S Ingen-Housz-Oro

Hôpital Albert Chenevier – Hôpitaux Universitaires Henri Mondor, Créteil, Île-de-France, France

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Publications (145)304.93 Total impact

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    ABSTRACT: Background: Two pemphigus severity scores, Autoimmune-Bullous-Skin-Disorder-Intensity-Score (ABSIS) and Pemphigus-Disease-Area-Index (PDAI), have been proposed to provide an objective measure of disease activity. However, the use of these scores in clinical practice is limited by the absence of cut-off values which allow differentiation between moderate, significant and extensive types of pemphigus. Objective: To calculate cut-off values defining moderate, significant and extensive pemphigus based on the ABSIS and PDAI scores. Methods: In 31 Dermatology Departments in six countries, consecutive patients with newly diagnosed pemphigus were assessed for pemphigus severity, using the ABSIS, PDAI, Physician-Global-Assessment (PGA) and Dermatology-Life-Quality-Index (DLQI) scores. Cut-off values defining moderate, significant and extensive subgroups were calculated based on the 25(th) and 75(th) percentiles of the ABSIS and PDAI scores. Median ABSIS, PDAI, PGA and DLQI scores of the three severity subgroups were compared to validate these subgroups. Results: Ninety-six patients with pemphigus vulgaris (n=77) or pemphigus foliaceus (n=19) were included. Median PDAI activity and ABSIS total scores were 27.5 [range:3-84] and 34.8 points [range:0.5-90.5], respectively. Cut-off values corresponding to the first and third quartiles of the scores were 15 and 45 for the PDAI, and 17 and 53 for the ABSIS. The moderate, significant and extensive subgroups were thus defined, and had distinguishing median ABSIS (p<0.0001), PDAI (p<0.0001), PGA (p<0.0001) and DLQI (p=0.03) scores. Conclusions: This study suggests cut-off values of 15/45 and 17/53 for PDAI and ABSIS respectively, to distinguish moderate, significant and extensive pemphigus forms. Identifying these pemphigus activity subgroups should help physicians to classify and manage pemphigus patients. This article is protected by copyright. All rights reserved.
    No preview · Article · Jan 2016 · British Journal of Dermatology
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    ABSTRACT: The classification of cutaneous follicular lymphoma (CFL) into primary cutaneous follicle center lymphoma (PCFCL) or secondary cutaneous follicular lymphoma (SCFL) is challenging. SCFL is suspected when tumor cells express BCL2 protein, reflecting a BCL2 translocation. However, BCL2 expression is difficult to assess in CFLs because of numerous BCL2 reactive T cells. To investigate these issues and to further characterize PCFCL, we studied a series of 25 CFLs without any extracutaneous disease at diagnosis, selected on the basis of BCL2 protein expression using 2 BCL2 antibodies (clones 124 and E17) and BOB1/BCL2 double immunostaining. All cases were studied using interphase fluorescence in situ hybridization with BCL2, BCL6, IGH, IGK, IGL breakapart, IGH-BCL2 fusion, and 1p36/1q25 dual-color probes. Nineteen CFLs were BCL2 positive, and 6 were negative. After a medium follow-up of 24 (6 to 96) months, 5 cases were reclassified as SCFL and were excluded from a part of our analyses. Among BCL2 PCFCLs, 60% (9/15) demonstrated a BCL2 break. BCL2-break-positive cases had a tendency to occur in the head and neck and showed the classical phenotype of nodal follicular lymphoma (CD10, BCL6, BCL2, STMN) compared with BCL2-break-negative PCFCLs. Del 1p36 was observed in 1 PCFCL. No significant clinical differences were observed between BCL2 or BCL2 PCFCL. In conclusion, we show that a subset of PCFCLs harbor similar genetic alterations, as observed in nodal follicular lymphomas, including BCL2 breaks and 1p36 deletion. As BCL2 protein expression is usually associated with the presence of a BCL2 translocation, fluorescence in situ hybridization should be performed to confirm this hypothesis.
    No preview · Article · Jan 2016 · American Journal of Surgical Pathology
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    ABSTRACT: Background. Lymphomatoid papulosis (LyP) is classified as an indolent cutaneous lymphoma, but outcome dramatically worsens if LyP is associated with lymphoma. The frequency of this association remains unclear in the literature. Here, we assess the frequency and risk factors of association between LyP and another lymphoma in an 11-year retrospective study conducted in 8 dermatology departments belonging to the French Study Group on Cutaneous Lymphoma (FSGCL). Patients and Methods. Patients with LyP were identified and data extracted from the FSGCL registry between 1991 and 2006. Patients were followed up to January 2014. Age, sex, number of skin lesions, histologic subtype, and genotype were recorded at baseline. Risk factors were determined using univariate and multivariate analysis. Cumulative probability of association was calculated using the Kaplan-Meier method. Results.Weobserved 52 cases of lymphomas (cutaneous, n= 38; systemic, n = 14) in 44 of 106 patients (41%). Lymphoma diagnosis was concomitant with or prior to LyP diagnosis in 31 cases and occurred during the course of LyP in 21 cases (cutaneous, n = 14; systemic, n = 7; median delay: 5 years; interquartile range: 1.5–7 years). In multivariate analysis, main prognostic factors for association between LyP and another lymphoma were older age (odds ratio [OR]: 1.05 per year; 95% confidence interval [CI]: 1.01–1.08;p=.011)and presence of a T-cell clone in LyP lesions (OR: 7.55; 95% CI: 2.18–26.18; p =.001). Conclusion. Older age and presence of a T-cell clone in LyP lesions are risk factors for associated lymphomas in patients with LyP. These findings should help to identify patients who require close management in clinical practice.
    No preview · Article · Dec 2015 · The Oncologist

  • No preview · Article · Dec 2015 · Annales de Dermatologie et de Vénéréologie
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    ABSTRACT: Background: The course of dermatomyositis (DM) can be chronic with relapses, which are associated with major morbidity. Objective: The aim of this study was to identify presentation features that predict DM relapses. Methods: We retrospectively reviewed data of patients with DM recorded from 1990 to 2011, including muscle biopsy results. Characteristics of patients with and without relapses were compared. Hazard ratios (HRs) were estimated using a Cox model. Results: We identified 34 patients, with a mean age of 46 ± 17 years (range, 18-77) and 24 (71%) women. The muscle and skin abnormalities relapsed in 21 (61%) patients. By univariate analysis, two presentation features were significantly associated with a subsequently relapsing course, namely, dysphonia [HR = 3.2 (1.2-8.5)] and greater skin lesion severity defined as a Cutaneous Disease Area Severity Index [CDASI] > 20 [HR = 3.5 (1.2-7.9)]. Conclusion: Dysphonia and skin lesion severity at disease onset must be recorded, as they significantly predict a relapsing disease course.
    No preview · Article · Dec 2015 · Journal of the European Academy of Dermatology and Venereology

  • No preview · Article · Dec 2015 · Annales de Dermatologie et de Vénéréologie
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    ABSTRACT: Kikuchi-Fujimoto disease (KFD) is a rare and benign disorder that usually occurs in young adults with enlarged lymph nodes containing infiltrate of cytotoxic T cells and nuclear debris. It can be a manifestation of systemic lupus erythematosus (SLE) although the strength of this association has varied among studies. Although specific KFD cutaneous lesions are well described, pure cutaneous lesions have never been reported. We studied a series of patients prospectively entered into a database between 2007 and 2014 with skin biopsies showing diffuse or localized inflammatory infiltrates reminiscent of cutaneous KFD, without lymph-node-related KFD. We called these skin lesions "Kikuchi disease-like inflammatory pattern" (KLIP). Twenty-nine patients, whose median age was 49 years at the time of skin biopsy, were selected and retrospectively analyzed using standardized clinical and histology charts. In skin biopsies, KLIP was localized to restricted areas within the inflammatory infiltrate (17%) or diffuse (83%), and was the only histological finding (45%) or accompanied interface dermatitis with or without dermal mucinosis (55%). Clinical dermatological findings varied widely. A definite diagnosis could be established for 24 patients: 75% had connective tissue diseases or vasculitis, mainly cutaneous lupus erythematosus (CLE) (n = 16, 67%), including 5 SLE with satisfying American College of Rheumatology criteria; 3 of the remaining patients had malignant hemopathies. CLE patients were mostly young females with acute (n = 5), subacute (n = 4), or chronic CLE (n = 6) or lupus tumidus (n = 1). Two were classified as having anti-tumor necrosis factor-alpha-induced lupus. Because two-thirds of these patients were finally diagnosed with CLE, we think that KLIP may represent a new histopathological clue for the diagnosis of lupus based on skin biopsy, requiring clinical-immunological comparison to make the correct diagnosis. KLIP should not be considered a variant of classical KFD, but rather as an elementary pattern of cutaneous inflammation, that might be the expression of the same cytotoxic process within skin infiltrates as that involved in KFD. This lesion might reflect a particular T-cell-mediated autoimmune process directed against mononuclear cells within cutaneous lupus infiltrates.
    Preview · Article · Nov 2015 · Medicine
  • Saskia Ingen-Housz-Oro · Françoise Foulet · Olivier Chosidow

    No preview · Article · Nov 2015 · BMJ (online)
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    ABSTRACT: Background: Kimura disease (KD) is a rare lymphoproliferative inflammatory disease of unknown etiology. Data regarding therapeutic modalities and pathophysiology are scarce. Objectives: Analyze therapeutic and follow-up data and compare KD with cutaneous IgG4-related disease (IgG4-RD). Methods: Multicentric retrospective study of 25 KD patients with analysis of treatment, follow-up and IgG4 immunostaining. Comparison with published cases of cutaneous IgG4-RD. Results: Patients were mostly male (84%), median-aged 42 years with lymph node, lacrimal/salivary gland and kidney involvements in 45, 24 and 12%, respectively. Surgical excision had 100% complete response and 60% relapse. Oral corticosteroids had 100% response with 50% relapse. Thalidomide, cyclosporine or interferon-α had 100% response, but 100, 20 and 50% relapse, respectively. KD showed clinicopathological similarities with 27 published cases of cutaneous IgG4-RD. Conclusion: Surgery may be used in resectable KD cases, whereas cyclosporine or thalidomide may represent interesting alternatives to oral corticosteroids in other cases. KD shares features with cutaneous IgG4-RD.
    No preview · Article · Oct 2015 · Dermatology
  • S Ingen-Housz-Oro · E Sbidian · N Ortonne · E Poirier · O Chosidow · P Wolkenstein

    No preview · Article · Aug 2015 · Journal of the European Academy of Dermatology and Venereology

  • No preview · Article · Aug 2015 · The Lancet Infectious Diseases
  • Caroline Lacoste · Saskia Ingen-Housz-Oro · Nicolas Ortonne
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    ABSTRACT: Skin manifestations associated with monoclonal gammapathy are common and can present with various clinical and pathological aspects. They can be the first events leading to the diagnosis of monoclonal gammapathy. They may be present either as specific lesions, including lymphoplasmacytic or pure plasma cell neoplastic infiltrates and monoclonal immunoglobulin deposits, or as non-specific dermatitis, such as leukocytoclastic vasculitis, neutrophilic dermatoses, mucinoses or xanthomatosis, giving little clues for the diagnosis of the underlying disease. Copyright © 2015 Elsevier Masson SAS. All rights reserved.
    No preview · Article · Jul 2015 · Annales de Pathologie
  • S Ingen-Housz-Oro · N Ortonne

    No preview · Article · Jul 2015 · Annales de Dermatologie et de Vénéréologie
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    ABSTRACT: Monoclonal T-cell receptor (TCR) rearrangement is detected in 57% to 75% of early-stage mycosis fungoides (MF) at diagnosis. A retrospective study showed molecular residual disease (MRD) in 31% of patients in complete clinical remission (CR) after 1 year of treatment. To confirm the frequency of MRD at 1 year and to determine its prognostic value for further relapse. Patients with T1-, T2- or T4-stage MF were prospectively included in this multicenter study. At diagnosis, clinical lesions and healthy skin were biopsied. After 1 year of topical treatment, previously involved skin (PIS) of patients in CR was biopsied for histology and analysis of TCR-γ gene rearrangement. Results were compared to the clinical status each year for 4 years. We included 214 patients, 133 at T1, 78 T2, and 3 T4 stage. At diagnosis, 126/204 cases (61.8%) showed TCR clonality in lesional skin. After 1 year, 83/178 patients (46.7%) still being followed up were in CR and 13/63 (20.6%) showed MRD. At 4 years, 55/109 (50.5%) patients still being followed up were in CR and 44/109 were in T1 stage (40.4%). MRD did not affect clinical status at 4 years (CR vs. T1/T2, p=1.0, positive predictive value 36.4%, negative predictive value 67.6%). T-cell clonality at diagnosis and MRD at 1 year are not a prognostic factor of clinical status at 4 years. This article is protected by copyright. All rights reserved. This article is protected by copyright. All rights reserved.
    No preview · Article · Jul 2015 · British Journal of Dermatology
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    ABSTRACT: Invasive aspergillosis (IA) has poor prognosis in immunocompromised patients. Skin manifestations, when present, should contribute to an early diagnosis. The authors aimed to provide prevalence data and a clinical and histologic description of cutaneous manifestations of primary cutaneous IA (PCIA) and secondary CIA (SCIA) in a unique clinical series of IA and present the results of an exhaustive literature review of CIA. Cases of proven and probable IA with cutaneous manifestations were retrospectively extracted from those registered between 2005 and 2010 in a prospective multicenter aspergillosis database held by the National Reference Center for Invasive Mycoses and Antifungals, Pasteur Institute, France. Patients were classified as having PCIA (i.e., CIA without extracutaneous manifestations) or SCIA (i.e., disseminated IA). Among the 1,410 patients with proven or probable IA, 15 had CIA (1.06%), 5 PCIA, and 10 SCIA. Hematological malignancies were the main underlying condition (12/15). Patients with PCIA presented infiltrated and/or suppurative lesions of various localizations not related to a catheter site (4/5), whereas SCIA was mainly characterized by disseminated papules and nodules but sometimes isolated nodules or cellulitis. Histologic data were available for 11 patients, and for 9, similar for PCIA and SCIA, showed a dense dermal polymorphic inflammatory infiltrate, with the epidermis altered in PCIA only. Periodic acid Schiff and Gomori-Grocott methenamine silver nitrate staining for all but 2 biopsies revealed hyphae compatible with Aspergillus. Aspergillus flavus was isolated in all cases of PCIA, with Aspergillus fumigatus being the most frequent species (6/10) in SCIA. Two out 5 PCIA cases were treated surgically. The 3-month survival rate was 100% and 30% for PCIA and SCIA, respectively. Our study is the largest adult series of CIA and provides complete clinical and histologic data for the disease. Primary cutaneous IA should be recognized early, and cases of extensive necrosis should be treated surgically; its prognosis markedly differs from that for SCIA. Any suppurative, necrotic, papulonodular, or infiltrated skin lesion in an immunocompromised patient should lead to immediate biopsy for histologic analysis and mycological skin direct examination and culture.
    Full-text · Article · Jul 2015 · Medicine
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    ABSTRACT: Sézary syndrome is a cutaneous T-cell lymphoma characterized by erythroderma and leukemic involvement. We sought to define the clinical, biologic, and histopathologic features of Sézary syndrome without erythroderma. Features of patients with Sézary syndrome and normal-appearing skin or stage-T1 patches, fulfilling Sézary syndrome hematologic criteria and with histologically documented disease in normal-appearing skin were collected. Expression of Sézary syndrome molecular biomarkers in peripheral blood and skin lymphocytes were studied. Five women and 1 man (median age: 71 years) were all referred for generalized pruritus. Four had no specific lesions; 2 had T1-stage patches. Histologic examination of normal-appearing skin from all patients showed lesions compatible with Sézary syndrome. Peripheral blood lymphocytes from 3 of 4 patients tested strongly expressed PLS3, Twist-1, and KIR3DL2. All normal-appearing skin biopsy specimens expressed programmed death-1. Median follow-up was 9 years. Although no patient developed erythroderma, tumors, or abnormal lymph nodes, specific skin lesions appeared in all patients during follow-up. Only 1 death, unrelated to Sézary syndrome, occurred. Retrospective design and small sample size are limitations. Sézary syndrome without erythroderma is a rare entity that may have a better prognosis than classic Sézary syndrome. Copyright © 2014 American Academy of Dermatology, Inc. Published by Elsevier Inc. All rights reserved.
    No preview · Article · Jun 2015 · Journal of the American Academy of Dermatology

  • No preview · Article · Jun 2015
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    ABSTRACT: ELISA-BP180 values and direct immunofluorescence (DIF) are prognostic factors for relapse after treatment cessation in bullous pemphigoid (BP). To determine the relevance of ELISA-BP230 antibodies for predicting relapse 6 months after treatment cessation. We retrospectively selected patients with BP and available data from ELISA-BP180 and -BP230 and DIF performed at treatment cessation. The rate of relapse was calculated at 6 months. We compared ELISA-BP180 and -BP230 values and DIF in patients with relapse and remission. We included 97 patients. At 6 months, 25.6% of patients showed relapse. The proportion of patients with an ELISA-BP230 value ≥27 UA/ml was higher, but not significantly, for those with relapse than for those with remission (p = 0.11). The frequency of positive DIF findings was significantly higher for patients with relapse (p = 0.005). DIF is of better value than ELISA-BP180 and -230 tests to predict relapse after treatment cessation in BP. © 2015 S. Karger AG, Basel.
    No preview · Article · Apr 2015 · Dermatology
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    ABSTRACT: Ear, nose, and throat (ENT) lesions are frequently involved in Stevens-Johnson syndrome and toxic epidermal necrolysis (SJS/TEN), although a detailed description is lacking in the literature. To describe ENT lesions at the acute stage and follow-up in a large series of patients with SJS/TEN and identify factors associated with the severe ENT form. Retrospective study of 49 patients with SJS/TEN hospitalized in a referral care center from 2005 to 2010. Patients who underwent a full ENT workup including examination and a nasal fiberoptic endoscopy by an otorhinolaryngologist in the acute phase and during follow-up at 2 and 12 months were included in the study. Recorded variables included maximal body surface area (BSA) detachment, SCORTEN (Score of Toxic Epidermal Necrosis [a severity of illness score]), sites and type of ENT mucosal lesions, intensive care unit transfer, pulmonary infection, and mortality. "Severe ENT form" was defined by the occurrence of laryngeal lesions with the risk of airways obstruction. Clinical characteristics associated with severe ENT form were analyzed in univariate and multivariable analysis. Of the 49 patients who underwent a full ENT workup (female to male ratio, 1.1:1), ENT symptoms (eg, odynophagia, dysphagia, dysphonia, dyspnea, earache, nasal obstruction) occurred in 48 (98%). Dyspnea or dysphonia were significantly associated with severe ENT form (21% [P = .03] and 50% [P < .001], respectively). Topographic frequencies of lesions were as followed: lips and oral cavity (n = 46 [93%]) and pharynx and vestibule of the nose (n = 26 [53%]). Fourteen patients (29%) had severe ENT form. Findings for other recorded variables for those with vs without ENT examination are as follows: maximal BSA detachment (20% [0%-95%] vs 5.5% [0%-95%]; P = .004), SCORTEN (1 [0-5] vs 1 [0-5]; P = .54), intensive care unit transfer (10 [20%] vs 9 [19%]; P = .80), pulmonary infection (9 [18%] vs 6 [13%]; P = .10), and mortality (3 [6%] vs 5 [10%]; P = .70). In multivariable analysis, pulmonary infection was significantly associated with severe ENT form (odds ratio, 5.9 [95% CI, 1.1-32.8] [P = .04]). After remission of SJS/TEN, a complete ENT mucosal healing occurred in 36 patients (74%) at 2 months and in nearly all patients (n = 48 [98%]) at 1 year of follow-up. Severe ENT form is associated with pulmonary infection and is easily detected by nasal fiberoptic endoscopy. ENT evaluation should be suggested when dysphonia or dyspnea is observed at the acute stage of SJS/TEN.
    No preview · Article · Feb 2015 · JAMA Dermatology
  • S. Hüe · S. Ingen-Housz-Oro · L. Fardet

    No preview · Article · Jan 2015 · Annales de Dermatologie et de Vénéréologie

Publication Stats

712 Citations
304.93 Total Impact Points


  • 2016
    • Hôpital Albert Chenevier – Hôpitaux Universitaires Henri Mondor
      Créteil, Île-de-France, France
  • 2009-2016
    • Hôpital Henri Mondor (Hôpitaux Universitaires Henri Mondor)
      • Service de Dermatologie
      Créteil, Île-de-France, France
  • 2013-2015
    • Assistance Publique – Hôpitaux de Paris
      Lutetia Parisorum, Île-de-France, France
    • Centre Hospitalier Universitaire de Nancy
      Nancy, Lorraine, France
  • 2011
    • Université Paris-Est Créteil Val de Marne - Université Paris 12
      • Faculty of medicine
      Créteil, Île-de-France, France
  • 2010
    • University of Paris-Est
      La Haye-Descartes, Centre, France
  • 2004-2009
    • Centre Hospitalier Victor Dupouy
      Argenteuil, Île-de-France, France
  • 2002
    • French Institute of Health and Medical Research
      • Unit of Immunology, Dermatology, Oncology
      Lutetia Parisorum, Île-de-France, France