Y de Prost

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

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Publications (270)781.78 Total impact

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    ABSTRACT: Kasabach-Merritt phenomenon (KMP) is a rare life-threatening vascular condition of infancy. Prognosis factors and long-term follow-up data are lacking. We retrospectively analysed the records of 24 infants (10 females, 14 males) treated for KMP in the Department of Dermatology of Necker-Enfants Malades Hospital, Paris, France, from 1984 to 2012. Mean duration of thrombocytopaenia (2,000-38,000 platelets/mm3, mean 10,500/µl) was 8.8 months (range 3 days-84 months), which correlated with tumour infiltration depth on imaging. D-dimer levels were always elevated, even before KMP onset. Each patient received a mean of 4.8 different treatments (range 1-10). Median follow-up was 6.5 years (range 2 months-22 years). All infants had residual cutaneous lesions, along with inflammatory manifestations (n = 9), elevated D-dimer (n = 5) and orthopaedic sequelae (n = 5). The permanent coagulopathy (elevated D-dimer) even after resolution of KMP suggests the presence of chronic low-grade platelet trapping, with possible sudden worsening, and raises the possibility of prophylactic anti-platelet therapy.
    No preview · Article · Jun 2015 · Acta Dermato-Venereologica
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    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.
    No preview · Article · Apr 2014 · British Journal of Dermatology

  • No preview · Article · Dec 2013 · Annales de Dermatologie et de Vénéréologie

  • No preview · Article · Dec 2013 · Annales de Dermatologie et de Vénéréologie

  • No preview · Article · Feb 2013 · The American Journal of dermatopathology

  • No preview · Article · Feb 2013 · The American Journal of dermatopathology
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    ABSTRACT: The role of imaging of the vascular anomalies in children has been transformed by the development of the non invasive techniques; CT and MRI that is not using ionizing radiations allow to obtain the majority of answers that are necessary to the diagnosis and therapeutic strategy. What is the nature of the lesion, its localisation, its extension, and its evolutivity. Imaging allows a classification of the anomaly thanks to the identification of the involved vessels and their hemodynamic basis of the ISSVA classification. Imaging is only useful in rare cases as in most of the time the diagnosis is established by clinical examination and Doppler.
    No preview · Article · Jan 2013 · Sang Thrombose Vaisseaux

  • No preview · Article · Dec 2012 · Annales de Dermatologie et de Vénéréologie
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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.
    No preview · Article · Feb 2012 · Pediatric Blood & Cancer

  • No preview · Article · Dec 2011 · Annales de Dermatologie et de Vénéréologie
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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.
    No preview · Article · Oct 2011 · Journal of the American Academy of Dermatology
  • Y. de Prost · S. Aractingi · M. Picot · L. Martin · C. Philippe · M. Fatras

    No preview · Article · Oct 2011 · Revue d Épidémiologie et de Santé Publique
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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.
    No preview · Article · Sep 2011 · British Journal of Dermatology
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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.
    No preview · Article · Jul 2011 · Journal of the American Academy of Dermatology
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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.
    No preview · Article · Jun 2011 · Journal of the American Academy of Dermatology
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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.
    No preview · Article · Mar 2011 · Journal of the American Academy of Dermatology
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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.
    Full-text · Article · Jan 2011 · Human Mutation
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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.
    No preview · Article · Nov 2010 · Journal of the American Academy of Dermatology
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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.
    No preview · Article · Jul 2010 · Archives of dermatology
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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.
    No preview · Article · Jun 2010 · Clinical Genetics

Publication Stats

5k Citations
781.78 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
  • 2013
    • Institut Curie
      Lutetia Parisorum, Île-de-France, France
  • 2007-2010
    • Hôpitaux Universitaires La Pitié salpêtrière - Charles Foix
      Lutetia Parisorum, Île-de-France, France
  • 2004-2007
    • Assistance Publique – Hôpitaux de Paris
      Lutetia Parisorum, Île-de-France, France
  • 2005
    • SickKids
      Toronto, Ontario, Canada
  • 1989-2005
    • Hôpital Universitaire Necker
      • Service de Dermatologie
      Lutetia Parisorum, Île-de-France, France
    • Hôpital Henri Mondor (Hôpitaux Universitaires Henri Mondor)
      • Service de Dermatologie
      Créteil, Ile-de-France, France
  • 2003
    • Concordia University–Ann Arbor
      Ann Arbor, Michigan, United States
  • 2002
    • Universitätsklinikum Tübingen
      Tübingen, Baden-Württemberg, Germany