleukin 10 expression in lesions (r = 0.77, P = .003) but was not predictive of disease evolution. E-cadherin expression by Langerhans cells was found in 7 patients with disease limited to the skin whether self-regressive or not. One patient with secondary disseminated disease showed loss of E-cadherin expression in Langerhans cells. Some morphologic traits of skin lesions can orient the diagnosis to a self-regressive form of cutaneous LCH. Regulatory T-lymphocyte density does not seem to be predictive of disease evolution. E-cadherin expression seems to be an indicator of limited skin disease but not of disease regression. Additional immunohistochemical study is required to confirm these data.
    Archives of dermatology 02/2010; 146(2):149-56. · 4.76 Impact Factor
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    ABSTRACT: Highly vascularized malignant soft-tissue tumors can clinically and radiologically mimic deep hemangiomas. We present a case of congenital rhabdomyosarcoma of the neck, which was initially identified as congenital hemangioma.
    Pediatric Dermatology 01/2010; 28(3):299-301. · 1.04 Impact Factor
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    ABSTRACT: Adult mastocytosis is an incurable clonal disease associated with c-KIT mutations, mostly in exon 17 (D816V). In contrast, pediatric mastocytosis often spontaneously regresses and is considered a reactive disease. Previous studies on childhood mastocytosis assessed only a few patients and focused primarily on codon 816 mutations, with various results. In this study, we analyzed the entire c-KIT sequence from cutaneous biopsies of 50 children with mastocytosis (ages 0-16 years). A mutation of codon 816 (exon 17) was found in 42% of cases, and mutations outside exon 17 were observed in 44%. Unexpectedly, half of the mutations were located in the fifth Ig loop of c-KIT's extracellular domain, which is encoded by exons 8 and 9. All mutations identified in this study were somatic and caused a constitutive activation of c-KIT. There was no clear phenotype-genotype correlation, no clear relationship between the mutations and familial versus spontaneous disease, and no significant change in the relative expression of the c-KIT GNNK+ and GNNK isoforms. These findings strongly support the idea that, although pediatric mastocytosis can spontaneously regress, it is a clonal disease most commonly associated with activating mutations in c-KIT.
    Journal of Investigative Dermatology 10/2009; 130(3):804-15. · 6.19 Impact Factor
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    ABSTRACT: Hypochromic streaks can be the only cutaneous sign of incontinentia pigmenti (IP) in adulthood (stage IV). Discovery of such lesions in an adult female with no family history of IP is essential for appropriate genetic counselling. To describe and to validate the histological features of residual skin lesions in adult IP. The analysis and comparison of skin biopsies of 26 women affected with molecularly confirmed IP. Results: Most biopsies showed slight atrophy and some scattered apoptotic cells in the epidermis, epidermal hypopigmentation and reduced melanocyte number. The dermis appeared thickened and homogeneous and revealed a complete absence of hair follicles (23/26) and sweat glands (22/26). There was no melanin incontinence or inflammatory cells, and the elastic network was normal. These features lead unequivocally to the diagnosis of a stage IV IP skin lesion. Consequently, histology is a major confirmatory criterion for diagnoses of these mild clinical forms of IP. It is therefore a useful tool in genetic counselling and prenatal diagnosis. Moreover, the observations described here may contribute to understanding the physiopathology of the late stages of IP.
    Journal of Cutaneous Pathology 10/2009; 36(9):966-71. · 1.77 Impact Factor
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    ABSTRACT: A multicentre randomized double-blind study of 143 patients was conducted to compare terbinafine (250 mg/day) and micronized griseofulvin (1 g/day) in the treatment of dermatophyte onychomycosis. The duration of treatment was up to 12 months, but the treatment could be interrupted in the event of complete cure, i.e. clinical disappearance of the pathological zone of the nail combined with mycological cure. The percentage of nails cured was significantly higher in the terbinafine than in the griseofulvin group (77.1% versus 45.7%, P=0.0001). Clinical and biological tolerability were good in both groups. Only 7.4% of patients treated with terbinafine showed one or more adverse events, but 31.5% with griseofulvin (P<0.001).
    07/2009; 8(2):93-97.
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    Nature Genetics 07/2009; 41(6):762. · 35.21 Impact Factor

Publication Stats

3k Citations
692.15 Total Impact Points

Institutions

  • 1994–2014
    • Université René Descartes - Paris 5
      • Faculté de Médecine
      Lutetia Parisorum, Île-de-France, France
    • Center for Molecular Genetics
      Gif, Île-de-France, France
  • 2004–2011
    • Assistance Publique – Hôpitaux de Paris
      Lutetia Parisorum, Île-de-France, France
  • 2009
    • Procter & Gamble
      Cincinnati, Ohio, United States
    • Military Hospital of Instruction of Tunis
      Tunis-Ville, Tūnis, Tunisia
  • 2007
    • Rady Children's Hospital
      San Diego, California, United States
  • 2006
    • Unité Inserm U1077
      Caen, Lower Normandy, France
    • Claude Bernard University Lyon 1
      Villeurbanne, Rhône-Alpes, France
    • Novartis
      Bâle, Basel-City, Switzerland
  • 2005
    • Probity Medical Research Inc.
      Waterloo, Ontario, Canada
    • University of Waterloo
      Waterloo, Ontario, Canada
    • SickKids
      Toronto, Ontario, Canada
  • 1989–2005
    • Hôpital Universitaire Necker
      • Service de Dermatologie
      Lutetia Parisorum, Île-de-France, France
  • 2003
    • Concordia University–Ann Arbor
      Ann Arbor, Michigan, United States
  • 2002
    • Wellcome Trust
      Londinium, England, United Kingdom
    • Humboldt-Universität zu Berlin
      Berlín, Berlin, Germany
    • University of Helsinki
      • Department of Dermatology
      Helsinki, Province of Southern Finland, Finland
  • 1997–2000
    • University of Oxford
      • Wellcome Trust Centre for Human Genetics
      Oxford, ENG, United Kingdom
  • 1998
    • University of Limoges
      Limages, Limousin, France
  • 1989–1990
    • Hôpital Henri Mondor (Hôpitaux Universitaires Henri Mondor)
      • Service de Dermatologie
      Créteil, Ile-de-France, France