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BACKGROUND: A new autoinflammatory syndrome related to somatic mutations of UBA1 was recently described and called VEXAS syndrome ('Vacuoles, E1 Enzyme, X-linked, Autoinflammatory, Somatic syndrome'). OBJECTIVES: To describe clinical characteristics, laboratory findings and outcomes of VEXAS syndrome. METHODS: One hundred and sixteen patients with VEXAS syndrome were referred to a French multicentre registry between November 2020 and May 2021. The frequency and median of parameters and vital status, from diagnosis to the end of the follow-up, were recorded. RESULTS: The main clinical features of VEXAS syndrome were found to be skin lesions (83%), noninfectious fever (64%), weight loss (62%), lung involvement (50%), ocular symptoms (39%), relapsing chondritis (36%), venous thrombosis (35%), lymph nodes (34%) and arthralgia (27%). Haematological disease was present in 58 cases (50%): myelodysplastic syndrome (MDS; n = 58) and monoclonal gammopathy of unknown significance (n = 12; all patients with MGUS also have a MDS). UBA1 mutations included p.M41T (45%), p.M41V (30%), p.M41L (18%) and splice mutations (7%). After a median follow-up of 3 years, 18 patients died (15·5%; nine of infection and three due to MDS progression). Unsupervised analysis identified three clusters: cluster 1 (47%; mild-to-moderate disease); cluster 2 (16%; underlying MDS and higher mortality rates); and cluster 3 (37%; constitutional manifestations, higher C-reactive protein levels and less frequent chondritis). The 5-year probability of survival was 84·2% in cluster 1, 50·5% in cluster 2 and 89·6% in cluster 3. The UBA1 p.Met41Leu mutation was associated with a better prognosis. CONCLUSIONS: VEXAS syndrome has a large spectrum of organ manifestations and shows different clinical and prognostic profiles. It also raises a potential impact of the identified UBA1 mutation.
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Gamopatia Monoclonal de Significância Indeterminada , Síndromes Mielodisplásicas , Humanos , Inflamação/genética , Mutação/genética , Síndromes Mielodisplásicas/diagnóstico , Enzimas Ativadoras de UbiquitinaRESUMO
Objectives: Familial Mediterranean fever (FMF) is the most frequent monogenic autoinflammatory disease. It is associated with MEFV mutations. Its main features are recurrent episodes of fever and serositis. Patients can display dermatological manifestations such as erysipelas-like erythema, generally considered as a neutrophilic dermatosis (ND). It has been suggested that FMF can be associated with other types of ND. Our aim was to perform a systematic review of the literature to assess the link between ND and FMF.Method: A systematic review of the literature was performed using MEDLINE from 1946 to 2018. Three independent investigators identified reports of non-erysipelas-like erythema neutrophilic dermatosis (NEND) associated with FMF, selected the criteria to establish the diagnosis of FMF and ND, and evaluated the link between the two conditions. FMF-associated NEND was supported by confirmation of both diagnoses and exclusion of other causes of ND.Results: Eighteen articles were selected. Nine articles reported FMF patients with the following NEND: neutrophilic panniculitis (n = 4), Sweet syndrome (n = 6), and pyoderma gangrenosum (n = 1). None of these cases was supported by histological confirmation, fulfilled diagnostic criteria for definitive or probable FMF, or confirmed the exclusion of all the most frequent diseases associated with NEND. As a result, there is insufficient evidence to support a potential relationship between NEND and FMF.Conclusions: The association between FMF and NEND remains unclear. In FMF patients with NEND, every differential diagnosis and alternative cause of NEND should be excluded before drawing any conclusions about a potential causal relationship.
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Febre Familiar do Mediterrâneo , Dermatopatias , Diagnóstico Diferencial , Febre Familiar do Mediterrâneo/complicações , Febre Familiar do Mediterrâneo/diagnóstico , Febre Familiar do Mediterrâneo/genética , Humanos , Mutação , Pirina/genética , Dermatopatias/complicações , Dermatopatias/diagnósticoRESUMO
BACKGROUND: Erythema multiforme (EM) is a muco-cutaneous inflammatory disease mainly triggered by herpes simplex virus (HSV) recurrences. Association of EM and circulating auto-antibodies against plakins (anti-PLK-Abs [EM-PLK+]) has been reported. However, little is known about this subset of EM. OBJECTIVES: We aimed to describe the clinical and immunological features and response to treatment of EM-PLK+. METHODS: We conducted a retrospective multicentric study of EM-PLK+ selected from the database of the immunological laboratory of Bichat hospital, Paris, France, from January 2009 to December 2020. Anti-PLK-Abs were detected in ≥1 immunological tests: immunofluorescence assay, immunoblotting and/or ELISA. Patients with alternative diagnoses were excluded. RESULTS: We included 29 patients (16 women, median age 25 [range 2-58] years). EM-PLK+ were mostly major (EM with ≥2 mucosal involvements; n = 24, 83%) and relapsing (≥2 flares; n = 23, 79%). Cutaneous lesions were target (n = 13, 54%) and target-like lesions (n = 9, 38%) with usual topography (acral, n = 19, 79%; limbs, n = 21, 88%). Mucosal lesions affected the mouth (n = 27, 96%) and genitalia (n = 19, 68%), with a median of 2 [range 0-5] mucous membranes. EM-PLK+ were suspected as certain or possible postherpetic (EM-HSV) in 19 cases (65.5%); no triggering factors were detected in 9 (31%) patients. Desmoplakin-I/II Abs were the most frequent anti-PLK-Abs (n = 20, 69%); envoplakin and periplakin Abs were detected in 11 and 9 cases. Relapsing EM-PLK+ (n = 23) were still active (≥1 flare within 6 months) in 13 (57%) patients despite immunosuppressive therapy (n = 8, 62%). Antiviral drugs were ineffective in preventing relapse in 15/16 (94%) EM-HSV. CONCLUSION: The rationale for anti-PLK-Ab detection in EM is not elucidated. More systematic research of anti-PLK-Abs is warranted to better understand whether this association reflects humoral immune activity in a subset of EM or is fortuitous, related to an epitope spreading process. However, EM-PLK+ seems to be associated with major and relapsing subtypes, and difficult-to-treat cases.
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Eritema Multiforme , Herpes Simples , Humanos , Feminino , Pré-Escolar , Criança , Adolescente , Adulto Jovem , Adulto , Pessoa de Meia-Idade , Estudos Retrospectivos , Eritema Multiforme/tratamento farmacológico , Simplexvirus , Herpes Simples/tratamento farmacológico , Antivirais/uso terapêutico , RecidivaRESUMO
The term Sneddon's syndrome (SS) has been used since 1965 to describe a vasculopathy characterized by a combination of cerebrovascular disease with livedo racemosa. SS may be classified as antiphospholipid+ (aPL+) or antiphospholipid- (aPL-). Little is known about aPL- SS; in this review we describe the epidemiology and pathogenesis of aPL- SS, as well as the clinical and histologic features. We discuss recent findings in terms of neurologic and cardiac involvement. Moreover, differential diagnoses of conditions that may present with both livedo racemosa and stroke are discussed. Finally, we discuss real-life practical issues such as the initial investigations to be performed, long-term follow-up, and therapeutic management of aPL- SS patients.
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Síndrome Antifosfolipídica , Livedo Reticular , Síndrome de Sneddon , Síndrome Antifosfolipídica/complicações , Síndrome Antifosfolipídica/diagnóstico , Síndrome Antifosfolipídica/epidemiologia , Diagnóstico Diferencial , Humanos , Livedo Reticular/diagnóstico , Livedo Reticular/epidemiologia , Livedo Reticular/etiologia , Síndrome de Sneddon/complicações , Síndrome de Sneddon/diagnóstico , Síndrome de Sneddon/epidemiologiaRESUMO
BACKGROUND: Store-and-forward (SAF) tele-dermatology (TD) platforms could help promote coordination between hospital and general practitioners (GPs). However, very little data exists on the performance accuracy and opinions of GPs participating in this type of project in France. METHODS: We report on the diagnostic and management plan accuracy of an SAF-TD platform developed for neighbouring GPs around our hospital compared with routine face-to-face (FTF) dermatological consultation in our department. We also compared the accuracy of SAF-TD with that of the participating GPs. Lastly, we collected feedback from GPs after their participation in this project. RESULTS: Overall, 298 patients were included by 58 GPs between November 2016 and January 2020, of whom 169 (57%) were female, and with a median age of 44.5 years (range 0-96). The diagnostic accuracy of TD was 62% (n=184/298) for the initial hypothesis and 80% (n=239/298) for aggregated diagnostic accuracy. Management plan accuracy for TD was 81% (n=225/277). At least 43% of consultations (n=127/298) met the criteria for preventable consultation. Diagnostic accuracy for the initial hypothesis was significantly lower for GPs than for TD (Odd Ratio [OR]=0.34; 95% Confidence Interval [95% CI]: 0.20-0.56; p<0.0001), as was management plan accuracy (OR=0.23; 95% CI: 0.10-0.46; p<0.0001). Among the responding GPs, 78% (n=29) reported very high satisfaction and 97% would consider integrating this type of programme in their long-term practice, but they highlighted the time-consuming nature of the platform (46%) and the lack of financial compensation (44%). CONCLUSION: SAF-TD in coordination with GPs seems safe and efficient in the management of outpatients, and enjoys a high satisfaction rate among GPs, despite its time-consuming nature and the lack of financial compensation. Healthcare policy should promote financial participation to help the expansion of TD.
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Dermatologia , Clínicos Gerais , Dermatopatias , Telemedicina , Humanos , Feminino , Recém-Nascido , Lactente , Pré-Escolar , Criança , Adolescente , Adulto Jovem , Adulto , Pessoa de Meia-Idade , Idoso , Idoso de 80 Anos ou mais , Masculino , Estudos Retrospectivos , Encaminhamento e Consulta , Dermatopatias/diagnóstico , Dermatopatias/terapiaRESUMO
BACKGROUND: Eosinophilic annular erythema (EAE) is a rare eosinophil-related skin disease which typically manifests with annular erythematous plaques and severe pruritus. Besides the diagnosis, the treatment of EAE is challenging since relevant published data are sparse. METHODS: The aim of this study was to assess the underlying diseases, treatments and outcomes of patients with EAE. To this end, we conducted a retrospective multicenter study and a systematic review of the MEDLINE database. RESULTS: We included 18 patients with EAE followed in 8 centers. The MEDLINE database search yielded 37 relevant publications reporting 55 cases of EAE with 106 treatment sequences. The most common and efficient treatments included topical or systemic corticosteroids, hydroxychloroquine and dapsone. In refractory patients, a combination of systemic corticosteroids with hydroxychloroquine was associated with 88% of complete clinical response. DISCUSSION: To improve the management of EAE patients, we discuss the following treatment strategy: in topical steroid-resistant patients, hydroxychloroquine can be given as first-line systemic treatment. Dapsone, hydroxychloroquine or systemic corticosteroids are second-line options to consider. Last, monoclonal antibodies or JAK inhibitors targeting type 2 inflammation could represent promising last-resort options in refractory patients.
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Eosinofilia , Hidroxicloroquina , Corticosteroides/uso terapêutico , Dapsona/uso terapêutico , Eosinofilia/complicações , Eosinofilia/tratamento farmacológico , Eritema/diagnóstico , Eritema/tratamento farmacológico , Humanos , Hidroxicloroquina/uso terapêutico , Estudos Multicêntricos como Assunto , Doenças Raras/tratamento farmacológico , Dermatopatias GenéticasRESUMO
Cutaneous drug-induced lupus erythematosus (CDILE) is a lupus-like syndrome related to drug exposure which typically resolves after drug discontinuation. It can present as a systemic or a sole cutaneous form and different drugs may be associated with each form. CDILE pharmacoepidemiology is constantly changing. Indeed, older drugs primarily associated with systemic CDILE are no longer prescribed and new drugs associated with either cutaneous or systemic CDILE have emerged. The present study discusses the clinical and laboratory aspects of CDILE and the postulated pathogenesis, and it provides an update on implicated drugs. We performed a literature review to single out the new drugs associated with CDILE in the past decade (January 2010-June 2020). Among 109 drugs reported to induce CDILE in 472 patients, we identified anti-TNFα, proton-pump inhibitors, antineoplastic drugs, and, in particular, checkpoint inhibitors, as emerging drugs in CDILE. Most of the published studies are cases reports or small case series, and further larger studies as well as the development of validated classification criteria are needed to better understand and characterize their implication in CDILE.
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Antineoplásicos , Lúpus Eritematoso Cutâneo , Lúpus Eritematoso Sistêmico , Preparações Farmacêuticas , Antineoplásicos/uso terapêutico , Humanos , Lúpus Eritematoso Cutâneo/induzido quimicamente , Lúpus Eritematoso Cutâneo/tratamento farmacológico , Lúpus Eritematoso Sistêmico/induzido quimicamente , Lúpus Eritematoso Sistêmico/tratamento farmacológico , Inibidores da Bomba de Prótons/uso terapêuticoRESUMO
INTRODUCTION: There are few studies focusing on ungual lesions in patients with lupus erythematosus (LE). The aim of this study is to describe the type and the prevalence of ungual lesions among LE patients. PATIENTS AND METHODS: A systematic literature review with analysis of individual data was performed by searching the MEDLINE database for scientific articles using the keywords "lupus erythematosus" and "nail". RESULTS: Two-hundred and eighty-seven cases were collated including 55.1% women, with an average age of 32.2±11 years. The most common ungual or peri-ungual lesions were longitudinal ridging (83 patients, 28.9%), peri-ungual erythema (62 patients, 21.6%), onycholysis (60 patients, 20.9%), melanonychia (34 patients, 11.8%) and dyschromia (33 patients, 11.5%). An association between the presence of onycholysis and peri-ungual erythema and disease activity was noted [respectively 33 (38.8%) and 26 (30.6%) patients out of 85 with active disease versus 3 (5.8%) and 4 (7.7%) patients out of 52 with non-active disease, P<0.001 and P=0.018]. Screening for fungal infection was performed in one third of the cases, with proven onychomycosis in 34.7% of cases. DISCUSSION: Ungual lesions are not specific and do not permit diagnosis of LE. They can in fact occur in other diseases such as connective tissue disorders. However, their diagnosis is important because they may be the presenting sign in LE, and certain of them may be associated with more active disease. Onychomycosis is frequently a confounding factor in such immunocompromised patients.
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Lúpus Eritematoso Sistêmico/complicações , Doenças da Unha/etiologia , Adolescente , Adulto , Idoso , Criança , Feminino , Humanos , Lúpus Eritematoso Sistêmico/epidemiologia , Lúpus Eritematoso Sistêmico/patologia , Masculino , Pessoa de Meia-Idade , Doenças da Unha/epidemiologia , Doenças da Unha/patologia , Onicomicose/epidemiologia , Onicomicose/etiologia , Onicomicose/patologia , Adulto JovemRESUMO
INTRODUCTION: There are few studies focusing on ungual lesions in patients with lupus erythematosus (LE). The aim of this study is to describe our experience with ungual lesions in LE patients. MATERIALS AND METHODS: A multicentric retrospective descriptive study was performed at the dermatology departments of the university hospitals in Strasbourg and at the Tenon hospital in Paris and involved reviewing the medical records and photographs of patients with ungual lesions. RESULTS: Fourteen patients were included: 12 (86 %) were women with a median age of 38 years (28-78 years). All patients had cutaneous LE presenting as follows: 3 isolated forms (21 %), and associated with systemic LE (LES) for remaining 11 patients (79 %). The most frequent ungual or peri-ungual lesions were longitudinal ridging (12 patients, 86 %), onycholysis and cuticular alterations (8 patients each, 57 %), pterygium (7 patients, 50 %), melanonychia, onychoschizia and subungual hyperkeratosis (5 patients with each, 36 %). Among patients with pterygium and onychoschizia, respectively 6 (86 %) and 5 (100 %) presented the discoid LE subtype, while respectively 6 (86 %) and 4 (80 %) had multisystemic involvement. DISCUSSION: Ungual lesions do not appear specific and do not in themselves allow diagnosis of LE. They can in fact occur in other diseases such as connective tissue disorders. However, their diagnosis is important because certain of them, such as pterygium, can lead to severe ungual dystrophia, with functional consequences. In our study, pterygium and onychoschizia appeared to be associated with cutaneous discoid lupus erythematosus and multisystemic involvement. The coexistence of peri-ungual lesions related to cutaneous lupus erythematosus and/or multisystemic involvement does not out differentiation of lupus ungual lesions and post-inflammatory lesions.
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Lúpus Eritematoso Cutâneo , Lúpus Eritematoso Discoide , Lúpus Eritematoso Sistêmico , Feminino , Humanos , Recém-Nascido , Lúpus Eritematoso Cutâneo/diagnóstico , Lúpus Eritematoso Sistêmico/complicações , Lúpus Eritematoso Sistêmico/epidemiologia , Estudos Retrospectivos , PeleRESUMO
BACKGROUND: Chilblains are inflammatory dermal lesions associated with hypersensitivity to cold, and they occur on the extremities bilaterally and symmetrically. Their onset during the course of pro-thermogenic and autoimmune diseases has been widely reported, but the association with predisposing locoregional causes is not well known. PATIENTS AND METHODS: Case 1: a 57-year-old man, who smoked 80 packets per year, presenting a deficit of the levator muscles in his right foot following lumbar sciatica with paralysis of L5, consulted for unilateral necrotic lesions of the toes recurring each winter in the paralysed limb only. Case 2: a 60-year-old man had a previous history of liposarcoma of the right side treated with radiotherapy and surgery, resulting in sequelae of monoparesis and radiation-induced arteritis. Each winter, he presented recurring unilateral purpuric macules of the toes on his right foot, with no necrotic progression. In both cases, clinical examination, disease progression over time, histology and laboratory tests confirmed the diagnosis of idiopathic chilblains. CONCLUSION: The physiopathological hypotheses posited to account for the unilateral appearance of chilblains in the event of paralysis include decreased blood flow to the paralysed limb, imbalance in neuromodulators, dysfunction of the autonomous nervous system, cutaneous atrophy with hypertrophy of underlying soft tissues, and finally, hypoesthesia aggravating the trophic disorders.
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Pérnio/etiologia , Paresia/complicações , Pérnio/patologia , Humanos , Masculino , Pessoa de Meia-IdadeRESUMO
BACKGROUND: Intravascular large B-cell lymphoma (ivLBCL) is a rare blood dyscrasia that is difficult to diagnose. Healthy skin biopsies may prove useful in diagnosis of the condition. Herein we report a case of ivLBCL diagnosed using this type of examination, and we provide a literature review to determine the sensitivity of such testing. PATIENTS AND METHODS: A 67-year-old woman was hospitalised for unexplained prolonged fever (UPF) and impaired general well-being. Laboratory tests revealed inflammatory syndrome, elevated LDH>2000IU/L, hepatic cytolysis and decreased prothrombin time at 47 %. Analysis for infection and medical imaging ruled out both an infectious or inflammatory origin and solid tumour. A healthy skin biopsy enabled confirmation of the diagnosis of ivLBCL. DISCUSSION: This clinical case illustrates the value of healthy skin biopsy in establishing a diagnosis of ivLBCL in patients hospitalised for UPF. Following a systematic literature review in PubMed/Medline, we included eight studies involving at least three patients designed to assess the value of healthy skin biopsy in the diagnosis of ivLBCL. The diagnostic sensitivity of this approach ranged from 67% to 100%, with a sensitivity of 100% being seen in four of the eight studies. Details of the biopsy sites were available in three studies and diagnostic sensitivity was similar overall between samples taken from the thigh, abdomen and arms. CONCLUSION: Healthy skin biopsy sampling from at least two sites constitutes a sensitive and relatively non-invasive procedure for early diagnosis of ivLBCL.
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Linfoma Difuso de Grandes Células B/parasitologia , Pele/patologia , Neoplasias Vasculares/patologia , Idoso , Biópsia , Diagnóstico Diferencial , Feminino , HumanosRESUMO
BACKGROUND: Identification of myositis-specific autoantibodies (MSAs) for dermatomyositis (DM) could allow the characterization of an antibody-associated clinical phenotype. OBJECTIVE: We sought to define the clinical phenotype of DM and the risk of cancer, interstitial lung disease (ILD) and calcinosis based on MSA. METHODS: A 3.5-year multicentre prospective study of adult DM patients was conducted to determine the clinical phenotype associated with MSAs and the presence of cancer, ILD and calcinosis. RESULTS: MSAs were detected in 47.1% of 117 included patients. Patients with antimelanoma differentiation-associated protein-5 antibodies (13.7%) had significantly more palmar violaceous macules/papules [odds ratio (OR) 9.9], mechanic's hands (OR 8), cutaneous necrosis (OR 3.2), articular involvement (OR 15.2) and a higher risk of ILD (OR 25.3). Patients with antitranscriptional intermediary factor-1 antibodies (11.1%), antinuclear matrix protein-2 antibodies (6.8%) and antiaminoacyl-transfer RNA synthetase (5.1%) had, respectively, significantly more poikiloderma (OR 5.9), calcinosis (OR 9.8) and articular involvement (OR 15.2). Cutaneous necrosis was the only clinical manifestation significantly associated with cancer (OR 3.1). CONCLUSION: Recognition of the adult DM phenotype associated with MSAs would allow more accurate appraisal of the risk of cancer, ILD and calcinosis.
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Anticorpos/sangue , Dermatomiosite/sangue , Dermatomiosite/complicações , Helicase IFIH1 Induzida por Interferon/imunologia , Neoplasias/complicações , Pele/patologia , Adenosina Trifosfatases/imunologia , Adulto , Idoso , Idoso de 80 Anos ou mais , Aminoacil-tRNA Sintetases/imunologia , Calcinose/sangue , Calcinose/complicações , Proteínas de Ligação a DNA/imunologia , Feminino , Dermatoses da Mão/sangue , Dermatoses da Mão/complicações , Humanos , Artropatias/sangue , Artropatias/complicações , Doenças Pulmonares Intersticiais/sangue , Doenças Pulmonares Intersticiais/complicações , Masculino , Pessoa de Meia-Idade , Necrose , Fenótipo , Estudos Prospectivos , Fatores de Transcrição/imunologia , Adulto JovemRESUMO
AIM: Our aim was to assess the prevalence of adverse effects (AEs) pertaining to the use and withdrawal of hydroxychloroquine (HCQ) in dermatological outpatients. PATIENTS AND METHODS: We conducted a retrospective study between January 2013 and June 2014 that included consecutive patients currently or previously receiving HCQ seen in our department. AEs were collated using a standardized questionnaire and validated by clinical and laboratory examination. Drug causality was evaluated using the updated French drug reaction causality assessment method. The main evaluation criterion was the prevalence of AEs in which HCQ had an intrinsic imputability score of I>2. RESULTS: We included 102 patients (93 of whom were women, with a median age of 44.5; range: 22-90years). HCQ was given for cutaneous lupus in most cases (n=70). At least one AE was reported for 55 patients. Among the 91 reported AEs, 59 (65%) had an HCQ intrinsic imputability score I>2. AEs were responsible for permanent HCQ discontinuation in 19 cases. Of these, 8 were unrelated to HCQ based on imputability score. The most common AEs associated with HCQ were gastrointestinal and cutaneous signs. Of the 8 patients diagnosed with retinopathy, only 3 were confirmed after reevaluation. CONCLUSION: AEs associated with HCQ were reported for over 50% of patients and were responsible for permanent HCQ discontinuation in one-third of cases. A more in-depth evaluation of imputability seems necessary, particularly regarding ophthalmological symptoms, since in two thirds of cases the reasons for discontinuation were not related to HCQ.
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Hidroxicloroquina/efeitos adversos , Dermatopatias/tratamento farmacológico , Adulto , Idoso , Idoso de 80 Anos ou mais , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Adulto JovemRESUMO
BACKGROUND: Fingolimod is an oral immunomodulator approved for relapsing-remitting multiple sclerosis. We report a case of a primary cutaneous CD30+ T-cell lymphoproliferation occurring 6 months after initiation of fingolimod. Based on a systematic literature review, the characteristics of these fingolimod-induced lymphoproliferative disorders are described. PATIENTS AND METHODS: A 56-year-old woman developed cutaneous indurated and ulcerated nodular lesions 6 months after starting fingolimod for active relapsing-remitting multiple sclerosis. Histological examination of a punch biopsy sample demonstrated a polymorphous dermal infiltrate containing large atypical CD30+ cells, leading to diagnosis of primary cutaneous CD30+ anaplastic large-cell lymphoma. Chest-abdomen-pelvis CT scans were performed to rule out secondary cutaneous anaplastic large-cell lymphoma. Spontaneous clinical regression was observed and after assessing the benefit/risk ratio, it was decided to continue fingolimod under strict surveillance, with no relapse occurring by month 18. DISCUSSION: A systematic review of PUBMED/Medline and Embase identified seven other cases of lymphoproliferative disorders occurring during fingolimod treatment, including two other cases of primitive cutaneous CD30+ lymphoproliferative disorders. CONCLUSION: Even if cutaneous CD30+ lymphoproliferative disorders occur only rarely during fingolimod treatment, dermatologists should nevertheless be aware of this association for which strict dermatological surveillance is required. We would also stress that these CD30+ lymphoproliferative disorders can disappear spontaneously, as in our case, even if treatment by fingolimod is continued.
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Cloridrato de Fingolimode/efeitos adversos , Antígeno Ki-1 , Transtornos Linfoproliferativos/induzido quimicamente , Transtornos Linfoproliferativos/imunologia , Linfócitos T/imunologia , Feminino , Humanos , Pessoa de Meia-IdadeRESUMO
BACKGROUND: The antimalarials (AMs) hydroxychloroquine (HCQ) and chloroquine (CQ) have demonstrated variable cutaneous response rates in cutaneous lupus erythematosus (CLE). OBJECTIVES: We sought to assess the global cutaneous response rates to HCQ and CQ, with respect to CLE subtypes, based on previously published studies. METHODS: We performed a systematic review and meta-analysis of studies published in MEDLINE, Embase and the Cochrane Library between 1965 and December 2015. The proportions of responders to AMs according to CLE subtypes were extracted from individual studies and pooled using random-effects or fixed models. The odds ratio (OR) was used as the measure of association to compare the response rates between CLE subtypes and AMs. RESULTS: Among 1990 courses of treatment with AMs from 31 included studies, the overall response rate to AMs was 63% [95% confidence interval (CI) 55-70], with important statistical heterogeneity across the included studies. HCQ had a higher overall efficacy than CQ, but this was not significant (OR 1·48, 95% CI 0·98-2·23). The response rate to AMs was different between CLE subtypes, ranging from 31% (95% CI 20-44) for chilblain lupus to 91% (95% CI 87-93) for acute CLE. The response was significantly higher for acute CLE than for subacute CLE and intermittent CLE. In case of failure of monotherapy with AM, the combination of quinacrine with HCQ or CQ seemed effective, whereas too little data were available to assess the efficacy of the switch to another AM agent. CONCLUSIONS: Wide discrepancies in cutaneous response to AMs are observed between CLE subtypes. A specific therapeutic approach considering CLE subtypes may improve CLE management.
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Antimaláricos/uso terapêutico , Cloroquina/uso terapêutico , Hidroxicloroquina/uso terapêutico , Lúpus Eritematoso Cutâneo/tratamento farmacológico , Adulto , Idoso , Idoso de 80 Anos ou mais , Fármacos Dermatológicos/uso terapêutico , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Observacionais como Assunto , Uso Off-Label , Ensaios Clínicos Controlados Aleatórios como Assunto , Resultado do TratamentoRESUMO
BACKGROUND: Cutaneous eruption around a peritoneal dialysis (PD) catheter exit site is a rare complication. Herein we report a case of bullous eruption; we discuss the diagnostic approach and the related therapeutic implications. PATIENTS AND METHODS: A 63-year-old man presented a bullous periumbilical eruption two months after initiation of PD. Cultures of laboratory samples ruled out an infectious origin and systemic corticosteroids initiated at 0.5mg/kg for suspected eosinophilic peritonitis produced significant improvement of the cutaneous eruption. Recurrence of the bullous eruption was observed upon dose-reduction of the corticosteroid. Skin histology showed a clinical picture of eczema and direct immunofluorescence was negative. Patch testing was carried out using the European Standard Battery comprising antiseptic, cosmetic and plastic series; a semi-open test was performed with the dressing used for PD, and ROAT was carried out on the povidone iodine (Betadine™) 10% used for topical care during PD. The patch testing and ROAT were positive (++), confirming contact dermatitis due to Betadine™. The eruption totally disappeared on substitution of Betadine™ by chlorhexidine for topical antisepsis of the PD catheter, thus enabling PD to be continued rather than instituting hemodialysis. DISCUSSION: Allergic contact dermatitis around a PD catheter is a rare and little-known complication. In the present case, ROAT testing showed sensitization to Betadine™ and enabled an alternative antisepsis solution to be found, allowing PD to be continued.