D Bessis

French Institute of Health and Medical Research, Lutetia Parisorum, Île-de-France, France

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Publications (214)454.65 Total impact

  • Annales de Dermatologie et de Vénéréologie 11/2014; · 0.60 Impact Factor
  • C. Pernet, J. Munoz, D. Bessis
    Annales de Dermatologie et de Vénéréologie 11/2014; · 0.60 Impact Factor
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    ABSTRACT: Background: Hypocomplementemic urticarial vasculitis (HUV) is an uncommon vasculitis of unknown etiology, rarely described in the literature. Patients and methods: To analyze the clinical spectrum and the therapeutic management of patients with HUV, we conducted a French nationwide retrospective study that included 57 patients with chronic urticaria, histological leukocytoclastic vasculitis and hypocomplementemia. Results: Urticarial lesions were typically erythematous papules more pruritic than painful, associated with angioedema in 51%, purpura in 35% and livedo reticularis in 14%. Extra-cutaneous manifestations included constitutional symptoms (56%), musculoskeletal (82%), ocular (56%), pulmonary (19%), gastrointestinal (18%) and kidney involvement (14%). Patients with HUV typically presented with low C1q complement level and normal C1 inhibitor level, in association with anti-C1q antibodies in 55% of patients. Hydroxychloroquine (HCQ) or colchicine seemed to be as effective as corticosteroids in 1(st) -line therapy. In patients with relapsing and/or refractory disease, cutaneous and immunological response rates seemed to be higher using immunosuppressive agents, in particular azathioprine (AZA), mycophenolate mofetil (MMF) or cyclophosphamide, while rituximab (RTX)-based regimen may have a higher efficacy. Finally, cutaneous response was strongly associated with immunological response after therapy. Conclusion: HUV represents an uncommon systemic and relapsing vasculitis with various manifestations, mainly musculoskeletal and ocular involvement associated with anti-C1q antibodies found in half of patients. The therapeutic strategy has yet to be defined. © 2014 American College of Rheumatology.
    Arthritis & rheumatology (Hoboken, N.J.). 11/2014;
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    ABSTRACT: Background Obesity is more frequent in adults with psoriasis than in the general population. Little data is available in children but this association seems to exist also in children.Objective To evaluate whether obesity was more frequent in French children (2y<age<18y) with psoriasis of any clinical type, and severity of psoriasis.Methods Multicentre case-control study performed in 23 French dermatology centres. Comorbidities in children and parents were routinely collected. Controls were children without chronic or genetic inflammatory disease matched for age, sex, and dermatology centre. We used three weight cut-offs to compare the two groups: overweight, overweight with abdominal obesity, and overweight with obesity according to French Health Authority guidelines.ResultsA total of 261 children with psoriasis and controls were included. Mean age was 9.8 years, M/F: 126/135, 42.5% of children had plaque psoriasis, 32.2% had severe psoriasis. If we compare all the frequencies of overweight children there was no difference between the two groups (20·7%in psoriasis group, vs. 17·1% in control group, p=0·18). Overweight with abdominal obesity including obesity (18·4% vs. 10·4%; p=0·009), and obesity alone (10·0% vs. 3·1%; p=0·001) were more common in psoriasis. This was not seen in patients with severe psoriasis or those with plaque psoriasis The only determinants for overweight or obesity in the psoriasis group were female sex, or to have a parent with overweight (also found in controls).Conclusion This study shows that overweight with abdominal obesity and obesity are more frequent in children with psoriasis than in controls. Risk factors are to be a female and to have a parent with overweight, similar to that seen iin French general population. Severity and clinical type of psoriasis are not associated with overweight and obesity.This article is protected by copyright. All rights reserved.
    British Journal of Dermatology 11/2014; · 3.76 Impact Factor
  • JAMA dermatology. 09/2014;
  • A. Debu, C. Girard, D. Bessis
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    ABSTRACT: A 72-year-old man with a 40-year history of discoid lupus erythematosus (DLE) localised on the face had been treated for 20 years with topical corticosteroids, tacrolimus and systemic therapies including chloroquine and hydroxychloroquine, withdrawn because of irreversible retinopathy. Thalidomide treatment lasted almost 2 years but was stopped because of peripheral neuropathy and arrhythmia. Systemic immunosuppressive therapies including methotrextate and mycophenolate mofetil were excluded because of concomitant comorbidities. Photodynamic therapy (PDT) was then considered after a 3-month washout period. The patient had erythematous, infiltrated, slightly scaly and atrophic plaques on the cheeks, chin and upper lip (Fig. 1a). The pre-treatment skin biopsy was consistent with the histological features of chronic lupus erythematosus.This article is protected by copyright. All rights reserved.
    British Journal of Dermatology 09/2014; · 3.76 Impact Factor
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    ABSTRACT: Skin involvement is reported during primary parvovirus B19 infection in adults. We sought to describe the cutaneous presentations associated with parvovirus B19 primary infection in adults. We conducted a descriptive, retrospective, multicenter study. The patients included (>18 years old) had well-established primary infections with parvovirus B19. Twenty-nine patients were identified between 1992 and 2013 (17 women, 12 men). The elementary dermatologic lesions were mostly erythematous (86%) and often purpuric (69%). Pruritus was reported in 48% of cases. The rash predominated on the legs (93%), trunk (55%), and arms (45%), with a lower frequency of facial involvement (20%). Four different but sometimes overlapping patterns were identified (45%): exanthema, which was reticulated and annular in some cases (80%); the gloves-and-socks pattern (24%); the periflexural pattern (28%); and palpable purpura (24%). The limitations of this study were its retrospective design and possible recruitment bias in tertiary care centers. Our findings suggest that primary parvovirus B19 infection is associated with polymorphous skin manifestations with 4 predominant, sometimes overlapping, patterns. The acral or periflexural distribution of the rash and the presence of purpuric or annular/reticulate lesions are highly suggestive of parvovirus B19 infection.
    Journal of the American Academy of Dermatology 04/2014; · 4.91 Impact Factor
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    ABSTRACT: First described in 1996, necrolytic acral erythema (NAE) is a rare entity characterized by well-demarcated hyperkeratotic scaly plaques involving predominantly acral sites.1 NAE is almost exclusively associated with hepatitis C virus (HCV) infection, often being considered an early cutaneous marker of this infection.2 Nevertheless, a few cases of NAE have been reported in the absence of HCV infection.3 We report the first case of NAE following hepatitis B (HBV) vaccination in a patient without associated HCV infection.This article is protected by copyright. All rights reserved.
    British Journal of Dermatology 04/2014; · 3.76 Impact Factor
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    ABSTRACT: Scabies has a clinical presentation that seems to vary according to age. We conducted a prospective study with the goal of delineating the clinical presentation of the disease into 3 groups of age: infants, <2 years; children, 2 to 15 years; and adults, >15 years. This trial was a prospective, multicenter observational study in consecutive patients with a confirmed diagnosis of scabies who were seen in 13 French Departments of Dermatology and Pediatric Dermatology between April 2010 and April 2011. A standardized questionnaire was completed for each patient. To identify factors associated with patient age, comparisons between the 3 age groups were conducted by using univariate and multivariate multinomial logistic regression analyses. A total of 323 individuals were included; the gender ratio (female:male) was 1.2:1. In univariate analysis, infants were more likely to have facial involvement. In multivariate logistic regression, relapse was more frequent in children (odds ratio [OR]: 2.45 [95% confidence interval (CI):1.23-4.88]) and infants (OR: 3.26 [95% CI: 1.38-7.71]). In addition, family members with itch (OR: 2.47 [95% CI: 1.04-5.89]), plantar (OR: 20.57 [95% CI: 7.22-58.60]), and scalp (OR: 16.94 [95% CI: 3.70-77.51]) involvement were also found to be independently associated with the age group <2 years. There is a specific clinical presentation of scabies in infants and children. Taking into account these specificities may be helpful for the early diagnosis and the identification of cases to prevent the propagation of the disease.
    PEDIATRICS 03/2014; · 4.47 Impact Factor
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    ABSTRACT: Abstract is missing (Short Communication).
    Acta Dermato-Venereologica 03/2014;
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    ABSTRACT: Three overlapping conditions, namely Rothmund-Thomson (RTS), Baller-Gerold (BGS) and RAPADILINO syndromes, have been attributed to RECQL4 mutations. Differential diagnoses depend on the clinical presentation, but the number of known genes remains low, leading to the widespread prescription of RECQL4 sequencing. The aim of our study was therefore to determine the best clinical indicators for the presence of RECQL4 mutations in a series of 39 patients referred for RECQL4 molecular analysis and belonging to the RTS (27 cases) and BGS (12 cases) spectrum. One or two deleterious RECQL4 mutations were found in 10/27 patients referred for RTS diagnosis. Clinical and molecular reevaluation led to a different diagnosis in 7/17 negative cases, including Clericuzio-type Poikiloderma with Neutropenia, hereditary sclerosing poikiloderma, and Craniosynostosis/anal anomalies/porokeratosis. No RECQL4 mutations were found in the BGS group without poikiloderma, confirming that RECQL4 sequencing was not indicated in this phenotype. One chromosomal abnormality and one TWIST mutation was found in this cohort. This study highlights the search for differential diagnoses before the prescription of RECQL4 sequencing in this clinically heterogeneous group. The combination of clinically-defined subgroups and next-generation sequencing will hopefully bring to light new molecular bases of syndromes with poikiloderma, as well as BGS without poikiloderma.
    Clinical Genetics 02/2014; · 4.25 Impact Factor
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    ABSTRACT: Aplasia cutis congenita (ACC) has been associated with all clinical forms of inherited epidermolysis bullosa (EB), including dominant and recessive dystrophic EB (DEB). To date, only a few patients with DEB specifically combined with ACC have been described and genotyped and almost all cases represent dominant forms of the condition. The aim of this study was to describe new mutations of COL7A1 in patients with DEB and ACC and investigate possible genotype/phenotype correlations. Twenty two patients with DEB and ACC were included among the 123 DEB patients whose COL7A1 mutations have been identified in the Reference Centre in Nice. Seven patients presented a severe generalized RDEB phenotype (RDEB-sev gen), while the other 15 suffered from milder phenotypes. We identified 28 mutations in COL7A1, of which nine are novel. Patients with severe phenotypes have almost mutations leading to premature termination codon (PTC) and/or splice site mutation or missense mutations. Patients with the milder phenotypes have mostly glycine or arginine substitutions associated or not with other types of mutations. All amino acid substitutions fell within the carboxyl portion of the triple helical domain (THD) of collagen VII, close to the THD interruptions. Our findings suggest that ACC is a frequent manifestation of DEB patients irrespective of the severity of the disease, and is due to leg rubbing in utero. In children with a moderate form of DEB with no or moderate skin fragility, a glycine substitution near the THD interruption domain of the collagen VII leading to thermolabile protein could explain this phenomenon. This article is protected by copyright. All rights reserved.
    British Journal of Dermatology 11/2013; · 3.76 Impact Factor
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    ABSTRACT: Terra firma-forme (i.e. resembling dry earth) is a condition chiefly affecting children wrongly considered as dermatosis arising out of negligence and inadequate corporal hygiene. It is in fact an acquired and asymptomatic grey-brown hyperpigmentation of the skin that persists despite normal washing with soap and water, but which subsides on rubbing with isopropyl alcohol 70%. Herein we report 10 new cases of this disorder. Ten patients aged between 7 months in 17 years were seen for acquired macular skin pigmentation, either brown or grey, fragmented and confluent. In six patients, this abnormality was the main reason for the consultation, generally on aesthetic grounds, and more rarely for diagnosis or suspicion of acanthosis nigricans. In all cases, questioning revealed normal hygiene measures. The condition comprised macular or acquired papular pigmentation, either brown or grey, of bilateral and symmetrical disposition and electively affecting the neck, trunk and retro-malleolar area of the ankles. Clinical examination together with a test involving rubbing with isopropyl alcohol 70° confirmed the diagnosis, revealing healthy underlying skin. Terra firma-forme dermatosis is frequently seen in clinical practice but is largely ignored in the French literature, possibly because of relevant indifference towards the condition. It affects both sexes equally, with no predilection for age or ethnicity, although it is classically seen to a greater extent during adolescence. Diagnosis of the condition, which is easily made thanks to the hyperpigmentation of dirty brown appearance on the neck and the ankles in particular, should not mislead the practitioner into blaming patients for supposedly deficient body hygiene. Knowledge of this form of dermatosis is useful because of its potentially harmful aesthetic and social effects, despite the ease of treatment by insistent rubbing of the affected areas with medical alcohol or ether. Early recognition also avoids pointless additional investigations associated with differential diagnosis in relation to various other acquired or hereditary dermatoses involving brown or grey pigmentation.
    Annales de Dermatologie et de Vénéréologie 11/2013; 140(11):693-8. · 0.60 Impact Factor
  • Journal of the American Academy of Dermatology 11/2013; 69(5):e268-e269. · 4.91 Impact Factor
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    ABSTRACT: Abstract is missing (short).
    Acta Dermato-Venereologica 10/2013;
  • C Girard, T Vincent, D Bessis
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    ABSTRACT: A new antibody known as anti-MDA-5 has recently been identified in association with a specific phenotype of dermatomyositis (DM), in which muscular involvement is minimal or absent, but where there is a particularly high risk of onset of aggressive interstitial lung disease. Below, we report a case associated with the presence of anti-MDA-5 antibodies complicated by aggressive interstitial lung disease ; the cutaneous phenotype of this patient is novel due to the constitution of diffuse ichthyosis and of profuse subcutaneous calcinosis. A 35-year-old man was hospitalised for a skin rash associated with arthralgia, dry cough, asthenia and weight-loss of 8kg in 5 months. The dermatological examination revealed purple erythema of the upper eyelids and cheeks, diffuse acquired ichthyosis of the trunk and limbs, and striated erythema on the proximal and distal metacarpal and interphalangeal joints of the fingers. A diagnosis of DM was suspected in patients treated with prednisolone, 1.5 mg/kg/d. Immunological tests identified the presence of anti-MDA-5 antibodies. One month later, the cutaneous signs continued to progress and were marked by the development of painful ulcers on the back of the fingers, as well as dyspnoea accompanying the slightest effort. Chest images showed constituted pulmonary fibrosis lesions. At the same time, within several months, diffuse, pseudo-tumoural calcifications developed in the subcutaneous tissue of the trunk and limbs. The patient was treated successively with intravenous gammaglobulins, cyclophosphamide, mycophenolate mofetil, azathioprine and rituximab in combination with oral corticosteroids, but with no improvement in respiratory function or in the skin lesions. Because of the decline in the patient's respiratory status, a lung graft was envisaged but subsequently abandoned because of the patient's excessively precarious state of health. Anti-MDA-5 antibody appears to constitute a specific immunological marker for a special dermatological phenotype of adult DM, significantly associated with the presence of: 1) keratotic papules in the skin folds of the palms and fingers ; 2) cutaneous ulcers along the periungueal edges, Gottron's papules on the back of the hands and on the extensor surface of the elbows and knees ; 3) pain and ulceration in the oral mucosa, diffuse alopecia, and appearance of "mechanic's hands" and Gottron's sign on the elbows and knees. To our knowledge, the presence of extensive ichthyosis and profuse subcutaneous calcinosis has not been previously reported in this particular form of DM. The risk of aggressive interstitial lung disease is particularly high and worsens the prognosis, since 40% of patients with anti-MDA-5 die, usually within the first year. Herein, we describe a patient presenting amyopathic DM complicated by aggressive interstitial lung disease associated with the presence of anti-MDA-5 antibodies, but with a hitherto unreported cutaneous phenotype combining diffuse ichthyosis and profuse subcutaneous calcinosis.
    Annales de Dermatologie et de Vénéréologie 10/2013; 140(10):628-634. · 0.60 Impact Factor
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    ABSTRACT: Capillary malformation-arteriovenous malformation (CM-AVM) is an autosomal dominant disorder, caused by heterozygous RASA1 mutations, and manifesting multifocal capillary malformations and high risk for fast-flow lesions. A limited number of patients has been reported, raising the question of the phenotypic borders. We identified new patients with a clinical diagnosis of CM-AVM, and patients with overlapping phenotypes. RASA1 was screened in 261 index patients with: CM-AVM (n = 100), common capillary malformation(s) (port-wine stain; n = 100), Sturge-Weber syndrome (n = 37), or isolated arteriovenous malformation(s) (n = 24). Fifty-eight distinct RASA1 mutations (43 novel) were identified in 68 index patients with CM-AVM and none in patients with other phenotypes. A novel clinical feature was identified: cutaneous zones of numerous small white pale halos with a central red spot. An additional question addressed in this study was the "second-hit" hypothesis as a pathophysiological mechanism for CM-AVM. One tissue from a patient with a germline RASA1 mutation was available. The analysis of the tissue showed loss of the wild-type RASA1 allele. In conclusion, mutations in RASA1 underscore the specific CM-AVM phenotype and the clinical diagnosis is based on identifying the characteristic capillary malformations. The high incidence of fast-flow lesions warrants careful clinical and radiologic examination, and regular follow-up. This article is protected by copyright. All rights reserved.
    Human Mutation 08/2013; · 5.21 Impact Factor
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    ABSTRACT: Children with multiple café-au-lait macules (CALMs) may be followed for years before a second National Institutes of Health clinical criterion of neurofibromatosis type 1 (NF1) develops to confirm the diagnosis. We sought to assess the prevalence of nevus anemicus (NA) in NF1 and its association with neuro-ophthalmologic complications. This was a prospective multicenter case-control study of 210 consecutive patients with multiple CALMs. Patients with NF1 were matched for age, sex, and center with control subjects. We documented the number, location, and morphologic appearance of NA; dermatologic features of NF1; magnetic resonance imaging results; and family history. In all, 77 (51%) patients with NF1 had NA compared with 6 (2%) control subjects. NA was not detected in 26 patients with other genodermatoses associated with CALMs. Patients with NF1 and NA were younger than those without NA (median age: 17 years) (P = .002). NA was mostly localized to the upper anterior aspect of the chest. NA was not significantly linked with other clinical manifestations of NF1, including optic glioma and unidentified bright objects. A potential referral bias associated with tertiary care centers is a limitation. NA appears to have a high prevalence and specificity in NF1 and might serve as a marker for NF1 in children with multiple CALMs.
    Journal of the American Academy of Dermatology 08/2013; · 4.91 Impact Factor
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    ABSTRACT: The use of reusable diapers, often made of cotton and bamboo material, is becoming more widespread in France - with the "eco" fashion, as they are considered more natural, ecologic and economic. We report 5 cases of papulonodular lesions in convex skin areas associated with the use of these diapers in infants. One case was typical Sevestre and Jacquet erosive dermatitis. The 4 others presented skin-coloured umbilicated papules or nodules with slight or no erythema and could be considered early-stage Sevestre and Jacquet erosive dermatitis, granuloma gluteale (1 case) or pseudo-verrucous papules. These 3 diagnoses probably belong to the same disease spectrum, proposed as "irritant napkin papulonodules". Napkin lesions occurred subsequent to 1) in all cases, use of reusable diapers, which are probably less absorbent than disposable diapers; 2) in 2 cases, insufficient food, which was responsible for lack of weight gain and delayed healing; and 3) in 1 case, diarrhea, which worsened the moisture. We alert physicians to possible papulonodular napkin dermatitis in infants wearing reusable diapers.
    European journal of dermatology : EJD. 04/2013;
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    ABSTRACT: Plexiform neurofibroma is a rare and benign tumor often associated with type 1 neurofibromatosis (NF1) or Von Recklinghausen's disease. Present in one third of cases of NF1, there are isolated forms where the diagnosis should remain a diagnosis of exclusion. We report four cases of isolated plexiform neurofibromas found in children from topographies and we discuss the pretherapeutic assessment, the surgical management and the long-term follow-up.
    Annales de Chirurgie Plastique Esthétique. 01/2013; 58(6):694–699.

Publication Stats

811 Citations
454.65 Total Impact Points


  • 2014
    • French Institute of Health and Medical Research
      Lutetia Parisorum, Île-de-France, France
  • 2007–2014
    • Université de Montpellier 1
      Montpelhièr, Languedoc-Roussillon, France
  • 2001–2013
    • Centre Hospitalier Universitaire de Montpellier
      • Département de dermatologie
      Montpelhièr, Languedoc-Roussillon, France
  • 2006
    • Centre Hospitalier Régional Universitaire de Nîmes
      Nismes, Languedoc-Roussillon, France
  • 1999
    • St. Charles Hospital
      Port Jefferson, New York, United States