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El síndrome de DRESS (por sus siglas en inglés Drug Reaction with Eosinophilia and Systemic Symptoms) representa una farmacodermia grave con diferentes manifestaciones clínicas y paraclínicas secundarias a una reacción de hipersensibilidad farmacológica. Su incidencia exacta es desconocida pero se estima entre 1 a 1000 y 1 a 10000 casos de exposición a fármacos asociados. Se caracteriza por dermatosis generalizada extensa en conjunto con afección orgánica, linfadenopatia, eosinofilia y linfocitosis atípica. Entre los fármacos comúnmente asociados se encuentran anticomiciales aromáticos, carbamazepina, sulfonamidas y el alopurinol. Mediante el uso de la puntuación RegiSCAR es posible confirmar o descartar una sospecha de diagnóstico. El tratamiento depende de la severidad de presentación incluyendo esteroides tópicos hasta esteroide sistémico de duración variable dependiendo respuesta clínica y bioquímica. Se reporta tasas de mortalidad del 10 al 20% siendo la insuficiencia hepática la principal causa de muerte en estos pacientes. Se presenta el caso de un paciente femenino de 71 años de edad que, posterior a tratamiento con alopurinol, debuta con eritrodermia secundaria a Síndrome de DRESS.
DRESS syndrome (Drug Reaction with Eosinophilia and Systemic Symptoms) represents severe pharmacodermia with different clinical and paraclinical manifestations secondary to a drug hypersensitivity reaction. The exact incidence is unknown, but it is estimated to be between 1 in 1,000 and 1 in 10,000 cases of exposure to associated drugs. It is characterized by extensive generalized dermatosis, in conjunction with organic involvement, lymphadenopathy, eosinophilia, and atypical lymphocytosis. Commonly associated drugs include aromatic anticonvulsants, carbamazepine, sulfonamides, and allopurinol. By using the RegiSCAR score, it is possible to confirm or rule out a suspected diagnosis. Treatment depends on the severity of presentation, including topical steroids to systemic steroids of variable duration, depending on clinical and biochemical responses. Mortality rates of 10 - 20% have been reported, with liver failure being the main cause of death in these patients. We present the case of a 71-year-old female patient who, after treatment with allopurinol, developed erythroderma secondary to DRESS Syndrome.
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Sézary syndrome (SS) is a rare and aggressive type of cutaneous T-cell lymphoma, with an abnormal inflammatory response in affected skin. The cytokines IL-1B and IL-18, as key signaling molecules in the immune system, are produced in an inactive form and cleave to the active form by inflammasomes. In this study, we assessed the skin, serum, peripheral mononuclear blood cell (PBMC) and lymph-node samples of SS patients and control groups (healthy donors (HDs) and idiopathic erythroderma (IE) nodes) to investigate the inflammatory markers IL-1B and IL-18 at the protein and transcript expression levels, as potential markers of inflammasome activation. Our findings showed increased IL-1B and decreased IL-18 protein expression in the epidermis of SS patients; however, in the dermis layer, we detected increased IL-18 protein expression. In the lymph nodes of SS patients at advanced stages of the disease (N2/N3), we also detected an enhancement of IL-18 and a downregulation of IL-1B at the protein level. Moreover, the transcriptomic analysis of the SS and IE nodes confirmed the decreased expression of IL1B and NLRP3, whereas the pathway analysis indicated a further downregulation of IL1B-associated genes. Overall, the present findings showed compartmentalized expressions of IL-1B and IL-18 and provided the first evidence of their imbalance in patients with Sézary syndrome.
Assuntos
Interleucina-18 , Síndrome de Sézary , Neoplasias Cutâneas , Humanos , Dermatite Esfoliativa/metabolismo , Inflamassomos/metabolismo , Interleucina-18/genética , Interleucina-18/metabolismo , Leucócitos Mononucleares/metabolismo , Síndrome de Sézary/metabolismo , Pele/metabolismo , Neoplasias Cutâneas/metabolismoRESUMO
BACKGROUND: Erythroderma, characterized by erythema and scaling that affects at least 90% of the body, has diverse causes. Most of the clinical manifestations and laboratory findings are nonspecific, making diagnosis challenging. MATERIAL AND METHODS: Retrospective study of patients treated between January 1, 2010, and June 1, 2020. We reviewed the records to identify all patients with erythroderma who were hospitalized in Hospital Italiano de Buenos Aires and followed for at least 6 months. We extracted information on clinical histories, the characteristics of the episodes, laboratory and histopathologic findings, and clinical course. RESULTS: Seventy patients were studied. The mean age at onset was 63 years, and the ratio of men to women was 1.2:1. Adverse drug reactions caused the largest proportion of the rashes (48%), and vancomycin was the most common culprit (involved in 30% of the cases). The next most frequent cause was a preexisting skin disease, psoriasis being the most common (in 42%). The clinicopathologic correlation was adequate for diagnosis after the first biopsy in 40% of patients, but the diagnostic yield increased to 76% with the second biopsy. The largest number of biopsies required was 8, in 2 patients. The outcome was favorable in 92% of the cases. CONCLUSION: Adverse reactions to medications accounted for the largest proportion of erythroderma cases in this series, and vancomycin was the main culprit. We found no statistically significant associations among the variables studied. Nor did we identify potential predictors of causes, poor outcomes, or mortality.
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Dermatite Esfoliativa , Efeitos Colaterais e Reações Adversas Relacionados a Medicamentos , Feminino , Hospitais de Ensino , Humanos , Masculino , Estudos Retrospectivos , VancomicinaRESUMO
RESUMEN La eritrodermia es una erupción eritematosa más o menos escamosa que afecte a más del 90% de la superficie corporal. Se trata de una enfermedad capaz de comprometer el pronóstico vital y que puede complicarse con desequilibrios hidroelectrolíticos, trastornos de la termorregulación, infecciones, así como con fallo cardiovascular. En la presente publicación referimos el caso de un paciente masculino de 72 años con antecedentes de hipertensión arterial y de Lepra que recibió poliquimioterapia, es ingresado en el servicio de dermatología en el período de observación, pues hace alrededor de un año comenzó a presentar enrojecimiento de la piel y múltiples escamas. Al momento del examen se observa paciente eritrodérmico, ectropión, distrofia ungueal importante, con adenopatías palpables axilares e inguinales, asociado un prurito intenso generalizado. Se realizan estudios para el diagnostico incluyendo biopsia de ganglios inguinal y de piel, esta última compatible con Micosis Fungoide. Actualmente se reconocen tres variantes eritrodérmicas en el linfoma cutáneo de células T: el síndrome de Sézary, la Eritrodermia en micosis fungoide y la Eritrodermia en linfomas cutáneos de células T:No Especificada. Si bien se entiende al síndrome de Sézary como una expresión leucémica del linfoma cutáneo de células T eritrodérmico, con numerosas células de Sézary en piel, sangre y otros tejidos, la Eritrodermia en micosis fungoide se determinaría ante la ausencia de estos mismos hallazgos hematológicos e histológico ante un cuadro clínico eritrodérmico. Se hace necesaria la publicación del artículo por la forma de presentación de la micosis fungoide, teniendo en cuenta que esta dermatosis tiene un sin número de diagnósticos diferenciales que la hace la gran simuladora en la Dermatología.
ABSTRACT Erythroderma is a more or less scaly erythematous rash that affects more than 90% of the body surface area. It is a disease capable of compromising the vital prognosis and that can be complicated by hydroelectrolytic imbalances, thermoregulation disorders, infections, as well as cardiovascular failure. In this publication we refer to the case of a 72-year-old male patient with a history of high blood pressure and leprosy who received polychemotherapy, is admitted to the dermatology service in the observation period, as about a year ago he began to develop redness of the skin and multiple scales. At the time of the examination, an erythrothermal patient, ectropion, important ungueal dystrophy, with axillary and inguinal palpable adenopathies, associated with a widespread intense itching, is observed. Studies are conducted for diagnosis including inguinal and skin node biopsy, the latter compatible with Mycosis Fungoide. Three erythrodermal variants are currently recognized in skin T-cell lymphoma: Sézary syndrome, Erythroderma in fungoid mycosis, and Erythroderma in skin T-cell lymphomas: Undepede. While Sézary syndrome is understood as a leukemia expression of erythrodermal T-cell skin lymphoma, with numerous Sézary cells in skin, blood and other tissues, Erythroderma in fungoid mycosis would be determined in the absence of these same haematological and histological findings before an erythrodermal clinical picture. It is necessary to publish the article by the form of presentation of fungoid mycosis, taking into account that this dermatosis has a number of differential diagnoses that makes it the great simulator in Dermatology.
RESUMO Eritrodermia é uma erupção cutânea mais ou menos erethema que afeta mais de 90% da superfície do corpo. É uma doença capaz de comprometer o prognóstico vital e que pode ser complicada por desequilíbrios hidroelegóticos, distúrbios termoreguladores, infecções, bem como insuficiência cardiovascular. Nesta publicação, refere-se ao caso de um paciente do sexo masculino de 72 anos com histórico de pressão alta e hanseníase que recebeu poliquimoterapia, é internado no serviço de dermatologia no período de observação, pois há cerca de um ano começou a desenvolver vermelhidão da pele e múltiplas escamas. No momento do exame, observa-se um paciente eritrótermal, ectropion, importante distrofia ungueal, com adenopatias palpáveis axilares e inguinais, associadas a uma coceira intensa generalizada. São realizados estudos para diagnóstico, incluindo biópsia inguinal e nódulo da pele, este último compatível com Mycosis Fungoide. Três variantes eritromicais são atualmente reconhecidas em linfoma de células T da pele: síndrome de Sézary, Erythroderma na micose fungoide, e Erythroderma em linfomas de células T da pele: Undepede. Enquanto a síndrome de Sézary é entendida como uma expressão de leucemia de linfoma de pele de células T eritorodérmica, com numerosas células Sézary na pele, sangue e outros tecidos, eritrorgema em micose fungoide seria determinada na ausência desses mesmos achados haematológicos e histológicos diante de um quadro clínico eritroxermal. É necessário publicar o artigo sob a forma de apresentação da micose fungoide, tendo em vista que essa dermatose possui uma série de diagnósticos diferenciais que o torna o grande simulador em Dermatologia.
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Abstract Annular epidermolytic ichthyosis is a rare subtype of epidermolytic ichthyosis that is characterized by erythematous, polycyclic, and migratory scaly plaques accompanied by palmoplantar keratoderma. This report presents the case of an 8-year-old girl who developed migratory, erythematous, scaly plaques associated with palmoplantar keratoderma. The initial hypothesis was erythrokeratodermia variabilis et progressiva; however, the finding of epidermolytic hyperkeratosis in histopathological examination led to the diagnosis of annular epidermolytic ichthyosis.
Assuntos
Humanos , Feminino , Criança , Hiperceratose Epidermolítica , Pele , Ceratodermia Palmar e Plantar , ExantemaRESUMO
Annular epidermolytic ichthyosis is a rare subtype of epidermolytic ichthyosis that is characterized by erythematous, polycyclic, and migratory scaly plaques accompanied by palmoplantar keratoderma. This report presents the case of an 8-year-old girl who developed migratory, erythematous, scaly plaques associated with palmoplantar keratoderma. The initial hypothesis was erythrokeratodermia variabilis et progressiva; however, the finding of epidermolytic hyperkeratosis in histopathological examination led to the diagnosis of annular epidermolytic ichthyosis.
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Hiperceratose Epidermolítica , Criança , Exantema , Feminino , Humanos , Ceratodermia Palmar e Plantar , PeleRESUMO
BACKGROUND: Autosomal recessive congenital ichthyoses (ARCI) have been associated with different phenotypes including: harlequin ichthyosis (HI), congenital ichthyosiform erythroderma (CIE), and lamellar ichthyosis (LI). While pathogenic variants in all ARCI genes are associated with LI and CIE phenotypes, the unique gene associated with HI is ABCA12. In HI, the most severe ARCI form, pathogenic variants in both ABCA12 gene alleles usually have a severe impact on protein function. The presence of at least one non-truncating variant frequently causes a less severe congenital ichthyosis phenotype (LI and CIE). METHODS: We report the case of a 4-year-old Ecuadorian boy with a severe skin disease. Genetic diagnosis was performed by NGS. In silico predictions were performed using Alamut software v2.11. A review of the literature was carried out to identify all patients carrying ABCA12 splice-site and missense variants, and to explore their genotype-phenotype correlations. RESULTS: Genetic testing revealed a nonsense substitution, p.(Arg2204*), and a new missense variant, p.(Val1927Leu), in the ABCA12 gene. After performing in silico analysis and a comprehensive review of the literature, we conclude that p.(Val1927Leu) affects a well conserved residue which could either disturb the protein function or alter the splicing process, both alternatives could explain the severe phenotype of our patient. CONCLUSION: This case expands the spectrum of ABCA12 reported disease-causing variants which is important to unravel genotype-phenotype correlations and highlights the importance of missense variants in the development of HI.
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Transportadores de Cassetes de Ligação de ATP/genética , Ictiose Lamelar/genética , Mutação com Perda de Função , Fenótipo , Pré-Escolar , Códon sem Sentido , Humanos , Ictiose Lamelar/patologia , Masculino , Mutação de Sentido Incorreto , Sítios de Splice de RNARESUMO
INTRODUCCIÓN: La eritrodermia es un síndrome inflamatorio cutáneo infrecuente caracterizado por compromiso eritematoso generalizado y descamación, de más del 90% de superficie cutánea total. OBJETIVO: Caracterizar clínica e histopatológicamente a los pacientes con eritrodermia en un hospital universitario chileno. METODOLOGÍA: Estudio retrospectivo, realizado en el Hospital Clínico Universidad de Chile, basado en revisión de fichas clínicas e informes histopatológicos de pacientes con eritrodermia, entre 2005 y 2018. Se evaluó edad, sexo y variables clínicas (co-morbilidades, síntomas, días de evolución, ingreso hospitalario, informe histopatológico, diagnóstico y evolución). RESULTADOS: Total de 28 pacientes, 18 hombres (64%), edad promedio 59 años. Causa más frecuente de eritrodermia fue dermatosis pre-exis-tentes, con 15 casos (54%), que incluyen: psoriasis 9 (32%), dermatitis de contacto 3 (11%), PRP 2 (7%), dermatitis atópica 1 (4%). A estas le siguen: reacción adversa medicamentosa 6 (21%), idiopática 6 (21%) y Síndrome de Sezary 1 (4%). CONCLUSIÓN: El presente estudio corresponde a la primera serie de eritrodermias realizada en Chile. Destacan las dermatosis preexistentes como la principal causa, lo que se correlaciona con la literatura.
INTRODUCTION: Erythroderma is an infrequent cutaneous inflammatory disorder characterized by generalized erythematous compromise and desquamation, of more than 90% of total cutaneous surface. OBJECTIVE: Clinical and histopathological cha-racterization of patients with erythroderma in a Chilean university hospital. METHODOLOGY: Retrospective study, performed at the University of Chile Clinical Hospital, based on review of clinical records and histopatho-logical reports of patients with erythroderma, between 2005 and 2018. Age, sex and clinical variables were evaluated (co-morbidities, symp-toms, days of evolution, hospital admission, histopathological report, diagnosis and evolu-tion). RESULTS: A total of 28 patients, 18 were men (64%), average age 59 years. Most frequent cause of erythroderma was pre-existing dermatosis, with 13 cases (52%), which included: psoriasis 9 (32%), contact dermatitis 3 (11%), PRP 2 (7%), atopic dermatitis 1 (4%). These are followed by adverse drug eruption 6 (21%), idiopathic 6 (21%) and Sezary syndrome 1 (4%). CONCLUSION: The present study corresponds to the first series of erythrodermas performed in Chile. The pre-existing dermatoses were the main cause of erythroderma, which coincides with other reports.
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Humanos , Masculino , Feminino , Adolescente , Adulto , Pessoa de Meia-Idade , Idoso , Idoso de 80 Anos ou mais , Adulto Jovem , Dermatite Esfoliativa/etiologia , Dermatite Esfoliativa/patologia , Dermatite Esfoliativa/epidemiologia , Psoríase/complicações , Psoríase/epidemiologia , Evolução Clínica , Chile , Estudos Transversais , Estudos Retrospectivos , Toxidermias/complicações , Toxidermias/epidemiologia , Dermatite Atópica/complicações , Dermatite Atópica/epidemiologia , Dermatite de Contato/complicações , Dermatite de Contato/epidemiologiaRESUMO
BACKGROUND: Erythroderma is characterized by erythema and scaling affecting more than 90% of the body surface area. Inflammatory, neoplastic and, more rarely, infectious diseases may culminate with erythroderma. Diagnosis of the underlying disorder is therefore crucial to institute the appropriate therapy. Leprosy is a chronic infectious disease that is endemic in Brazil. Here we present an unusual case of leprosy and reversal reaction causing erythroderma, and we discuss the underlying immunological mechanisms which could contribute to the generalized skin inflammation. CASE PRESENTATION: We report a case of a patient with reversal reaction (RR) in borderline borderline leprosy presenting with erythroderma and neural disabilities. Histopathology of the skin showed regular acanthosis and spongiosis in the epidermis and, in the dermis, compact epithelioid granulomas as well as grouped and isolated bacilli. This duality probably reflects the transition from an anergic/multibacillary state to a state of more effective immunity and bacillary control, typical of RR. Leprosy was successfully treated with WHO's multidrug therapy, plus prednisone for controlling the RR; the erythroderma resolved in parallel with this treatment. Immunologic studies showed in situ predominance of IFNγ + over IL-4+ lymphocytes and of IL-17+ over Foxp3+ lymphocytes, suggesting an exacerbated Th-1/Th-17 immunoreactivity and poor Th-2 and regulatory T-cell responses. Circulating Tregs were also diminished. We hypothesize that the flare-up of anti-mycobacteria immunoreactivity that underlies RR may have triggered the intense inflammatory skin lesions that culminated with erythroderma. CONCLUSIONS: This case report highlights the importance of thorough clinical examination of erythrodermic patients in search for its etiology and suggests that an intense and probably uncontrolled leprosy RR can culminate in the development of erythroderma.
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Dermatite Esfoliativa/etiologia , Hanseníase Dimorfa/complicações , Pele/patologia , Anti-Inflamatórios/uso terapêutico , Biópsia , Dermatite Esfoliativa/tratamento farmacológico , Dermatite Esfoliativa/patologia , Quimioterapia Combinada , Humanos , Interferon gama/metabolismo , Hansenostáticos/uso terapêutico , Hanseníase Dimorfa/tratamento farmacológico , Hanseníase Dimorfa/imunologia , Linfócitos/metabolismo , Masculino , Pessoa de Meia-Idade , Prednisolona/uso terapêutico , Linfócitos T Reguladores/imunologiaRESUMO
Resumen La infección por VIH puede presentarse con distintas manifestaciones cutáneas, que en algunas ocasiones son consideradas marcadores de infección. Una posible manifestación es la eritrodermia psoriática, que corresponde a una forma generalizada de psoriasis. Presentamos un caso clínico de un hombre joven en que se sospechó una infección por VIH por un cuadro de eritrodermia psoriática confirmada por biopsia, asociado a un sarcoma de Kaposi. Posteriormente, la infección por VIH fue confirmada por serología. Se manejó con terapia antirretroviral, con buena respuesta al mes de tratamiento. La eritrodermia psoriática se puede considerar un marcador cutáneo de infección por VIH cuando ocurre en pacientes previamente sanos o con psoriasis recalcitrante.
HIV infection can be manifested with different skin symptoms, which are sometimes considered infection markers. Erythrodermic psoriasis is a possible manifestation, which is a widespread form of psoriasis. We report a clinical case of a young man suspected of HIV infection due to a psoriatic erythroderma confirmed by biopsies, associated with Kaposi sarcoma. Afterwards, HIV infection was confirmed by serological tests. Antiretroviral therapy was started, with positive response at one month of treatment. Erythrodermic psoriasis can be considered a skin marker of HIV infection when occurs in previously healthy patients or in recalcitrant psoriasis.
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Humanos , Masculino , Adulto , Psoríase/virologia , Infecções por HIV/complicações , Dermatite Esfoliativa/virologia , Psoríase/patologia , Pele/patologia , Biópsia , Infecções por HIV/patologia , Infecções por HIV/tratamento farmacológico , Dermatite Esfoliativa/patologia , AntirretroviraisRESUMO
Resumo A ictiose lamelar é uma doença congênita de herança autossômica recessiva que se caracteriza clinicamente por descamação de toda a superfície corporal devido hiperceratose mucocutânea que pode levar a comprometimento oftalmológico. Neste estudo relatamos um caso de cisto ductal de glândula lacrimal em paciente portador de ictiose lamelar atendido no Hospital Oftalmológico de Anápolis.
Abstract Lamellar ichthyosis is a congenital disease autosomal recessive which is characterized clinically by peeling of all the body surface due hyperkeratosis mucocutaneous that can cause ocular involvement. We reported a case of ductal cyst of the lacrimal gland in patient with lamellar ichthyosis attended in the Ophthalmological Hospital of Anápolis.
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Humanos , Feminino , Pessoa de Meia-Idade , Eritrodermia Ictiosiforme Congênita/complicações , Cistos/diagnóstico , Cistos/etiologia , Doenças do Aparelho Lacrimal/diagnóstico , Doenças do Aparelho Lacrimal/etiologia , Espectroscopia de Ressonância Magnética , Cistos/cirurgia , Cistos/patologia , Doenças do Aparelho Lacrimal/cirurgiaRESUMO
BACKGROUND: Dermatophytoses are skin superficial mycoses in which clinical manifestations are directly related to the virulence of the infecting microorganism or the host immunity. CASE REPORT: We describe a severe case of dermatophytosis associated with exfoliative erythroderma, substantial palmoplantar keratoderma, onychodystrophy affecting all nails, diffuse non-scarring alopecia and tissue fungal invasion by Trichophyton tonsurans, which led us to the diagnosis of AIDS. Direct examination and culture for fungi from skin scraping from two different sites were performed. Biopsy and histopathological exam were also performed on three different sites. Direct examination of the lesions' scraping revealed septate hyaline hyphae and arthroconidia, identified as Trichophyton tonsurans by culture in glucose Sabouraud agar and Mycosel agar. A scalp biopsy revealed follicular fungal invasion and Majocchi's granuloma. Due to the severity of the presentation we requested an anti-HIV serology, which was positive. The patient was treated with itraconazole, 200mg/day, for 120 days, which promoted a complete regression of the lesions. CONCLUSIONS: Severe and atypical forms of dermatophytosis could lead to a diagnosis of AIDS.
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Erythroderma consists on erythema and scaling that covers more than 90% of the cutaneous surface. It has multiple causes and for the most common we can use the acronym PALM (psoriasis, atopic dermatitis, lymphoproliferative disease and medication). It is important to diagnose the cause in order to get a prognosis and a targeted treatment. In 30% of the cases the cause is not evident and the patient requires periodic assessment to rule out evolution to a lymphoproliferative disease. In many cases, erythroderma is a chronic disease but it can also be a life-threatening dermatologic urgency that requires hospitalization.
La eritrodermia se caracteriza por eritema y escama en más del 90% de la superficie cutánea. Tiene muchas causas y es un reto para el dermatólogo identificarlas. Las más comunes se engloban en el acrónimo derivado de sus siglas en inglés PALM (psoriasis, dermatitis atópica, proceso linfoproliferativo y medicamentos). La importancia del diagnóstico certero es el tratamiento dirigido y el pronóstico. En los casos idiopáticos que llegan a ser de hasta el 30%, el paciente requiere de valoraciones periódicas por probable evolución a un proceso linfoproliferativo. Aunque en muchas ocasiones la eritrodermia es un cuadro crónico, también puede presentarse como una urgencia dermatológica que pone en riesgo la vida del paciente y requiere hospitalización.
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Dermatite Esfoliativa/diagnóstico , Dermatite Esfoliativa/etiologia , Doença Aguda , Adulto , Doença Crônica , Dermatite Esfoliativa/terapia , Hospitalização , Humanos , Prognóstico , Fatores de RiscoRESUMO
Fundamento: La eritrodermia ictiosiforme congénita no ampollosa es una genodermatosis que está presente desde el nacimiento y el pénfigo vegetante es una enfermedad ampollar que aparece en edades tempranas de la vida, las manifestaciones clínicas de estas entidades son diferentes, ambas son infrecuentes cuando se presentan aisladamente, mucho más cuando aparecen en un mismo paciente. Objetivo: Describir el caso de un paciente con eritrodermia ictiosiforme congénita no ampollosa y pénfigo vegetante por ser una situación clínica rara por su incidencia. Presentación de caso: Paciente de 28 años, con antecedentes de padecer de eritrodermia ictiosiforme congénita no ampollosa, que comenzó a desarrollar ampollas y al romperse dejaban erosiones que más tarde dieron lugar a vegetaciones hipertróficas y papilomatosas. Para arribar al diagnóstico se tuvo en cuenta el criterio clínico y la histopatología. Conclusiones: La eritrodermia ictiosiforme congénita no ampollosa y el pénfigo vegetante son entidades infrecuentes en la práctica médica, mucho más cuando se presenten ambas en un mismo paciente.
Background: The non-bullous congenital ichthyosiform erythroderma is a genodermatosis that is present from the birth and the vegetating pemphigus is a bullous disease that occurs at early ages of life, the clinical manifestations of these entities are different, both are uncommon when they are presented in isolation, much more when they appear in a patient Objective: To describe the case of a patient with a non-bullous congenital ichthyosiform erythroderma and vegetating pemphigus a strange clinical situation for their incidence. Case presentation: Patient of 28 years, with antecedents of suffering of non-bullous congenital ichthyosiform erythroderma that began to develop blisters and when breaking they left erosions that later gave place to hypertrophic and papillomatous vegetation. To arrive to the diagnosis was kept in mind the clinical approach and the histopathology. Conclusions: The non-bullous congenital ichthyosiform erythroderma and the vegetating pemphigus are uncommon entities in the medical practice, much more when both are presented in a patient.
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Ictiose Lamelar/diagnóstico , Penfigoide Bolhoso/diagnósticoRESUMO
INTRODUCCIÓN: La ictiosis tipo laminar es una enfermedad dermatológica infrecuente perteneciente al grupo de las llamadas genodermatosis. Es una forma de ictiosis congénita que es evidente desde el nacimiento. PRESENTACIÓN DEL CASO: Recién nacido por cesárea, sexo masculino, de 36 semanas de gestación, adecuado para la edad gestacional y con APGAR 8. Antecedentes familiares: padres no consanguíneos y hermano con ictiosis tipo laminar. Luego de nacer es hospitalizado en la Unidad de Neonatología del Hospital de San Fernando, por presentar piel de aspecto rojo brillante, engrosada en cara y parte anterior de tronco con algunas fisuras en zona torácica, sin presencia de láminas de queratina, por lo que estableció el diagnóstico clínico de ictiosis tipo laminar. Se manejó con precauciones de contacto, analgesia, lubricación de la piel y suplementación con ácido retinoico. Evolucionó con descamación y aumento delas fisuras, las que posteriormente empezaron a disminuir quedando una membrana residual y con una adecuada hidratación de piel. Durante su estadía presentó alzas febriles intermitentes por lo que se realizó un hemocultivo que fue positivo a Staphylococcus aureus y cultivos de axilas, ombligo y zona inguinal que resultaron positivos para Enterococcus y Staphylococcus aureus, iniciando tratamiento con Vancomicina. Luego de 7 días de tratamiento, evolucionó favorablemente con disminución de sus lesiones dermatológicas por lo que se dio alta médica. DISCUSIÓN: El diagnóstico oportuno en base al cuadro clínico y manejo adecuado de este paciente ha permitido una adecuada evolución en ausencia de complicaciones.
INTRODUCTION: Lamellar Ichthyosis is a rare skin diseases belonging to the Group of the so-called genodermatoses. It is a form of congenital ichthyosis evident at birth. CASE REPORT: Male neonate, born at 36 weeks of gestation via cesarian section, appropriate for gestational age and Apgar Score 8. Nonconsanguineous parents. Affected brother with Ichthyosis lamellar. Is hospitalized in the Neonatal Intermediary Care Unit of the Hospital of San Fernando due to presence of Glossy red skin, thicker in face and fissures in the chest without collodion membrane. The patient was diagnosed with Lamellar Ichthyosis. Treatment was initiated with insolations precautions, pain relievers and lubrication of the skin, as well as retinoic acid supplementation. Progressed with cracked skin and scaling that subsequently improves leaving a residual membrane and an adequate skin hydration. During his stay, he also presented intermittent fever. Blood culture was positive for Staphylococcus aureus. Skin cultures of Armpits, navel and groin were positive for Enterococcusand Staphylococcus aureus, so treatment with vancomycin was started. After 7 days of antibiotic treatment and a favourable evolution with evident improvement of his skin lesions, the patient was discharged from hospital for outpatient management. DISCUSSION: Early diagnosis based on clinical presentation and an appropriate management of this patient, allowed an adequate evolution in the absence of complications.
Assuntos
Humanos , Masculino , Recém-Nascido , Ictiose Lamelar/diagnóstico , Ictiose Lamelar/terapia , Ictiose Lamelar/classificaçãoRESUMO
Eritrodermia esfoliativa é uma entidade incomum com prognóstico reservado, caracterizada por lesões cutâneas eritematosas e esfoliativas generalizadas, que acometem mais de 80% da superfície corporal. As condições predisponentes em adultos incluem psoríase, eczema (atópico, seborréico ou de contato), efeitos adversos a drogas, ictiose, linfoma cutâneo de células T, pitiríase rubra pilar, sarna norueguesa e síndrome de Sézary. Nosso objetivo é relatar um caso de eritrodermia esfoliativa com dados clínicos e histopatológicos conclusivos, enfatizando dificuldades diagnósticas. Relatamos um homem de 68 anos apresentando eritrodermia esfoliativa. Havia antecedente de alcoolismo, hipertensão arterial e psoríase em placas; e ele havia reduzido rapidamente a dose diária de corticosteroide oral. O tratamento do paciente incluiu prednisona, metotrexato, hidroxizina, loratadina, furosemida e diltiazem, além de cuidados locais e suporte nutricional. Após um período de melhora clínica, ele teve recorrência de eritrodermia esfoliativa e faleceu em virtude de septicemia bacteriana por Pseudomonas aeruginosa resistente a carbapenêmicos. Por causa de frequentes dificuldades diagnósticas envolvendo essa entidade clínica incomum e grave, as principais características, condições predisponentes e fatores precipitantes devem ser ressaltados para aumentar o índice de suspeita, contribuindo para o diagnóstico precoce.
Exfoliative erythroderma is an uncommon entity with guarded prognosis. It is characterized by generalized erythematous and exfoliative skin lesions, which involve over 80% of the body surface. The predisposing factors in adults include psoriasis, eczema (atopic, seborrheic, or contact), drug adverse effects, ichthyosis, cutaneous T-cell lymphoma, pityriasis rubra pilaris, Norwegian scabies, and Sézary syndrome. Our aim is to report a case study with conclusive clinical and histopathological data, emphasizing diagnosis challenges. We report the case of a 68-year-old man presenting with exfoliative erythroderma. He had a history of alcoholism, arterial hypertension, and plaque psoriasis, and he had rapidly reduced his daily oral corticoid dosage. The patients management included prednisone, methotrexate, hydroxyzine, loratadine, furosemide, and diltiazem, in addition to topical care and nutritional support. After a period of clinical improvement, he had a recurrence of exfoliative erythroderma and died due to bacterial sepsis. The infectious agent that caused sepsis was a carbapenem-resistant strain of Pseudomonas aeruginosa. Because of frequent diagnosis challenges involving this uncommon and severe clinical entity, main features, predisposing factors, and precipitating factors should be highlighted to enhance the index of suspicion, which contributes to early diagnosis.
RESUMO
Las euforbiáceas son una familia de plantas que tienen en común la producción de látex. Existen más de 8000 especies.Tienen amplia distribución mundial, y adoptan la forma de árboles, arbustos o hierbas. Pertenecen a esta familia numerosas especies de importancia y, en nuestro país, se emplean con fines industriales y medicinales. También existen plantas ornamentales, cuya atractiva apariencia estimula la curiosidad de los niños, quienes pueden ingerirlas o manipularlas.En el Centro Nacional de Intoxicaciones, las consultas por plantas representan un escaso número (0,2 por ciento) dentro del total de accidentes en pediatría; sin embargo, pueden revestir gravedad.Presentamos el caso de una niña de 4 años, quien manipuló e ingirió parte de una planta de la familia Euforbiáceas(Synadenium grantii).
The Euphorbiaceae family are plants that have in common latex production. There are over 8000 species. They have worldwide distribution. We can find trees, shrubs or herbs. Many importantspecies belong to this family and they are used in our country for industrial and medical purposes. There are alsoornamental plants with attractive appearence that stimulate children curiosity, intake or manipulation.In the National Poison Center consultations for plants represent a small number (0.2%) of the total accidents in children, however they can be serious. We report the case of a 4 years old girl who manipulated andswallowed part of an Euphorbiaceae family plant (Synadenium grantii).
Assuntos
Humanos , Feminino , Pré-Escolar , Dermatite Esfoliativa , Euphorbiaceae , Hipersensibilidade ao Látex , Plantas TóxicasRESUMO
La eritrodermia es una enfermedad inflamatoria cutánea generalizada. Generalmente se presenta en la edad media de la vida y en la mayoría de los casos es debida a exacerbación de dermatosis preexistentes. Puede asociarse a manifestaciones sistémicas como poliadenopatías, hepatomegalia, eritrosedimentación acelerada, leucocitosis y/o eosinofilia, entre otros. Con el fin de conocer la incidencia y la etiología de esta patología en nuestro hospital realizamos un estudio retrospectivo y observacional sobre 3160 historias clínicas de pacientes internados en el servicio de clínica médica. Detectamos 25 casos de eritrodermia lo cual implica una incidencia del 0.79%. La edad media de presentación fue 48 años. La etiología más frecuente fue la secundaria a medicamentos, siendo los β lactámicos los más involucrados. La alteración sistémica mayormente observada fue la eosinofilia (62%). El comienzo de la enfermedad en la mayoría de los casos fue agudo.
Erythroderma is an inflammatory and generalized skin disorder (more than 90% of the body surface area) accompanied by a variably degree of scaling. Often affects middle ages of life and it is usually caused by the exacerbation of a preexisting dermatoses. It could be associated to systemic manifestations like lymphadenopathy, liver enlargement, leukocytosis and elevated erythrocyte sedimentation. The aim of this study was to determinate the incidence and also the etiology of this pathology in our environment. We made a retrospective and observable study based on the research of 3160 clinic stories of patients that were in the hospital at the Clinical Department. We found that 25 patients were diagnosed with erithroderma (incidence 0.79%). The mean age at diagnosis was 48 years. The most common causative factor was drug reactions been the β lactams the most involved drug. Eosinophilia was the most important systemic manifestation (62%). The onset of erithroderma was usually abrupt and florid.