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3.
Medicine (Baltimore) ; 98(46): e17895, 2019 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-31725635

RESUMO

RATIONALE: Erythema multiforme (EM) is an immune-mediated disease with mucocutaneous localization and plurietiologic determinism. The term "multiforme" refers to the variety of aspects that the lesions can take from patient to patient and during evolution in a single patient. PATIENT CONCERNS: We have selected 2 cases of small children diagnosed with different etiology of EM to illustrate the importance of a correct and fast diagnosis. Case 1 involves a 2-year-old girl from a rural area who presented with fever and pruritic erythematous papular eruption. The onset of the symptoms was 3 days before presentation with fever and ulcerative lesions on the oral and labial mucosa, followed by the appearance of erythematous macular lesions, with progressive confluence to intense pruritic patches. The 2nd involves a 2-year-old boy with fever, loss of appetite, productive cough, and petechiae. He had corticosensible immune thrombocytopenia from the age of 6 months, with many recurrences. The patient received treatment with ampicillin/sulbactam and symptomatics for an erythemato-pultaceous angina. During the 2nd day of treatment the patient developed an erythematous macular eruption on the face, scalp, trunk, and limbs, with bullae formation. DIAGNOSES: The 1st patient was diagnosed based on biologic findings: positive inflammatory syndrome, elevated level of anti-Mycoplasma pneumoniae immunoglobulin M antibodies and immunoglobulin E. Histopathologic examination described papillary dermal edema, inflammatory infiltrate, and lymphocyte exocytosis. In the 2nd case, the hemoleucogram identified 12,000/mm platelets and the medulogram aspect was normal. Serology for Epstein-Barr virus was negative. The diagnosis was EM secondary to M pneumoniae infection in case 1 and secondary to administration of ampicillin/sulbactam in case 2. INTERVENTIONS: In both cases, etiopathogenic treatment consisting of steroidal antiinflammatory drugs, antihistamines was administered. Because of specific etiology, the 1st case received antibiotics. OUTCOMES: The evolution was favorable in 10 to 14 days; the patients were discharged after etiopathogenic treatment consisting of steroidal antiinflammatory drugs, antihistamines, and/or antibiotics. LESSONS: Performing a detailed clinical examination, medical history of drug use, infection or general diseases can establish a good diagnosis of EM. Histopathologic examination can help. The treatment is etiologic, pathogenic, and symptomatic. EM usually has a self-limited evolution.


Assuntos
Eritema Multiforme/diagnóstico , Eritema Multiforme/fisiopatologia , Antibacterianos/uso terapêutico , Anti-Inflamatórios/uso terapêutico , Pré-Escolar , Eritema Multiforme/tratamento farmacológico , Eritema Multiforme/microbiologia , Feminino , Antagonistas dos Receptores Histamínicos/uso terapêutico , Humanos , Masculino , Romênia
4.
Lupus ; 28(14): 1716-1721, 2019 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-31674268

RESUMO

Rowell syndrome (RS) is a rare disease characterized by the association of systemic lupus erythematosus (SLE) or cutaneous lupus with lesions similar to erythema multiforme and the presence of autoantibodies including ANA, SSA, SSB, or rheumatoid factor. Due to the low incidence of this disease, the epidemiology of RS is not clear. So far there are 95 cases reported in the literature; of these, only seven cases are pediatric patients. Macrophage activation syndrome (MAS) is an increasingly recognized complication of SLE, although its true prevalence in childhood is still unknown. We describe a unique pediatric patient with RS who developed MAS.


Assuntos
Eritema Multiforme/diagnóstico , Lúpus Eritematoso Sistêmico/diagnóstico , Síndrome de Ativação Macrofágica/etiologia , Pele/patologia , Criança , Diagnóstico Diferencial , Eritema Multiforme/patologia , Humanos , Lúpus Eritematoso Sistêmico/patologia , Masculino
6.
Acta Dermatovenerol Croat ; 27(3): 200-201, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31542069

RESUMO

Dear Editor, Systemic lupus erythematosus (SLE) is a heterogeneous autoimmune disease characterized by diverse patterns of auto-antibody production with multi-organ affectation. Cutaneous involvement, either alone or in association with other systemic illnesses, is one of its most common manifestations (1). Dermatologic disorders like malar and discoid rashes are quite suggestive of SLE. However, the occurrence of non-specific skin lesions like erythema multiforme (EM) in patients with SLE (Rowell syndrome) can rarely occur (1). In such patients, a diagnosis of SLE may be missed or delayed in the absence of other overt clinical features of lupus. Herein we report a case of recurring EM-like eruptions as the cardinal cutaneous manifestation of previously undiagnosed, active SLE in a young Nigerian woman. A 26-year-old Nigerian woman presented with a three-day history of non-pruritic, generalized, and target-like, erythematous annular patches and plaques which mostly affected the trunk. A few lesions had presented with crusting and erosions at the time of examination (Figure 1). Associated symptoms included oral painful ulcers, low grade fever, and malaise. The patient had no other systemic symptoms and her prior drug history was not remarkable. Her erythrocyte sedimentation rate (ESR) was 66 mm/hour using the Westergren method. Screening for HIV and hepatitis B and C was negative. Herpes simplex, cytomegalovirus, and Epstein Barr viruses could not be screened for. Other baseline investigations (complete blood count, electrolytes, urea and creatinine as well as urinalysis) were within normal limits. The patient was managed as a case of EM of an unidentified inciting agent and her symptoms resolved with supportive care and antibiotics. However, she developed a recurrence about 5 weeks later, with more extensive and coalescent skin lesions (Figure 2). Additionally, there was a new onset of alopecia and pain in the small joints of the hands as well as both knees and ankles. At this time, the patient's ESR had gone up to 112 mm/h and she had developed significant proteinuria, with a protein creatinine ratio of 1.3 g/g (reference <0.5 g/g). Her antinuclear antibody (ANA) titer was high (1:320) with a speckled pattern. Anti-Smith antibody was also positive. A renal biopsy was declined. A tentative diagnosis of Rowell syndrome was made. The patient was started on high-dose steroids and hydroxychloroquine 200 mg twice daily. Subsequent care included the use of mycophenolate mofetil 1 g twice daily for 6 months. This was then changed to azathioprine at 50 mg twice daily. Follow-up after 6 months showed sustained clearance of skin lesions, resolution of fever and joint pains, as well as improvement in the renal profile, with a urine protein-creatinine ratio of 0.77 g/g. The presence of systemic lupus erythematosus, EM-like lesions, and a speckled pattern of antinuclear antibody in our patient fulfils the revised diagnostic criteria for RS put forward by Zeitouni et al. at the turn of the twenty-first century (2). Considering the rarity of EM-like lesions in SLE and the possibility of constitutional symptoms in EM, a diagnosis of RS may be readily overlooked in patients like the one described, whose major cutaneous manifestation of severe active SLE was EM-like lesions. In contrast to classic EM, where skin lesions are concentrated in the extremities, a predominant truncal distribution of EM-like lesions as found in our patient may favor a clinical consideration of RS (3). However, some authors have challenged the existence of Rowell syndrome as a distinct clinical laboratory entity. Arguments put forward in this regard include the fact that none of the immunological markers that have been described in RS are specific to any disorder. Additionally, the annular polycyclic dermatosis seen in sub-acute cutaneous lupus erythematosus (SCLE) can be difficult to clinically and histologically differentiate from EM (4,5). Patients with SLE also have a higher likelihood of developing adverse drug reactions (6). The inherent complexity of SLE may make for delayed and oftentimes difficult diagnosis, especially in a country where immunologic tests are expensive and rheumatologists are scarce. When patients do occasionally present with recurrences of skin lesions in the spectrum of EM, Steven-Johnson syndrome, and toxic epidermal necrolysis in the absence of a definite inciting agent, undiagnosed lupus may indeed be present in some of these individuals and should be considered in the differential diagnosis. In conclusion, while it is very rare, SLE may present first with recurrent episodes of EM-like rash. Despite the various possibilities which underlie their association, prompt identification and treatment of SLE in patients presenting with EM is important to prevent death or irreversible organ damage.


Assuntos
Eritema Multiforme/diagnóstico , Eritema Multiforme/etiologia , Lúpus Eritematoso Sistêmico/complicações , Lúpus Eritematoso Sistêmico/diagnóstico , Adulto , Feminino , Humanos , Nigéria
8.
BMJ Case Rep ; 12(8)2019 Aug 20.
Artigo em Inglês | MEDLINE | ID: mdl-31434669

RESUMO

Good's syndrome (GS) is a rare, adult-onset combined B cell and T cell immunodeficiency with an associated thymoma. These patients have an increased risk of bacterial, fungal, viral and opportunistic infections. This report describes a 75-year-old female patient who presented with a full body rash and an anterior mediastinal mass. She underwent a biopsy of her rash and mass, which revealed erythema multiforme and WHO Type A thymoma, respectively. During her hospitalisation, she was also found to have oropharyngeal candidiasis, methicillin-susceptible Staphylococcus aureus bacteraemia and herpes simplex virus type 2 (HSV-2) skin lesions. Based on the number of infections and severity of her rash, an immunocompromised state was suspected. Immunological testing revealed a B cell and T cell deficiency as well as low serum immunoglobulins. This combination of hypogammaglobulinaemia and thymoma led to a diagnosis of GS. While there have been many case reports of GS, this is the first report of the immunodeficiency presenting with erythema multiforme.


Assuntos
Antibacterianos/uso terapêutico , Reabilitação Cardíaca , Eritema Multiforme/diagnóstico , Síndromes de Imunodeficiência/diagnóstico , Infecções Estafilocócicas/diagnóstico , Timoma/diagnóstico , Combinação Trimetoprima e Sulfametoxazol/uso terapêutico , Idoso , Eritema Multiforme/complicações , Eritema Multiforme/tratamento farmacológico , Eritema Multiforme/imunologia , Evolução Fatal , Feminino , Hidratação , Humanos , Hospedeiro Imunocomprometido , Síndromes de Imunodeficiência/complicações , Síndromes de Imunodeficiência/tratamento farmacológico , Síndromes de Imunodeficiência/imunologia , Pneumonia , Choque Séptico , Infecções Estafilocócicas/tratamento farmacológico , Infecções Estafilocócicas/imunologia , Timoma/imunologia
9.
J Am Acad Dermatol ; 81(3): 813-822, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31331726

RESUMO

BACKGROUND: Erythema multiforme (EM) is an acute inflammatory mucocutaneous condition. EM is rarely described in children and infants. OBJECTIVE: To investigate the triggers, clinical manifestations, and treatment of pediatric EM. METHODS: Systematic literature review of pediatric EM. RESULTS: After full-text article review, we included 113 articles, representing 580 patients. The mean age was 5.6 years, ranging 0.1-17 years. Infectious agents were the main triggers: herpes simplex virus (HSV) in 104 patients (17.9%) and Mycoplasma pneumoniae in 91 patients (15.7%). In total, 140 cases (24.1%) were drug-related and 89 cases (15.3%) had other triggers, such as vaccines (19 patients, 3.2%). In total, 229 patients had EM major (39.5%). Treatment was supportive care only (180 patients, 31.1%), systemic corticosteroids (115 patients, 19.8%), antivirals (85 patients, 14.6%), and antibiotics (66 patients, 11.3%), mostly macrolides (45 patients, 7.7%). Long-term sequelae were rare (1.3%). Pediatric EM was reported in 19 infants (3.2%). The main trigger was vaccination (9 patients). Infantile EM was EM major in 2 cases and EM minor in 17. Infants were less prone to develop EM major than older children (P < .01). Pediatric EM was recurrent in 83 cases (14.3%), which was triggered by HSV in 36 patients (61%). Recurrence affected older children. LIMITATIONS: Potential confusion between Steven Johnson syndrome and EM major in addition to publication bias. CONCLUSION: Pediatric EM is a rare disease, mainly triggered by infections. This condition can affect all mucosal surfaces, most commonly the oral mucosae. The diagnosis is clinical, and management relies on supportive care. Vaccines are a particular trigger in infants. Recurrent cases are most commonly linked to HSV. Dermatologists and pediatricians should be aware of this potentially recurrent and severe condition.


Assuntos
Eritema Multiforme/etiologia , Cuidados Paliativos/métodos , Doenças Raras/etiologia , Vacinação/efeitos adversos , Adolescente , Fatores Etários , Antibacterianos/uso terapêutico , Antivirais/uso terapêutico , Criança , Pré-Escolar , Diagnóstico Diferencial , Erupção por Droga/diagnóstico , Erupção por Droga/etiologia , Erupção por Droga/terapia , Eritema Multiforme/diagnóstico , Eritema Multiforme/terapia , Glucocorticoides/uso terapêutico , Herpes Simples/complicações , Herpes Simples/tratamento farmacológico , Humanos , Lactente , Mucosa Bucal/microbiologia , Mucosa Bucal/patologia , Pneumonia por Mycoplasma/complicações , Pneumonia por Mycoplasma/tratamento farmacológico , Doenças Raras/diagnóstico , Doenças Raras/terapia , Recidiva , Índice de Gravidade de Doença
10.
Am Fam Physician ; 100(2): 82-88, 2019 07 15.
Artigo em Inglês | MEDLINE | ID: mdl-31305041

RESUMO

Erythema multiforme is an immune-mediated reaction that involves the skin and sometimes the mucosa. Classically described as target-like, the erythema multiforme lesions can be isolated, recurrent, or persistent. Most commonly, the lesions of erythema multiforme present symmetrically on the extremities (especially on extensor surfaces) and spread centripetally. Infections, especially herpes simplex virus and Mycoplasma pneumoniae, and medications constitute most of the causes of erythema multiforme; immunizations and autoimmune diseases have also been linked to erythema multiforme. Erythema multiforme can be differentiated from urticaria by the duration of individual lesions. Erythema multiforme lesions are typically fixed for a minimum of seven days, whereas individual urticarial lesions often resolve within one day. Erythema multiforme can be confused with the more serious condition, Stevens-Johnson syndrome; however, Stevens-Johnson syndrome usually contains widespread erythematous or purpuric macules with blisters. The management of erythema multiforme involves symptomatic treatment with topical steroids or antihistamines and treating the underlying etiology, if known. Recurrent erythema multiforme associated with the herpes simplex virus should be treated with prophylactic antiviral therapy. Severe mucosal erythema multiforme can require hospitalization for intravenous fluids and repletion of electrolytes.


Assuntos
Eritema Multiforme/diagnóstico , Eritema Multiforme/terapia , Eritema Multiforme/etiologia , Humanos
12.
BMJ Case Rep ; 12(5)2019 May 14.
Artigo em Inglês | MEDLINE | ID: mdl-31092494

RESUMO

Erythema multiforme is a skin disorder characterised by target epithelial eruption, which is mainly caused by infection or drugs. In this case, we report an erythema multiforme like reaction caused by contact dermatitis against wood, especially santos rosewood. During the hospitalisation, we performed a patch test with lumber used in the patient's workplace, and recognised a positive response to multiple woods and a simultaneous recurring eruption (flare up) outside of the test site. The findings from this case of contact dermatitis caused by frequently used industrial wood type is important for the management of occupational environments. A review of the literature on erythema multiforme like reaction due to contact dermatitis, including past case reports, has also been provided.


Assuntos
Eritema Multiforme/etiologia , Doenças Profissionais/etiologia , Madeira/efeitos adversos , Administração Cutânea , Adulto , Clobetasol/administração & dosagem , Dermatite Alérgica de Contato , Eritema Multiforme/diagnóstico , Eritema Multiforme/tratamento farmacológico , Fabaceae/efeitos adversos , Humanos , Masculino , Doenças Profissionais/diagnóstico , Doenças Profissionais/tratamento farmacológico , Testes do Emplastro , Taxaceae/efeitos adversos
13.
J Nepal Health Res Counc ; 17(1): 119-121, 2019 Apr 28.
Artigo em Inglês | MEDLINE | ID: mdl-31110391

RESUMO

Porphyria cutaneatarda, is the most common type of porphyria.It is characterized by defective uroporphyrinogen III decarboxylase enzyme.It presents with erosion, bulla with milia formation and sometimes with hypertrichosis and abnormal pigmentation mostly on the photo-exposed sites. A urine fluorescence of coral red color helps in the diagnosis. Here, we present a rare case of porphyria cutanea tarda in a 15 years old male who presented with multiple targetoid plaques. Keywords: Erythema-multiforme; porphyria cutanea tarda; targetoid.


Assuntos
Eritema Multiforme/diagnóstico , Porfiria Cutânea Tardia/diagnóstico , Adolescente , Diagnóstico Diferencial , Eritema Multiforme/patologia , Humanos , Masculino , Porfiria Cutânea Tardia/patologia , Pele/patologia
14.
Clin Dermatol ; 37(2): 148-158, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-30981295

RESUMO

We have explored the rash that appears as target lesions, with the central and dominant diseases belonging to the Stevens-Johnson syndrome/toxic epidermal necrolysis group. After presenting the clinical patterns of an individual target lesion and classifying them into different types of lesions, the contribution has been organized with groups characterized by such specific findings according to the type of lesion: flat or raised, typical or atypical, presence or absence of fever, presence or absence of mucosal ulcerations, presence or absence of arthralgias, and/or internal organ involvement. Other specific features, such as histologic appearance, immunofluorescence findings, and laboratory changes, are considered. We provide clinicians with an algorithmic, systematic, and logical approach to diagnose the condition of the patients who present with targetoid lesions, and enable them to differentiate between those with serious systemic and life-threatening diseases from others with ordinary skin ailments.


Assuntos
Eritema Multiforme/complicações , Eritema Multiforme/diagnóstico , Exantema/diagnóstico , Exantema/etiologia , Pele/patologia , Síndrome de Stevens-Johnson/complicações , Síndrome de Stevens-Johnson/diagnóstico , Artralgia , Diagnóstico Diferencial , Eritema Multiforme/patologia , Exantema/patologia , Feminino , Febre , Imunofluorescência , Doença Enxerto-Hospedeiro/complicações , Doença Enxerto-Hospedeiro/diagnóstico , Doença Enxerto-Hospedeiro/patologia , Humanos , Masculino , Síndrome de Linfonodos Mucocutâneos/complicações , Síndrome de Linfonodos Mucocutâneos/diagnóstico , Síndrome de Linfonodos Mucocutâneos/patologia , Membrana Mucosa , Penfigoide Bolhoso/complicações , Penfigoide Bolhoso/diagnóstico , Penfigoide Bolhoso/patologia , Gravidez , Complicações na Gravidez , Choque Séptico/complicações , Choque Séptico/diagnóstico , Choque Séptico/patologia , Síndrome de Stevens-Johnson/patologia , Sífilis/complicações , Sífilis/diagnóstico , Sífilis/patologia , Úlcera , Urticária/complicações , Urticária/diagnóstico , Urticária/patologia
16.
Am J Case Rep ; 20: 212-218, 2019 Feb 18.
Artigo em Inglês | MEDLINE | ID: mdl-30773528

RESUMO

BACKGROUND The hallmark of bullous pemphigoid (BP) is widespread tense blisters arising on normal or erythematous skin, often with marked pruritus, the diagnosis of which is confirmed by direct immunofluorescence (DIF). BP is an autoimmune process that can be induced, though rarely, by medications. Drug-induced BP often has atypical clinical presentation, which requires a good understanding of other dermatological conditions with similar presentations, in particular, bullous subtype of erythema multiforme. End organ involvement warrants differentiating it from one of the severe cutaneous adverse reaction (SCAR) syndromes. CASE REPORT A 76-year-old African American male presented with extensive targetoid purplish skin lesions that clinically resembled atypical erythema multiforme, and one tense blister that raised a concern for BP. The patient presented 6 weeks after treatment with cephalexin for a urinary tract infection. Initial workup showed serum eosinophilia, acute kidney injury and eosinophiluria requiring deliberations on SCAR syndromes. A skin biopsy at an intralesional location showed a negative DIF, however, a skin biopsy at a perilesional site showed a positive DIF, confirming the diagnosis of BP. CONCLUSIONS This case demonstrates an atypical presentation of BP induced by drugs. It emphasizes the need for a greater level of awareness of diagnosis and treatment of the various entities that fall under adverse drug reactions in the elderly. It also highlights the need for appropriate choice of skin biopsy techniques (intralesional versus perilesional) to avoid misdiagnosis, as well as lessons on how to approach dermatologic conditions with end organ involvement for hospitalists and other medical professionals who routinely deal with undifferentiated disease conditions.


Assuntos
Nefrite Intersticial/etiologia , Penfigoide Bolhoso/induzido quimicamente , Penfigoide Bolhoso/diagnóstico , Idoso , Antibacterianos/efeitos adversos , Cefalexina/efeitos adversos , Diagnóstico Diferencial , Eritema Multiforme/diagnóstico , Humanos , Masculino
17.
J Dermatol ; 46(3): 234-239, 2019 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-30623481

RESUMO

Growing and sustainable consumption of health-care products raises a controversial issue underlying the reliability of an in vitro diagnostic approach for adverse skin reaction. This report aimed to: (i) discuss the causative nature of a commercial dietary supplement composed of natural ingredients, particularly an Euglena-containing product, suspicious for erythema multiforme in our exemplified case; and (ii) to address the assay suitability of the lymphocyte transformation test (LTT) for identifying allergic reaction to any ingredient(s) of the product. A Japanese elderly man developed erythema multiforme after intake of a commercially available natural dietary product, whose LTT was positive. His clinical course and positive LTT suggested a provisional diagnosis of natural dietary product-induced eruption. We conducted an inquiry survey for the standard LTT with any commercial products containing Euglena in three major Japanese laboratory services and identified 22 subjects, almost all of whom (21/22, 95.6%) showed a positive LTT for any Euglena-containing products as a suspected causative. Seven normal healthy volunteers who had no intake history of Euglena-containing products showed an equivalent LTT positivity rate with the same product taken by our case; culprit components of the product included Euglena, Angelica keiskei, Barley grass and Chlorella. A cell-free culture system and enzyme-linked immunoassay suggest that the high LTT positivity relies on the non-specific lymphoproliferative activity, and not contamination of uncharacterized microorganisms and endotoxins. Because of the constitutive false positivity of LTT, this assay is unreliable for in vitro supportive diagnosis of adverse skin events caused by dietary products containing particular natural ingredients, as well as herbal materials.


Assuntos
Suplementos Nutricionais/efeitos adversos , Eritema Multiforme/diagnóstico , Euglena/química , Testes Cutâneos/normas , Idoso , Ensaio de Imunoadsorção Enzimática , Eritema Multiforme/sangue , Eritema Multiforme/induzido quimicamente , Reações Falso-Positivas , Humanos , Ativação Linfocitária , Masculino , Reprodutibilidade dos Testes
19.
Int J STD AIDS ; 30(3): 304-309, 2019 03.
Artigo em Inglês | MEDLINE | ID: mdl-30482099

RESUMO

Early syphilis can rarely cause erythema multiforme-type eruptions as well as triggering erythema multiforme (EM). EM-like lesions in secondary syphilis are characterized by clinical features of EM and laboratory tests consistent with secondary syphilis and the skin histology shows predominantly a plasma cell infiltrate with the presence of treponemes. When EM is triggered by early syphilis, the skin histology shows mixed inflammatory cells usually in the absence of treponemes in the skin lesion. There may also be mixed histology with the presence of treponemes in the absence of a plasma cell infiltrate and vice versa. We describe a case of secondary syphilis presenting as EM with bullae and histology showing EM features without a plasma cell infiltrate but positive for Treponema pallidum by immunohistochemical staining. The patient was also coinfected with cytomegalovirus, human immunodeficiency virus, and anal warts. The EM eruptions resolved with treatment for secondary syphilis with benzathine penicillin G.


Assuntos
Condiloma Acuminado/complicações , Infecções por Citomegalovirus/complicações , Soropositividade para HIV/complicações , Homossexualidade Masculina , Sífilis/diagnóstico , Antibacterianos/administração & dosagem , Antibacterianos/uso terapêutico , Coinfecção , Citomegalovirus/isolamento & purificação , Eritema Multiforme/diagnóstico , Eritema Multiforme/tratamento farmacológico , Humanos , Masculino , Papillomaviridae/isolamento & purificação , Penicilina G Benzatina/uso terapêutico , Sífilis/tratamento farmacológico , Sorodiagnóstico da Sífilis , Resultado do Tratamento , Treponema pallidum/isolamento & purificação
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