Fulvestrant-induced toxic epidermal necrolysis

Abstract Toxic epidermal necrolysis is a condition with massive keratinocyte apoptosis, and it is associated with high mortality rates. Fulvestrant, an estrogen receptor antagonist, is indicated in the treatment of estrogen receptor-positive metastatic breast cancer in postmenopausal women. To our knowledge, this is the first described case of toxic epidermal necrolysis due to fulvestrant. A 56-year-old woman received 500 mg of intramuscular fulvestrant monthly for metastatic ductal carcinoma of the breast. Five days after the first dose, the patient presented with a maculopapular rash that evolved to blisters, and a detachment of the epidermis in over 30% of the total body surface area. Histological analysis was compatible with toxic epidermal necrolysis. Fulvestrant was discontinued, topical management and supportive care were initiated.

Epidermal necrolysis: SCORTEN performance in AIDS and non-AIDS patients

Abstract: Background: Stevens-Johnson syndrome and toxic epidermal necrolysis are life-threatening blistering drug reactions with high incidence of ocular sequela. The term 'Epidermal Necrolysis' has been recently used to better describe the full spectrum of the disease that includes Stevens-Johnson syndrome and toxic epidermal necrolysis at opposite ends, which differ by the extent of body surface area with epidermal detachment. SCORTEN is a mortality prognosis score for 'Epidermal Necrolysis' cases that still needed validation in acquired immunodeficiency syndrome. Objective: To evaluate the SCORTEN performance in acquired immunodeficiency syndrome, and the differences in outcomes between acquired immunodeficiency syndrome and non- acquired immunodeficiency syndrome cohorts. Methods: Retrospective cohort study of AIDS and non-AIDS 'Epidermal Necrolysis' cases admitted to a Brazilian reference center from 1990-2014. Results: Five deaths (16.7%) occurred as a consequence of EN in 30 AIDS patients, and seven (17.9%) in 39 non-AIDS patients, relative risk (RR) .92 (p=1.0). SCORTEN showed great performance, with an Area Under the Receiver Operating Curve (AUC) (ROC) of 0.90 with a 95% confidence interval ranging from .81 to .99. The performance of SCORTEN was better among non- AIDS patients than AIDS patients: AUC non- acquired immunodeficiency syndrome =0.99 (CI 05% 0.96-1.00), AUC acquired immunodeficiency syndrome = 0.74 (CI 95% 0.53-0.95), p=.02. Study Limitations: Heterogeneity of cases, wide variation of systemic corticosteroid doses when used. Conclusion: SCORTEN is valid for the Brazilian population, including among those patients with acquired immunodeficiency syndrome, and, as such, its use is recommended for aiding treatment choice in this subgroup of patients.

Necrolisis epidérmica tóxica en el curso de un síndrome hemofagocítico secundario a linfoma de Hodgkin: caso clínico / Toxic epidermal necrolysis during a hemophagocytic syndrome secondary to Hodgkin lymphoma: report of one case

Toxic epidermal necrolysis (TEN) is a lethal entity, characterized by extensive epidermal necrosis and multiorgan failure. Hemophagocytic syndrome (HFS) is also a rare and lethal syndrome characterized by hyperinflammation that leads to the appearance of fever, pancytopenia, organomegaly and hemophagocytosis. The concomitance of these diseases is extremely uncommon. We report a 38 years old female, who during the course of a HFS secondary to Hodgkin Lymphoma (HL), presented a TEN secondary to antibiotics. She was admitted due to a consumptive syndrome, lymphadenopathy, visceromegaly and severe pancytopenia. Laboratory and bone marrow tests confirmed HFS. Due to constant fever, imipenem was indicated. On the third day she started with pain and skin rash. She evolved with positive Nikolsky sign. Cutaneous biopsy was concordant with extensive TEN, which was managed with intravenous immunoglobulin and dexamethasone. A complete response and normalization of the blood count were achieved. Finally, the lymph node biopsy showed HL of mixed cellularity type, which was managed with 8 cycles of ABVD chemotherapy, achieving complete remission.

Vancomycin-associated linear IgA disease mimicking toxic epidermal necrolysis

Abstract Linear IgA dermatosis is a rare subepidermal autoimmune blistering disease characterized by linear deposition of IgA along the basement membrane zone. In the last three decades, many different drugs have been associated with the drug-induced form of the disease, especially vancomycin. We report a case of vancomycin-induced linear IgA disease mimicking toxic epidermal necrolysis. The aim of this work is to emphasize the need to include this differential diagnosis in cases of epidermal detachment and to review the literature on the subject and this specific clinical presentation.

Stevens-Johnson syndrome and toxic epidermal necrolysis: a review / Síndrome de Stevens-Johnson e necrólise epidérmica tóxica: revisão

SUMMARY Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are uncommon, acute and potentially life-threatening adverse cutaneous drug reactions. These pathologies are considered a hypersensitivity reaction and can be triggered by drugs, infections and malignancies. The drugs most often involved are allopurinol, some antibiotics, including sulfonamides, anticonvulsants such as carbamazepine, and some non-steroid anti-inflammatory drugs (NSAIDs). Necrosis of keratinocytes is manifested clinically by epidermal detachment, leading to scalded skin appearance. The rash begins on the trunk with subsequent generalization, usually sparing the palmoplantar areas. Macular lesions become purplish, and epidermal detachment occurs, resulting in flaccid blisters that converge and break, resulting in extensive sloughing of necrotic skin. Nikolsky's sign is positive in perilesional skin. SJS and TEN are considered to be two ends of the spectrum of one disease, differing only by their extent of skin detachment. Management of patients with SJS or TEN requires three measures: removal of the offending drug, particularly drugs known to be high-risk; supportive measures and active interventions. Early diagnosis of the disease, recognition of the causal agent and the immediate withdrawal of the drug are the most important actions, as the course of the disease is often rapid and fatal.

RESUMO A síndrome de Stevens-Johnson (SSJ) e a necrólise epidérmica tóxica (NET) são doenças mucocutâneas pouco frequentes, agudas e potencialmente ameaçadoras à vida. Representam uma reação de hipersensibilidade e podem ser desencadeadas por fármacos, infecções e neoplasias. Dentre os principais medicamentos descritos como causadores do quadro estão o alopurinol, alguns antibióticos do grupo das sulfonamidas, anticonvulsivantes, como carbamazepina, e alguns anti-inflamatórios não esteroidais. A necrose dos queratinócitos manifesta-se clinicamente pelo descolamento epidérmico, levando a um aspecto de pele escaldada. A erupção inicia-se no tronco, com posterior generalização, geralmente poupando as áreas palmoplantares. As máculas tornam-se violáceas e há descolamento epidérmico, dando origem a bolhas flácidas, que confluem e se rompem, deixando áreas extensas erosadas. A pele perilesional apresenta sinal de Nikolsky positivo. A SSJ e a NET representam espectros da mesma doença, diferenciando-se pelo grau de descolamento epidérmico. O tratamento da SSJ e da NET é fundamentado em três medidas: retirada da droga ofensora, especialmente as medicações conhecidamente de alto risco; medidas de suporte e intervenções ativas. O diagnóstico precoce da entidade, o reconhecimento do agente causal e a retirada imediata do fármaco são as mais importantes ações, visto que a evolução dos casos é muitas vezes rápida e fatal.

Drug reaction with eosinophilia and systemic symptoms (DRESS syndrome) / Reação a drogas com eosinofilia e sintomas sistêmicos (síndrome DRESS)

SUMMARY Objective: To review the hypersensitivity reaction to drugs known as drug reaction with eosinophilia and systemic symptoms (DRESS syndrome), based on a case report. We also intend to discuss the difficulty and importance of disease recognition, since none of the changes is pathognomonic of this disease and failure to identify it may have disastrous consequences for the patient. Method: To describe this case report, in addition to the information collected for clinical assessment, a literature review was performed in the PubMed and Bireme databases in order to retrieve the latest information published in literature on DRESS syndrome. Results: The case of a 20-year old patient is reported. After anamnesis, physical examination and laboratory tests a diagnosis of DRESS syndrome was performed, characterized by rash, hematologic alterations, lymphadenopathy and lesions in target organ. This is a rare syndrome, whose frequency varies according to the drug used and the immune status of the patient, being more often associated with the use of anticonvulsants. Conclusion: The approach and discussion of the topic are of paramount importance, in view of the potential lethality of this treatable syndrome. Recognizing the occurrence of DRESS syndrome and starting treatment as soon as possible is crucial to reduce the risk of mortality and improve prognosis.

RESUMO Objetivo: fazer uma revisão da reação de hipersensibilidade a drogas denominada reação a drogas com eosinofilia e sintomas sistêmicos (síndrome DRESS), com base em um relato de caso clínico. Pretende-se ainda discutir a dificuldade e importância de seu reconhecimento, uma vez que nenhuma alteração dessa doença é patognomônica e sua não identificação pode trazer consequências desastrosas para o paciente. Método: para descrever este relato, além das informações coletadas no caso clínico, uma revisão da literatura nas bases de dados PubMed e Bireme foi realizada com o intuito de rever as informações mais recentes publicadas na literatura acerca da síndrome DRESS. Resultados: relatou-se o caso de uma paciente de 20 anos de idade que, após anamnese, exame físico e exames laboratoriais, foi diagnosticada com síndrome DRESS, caracterizada por erupção cutânea, alterações hematológicas, linfonodomegalia e lesões em órgão-alvo. É uma síndrome rara, cuja frequência varia conforme a droga utilizada e o estado imunológico do indivíduo, sendo mais associada ao uso de anticonvulsivantes. Conclusão: a abordagem e discussão do tema são de extrema importância, tendo em vista o potencial de letalidade dessa síndrome, que possui tratamento. Reconhecer precocemente a DRESS e instituir terapêutica é fundamental para reduzir o risco de mortalidade e melhorar o prognóstico.

Compensation for Occupational Skin Diseases

The Korean list of occupational skin diseases was amended in July 2013. The past list was constructed according to the causative agent and the target organ, and the items of that list had not been reviewed for a long period. The revised list was reconstructed to include diseases classified by the International Classification of Diseases (10th version). Therefore, the items of compensable occupational skin diseases in the amended list in Korea comprise contact dermatitis; chemical burns; Stevens-Johnson syndrome; tar-related skin diseases; infectious skin diseases; skin injury-induced cellulitis; and skin conditions resulting from physical factors such as heat, cold, sun exposure, and ionized radiation. This list will be more practical and convenient for physicians and workers because it follows a disease-based approach. The revised list is in accordance with the International Labor Organization list and is refined according to Korean worker's compensation and the actual occurrence of occupational skin diseases. However, this revised list does not perfectly reflect the actual status of skin diseases because of the few cases of occupational skin diseases, incomplete statistics of skin diseases, and insufficient scientific evidence. Thus, the list of occupational diseases should be modified periodically on the basis of recent evidence and statistics.

Steven Johnson Syndrome / Síndrome de Steven Johnson

Ulcerative vesiculobullous disorders are common in Dermatology and Oral Medicine. Diagnosis of these conditions is pretty effortless if they give a classic appearance as described in the literature. Steven Johnson syndrome is one such condition which is a type of erythema multiforme. It is a disorder involving mucous membrane, skin and even the multiple organs in severe form. Multiple etiologies persist so be acquainted with the cause and prohibit the root is crucial. But still drugs are considered to be common cause. Early management is vital as complications are serious for this condition even leading to death. We hereby report a typical case with classic appearance of Steven Johnson syndrome.

Doenças ulcerativas vésicobolhosas são comuns em Dermatologia e Medicina Oral. O diagnóstico destas condições é bastante fácil se apresentar uma aparência clássica, tal como descrito na literatura. Steven Johnson é um tipo de eritema multiforme. É uma desordem envolvendo as mucosas, pele e até múltiplos órgãos de forma grave. Múltiplas etiologias são apontadas como causa. Mas ainda os medicamentos são considerados como causas comuns. O tratamento precoce é vital, pois complicações são graves para essa condição, podendo levar à morte. Vimos por meio deste relatar um caso típico, com aparência clássica de Síndrome de Steven Johnson.

Síndrome de Stevens-Johnson: necrólisis epidérmica tóxica asociada a lamotrigina: presentación de un caso / Stevens-johnson syndrome: toxic epidermic necrolysis related to lamotrigina: case presentation

El síndrome de Stevens-Johnson/necrólisis epidérmica tóxica (SSJ/NET) corresponde a una forma clínica de superposición en el espectro del eritema multiforme. Es de presentación poco frecuente y pronóstico reservado. Los desencadenantes más comunes son los fármacos. Se presenta el caso de una paciente, de 50 años, que desarrolló un episodio de superposición (SSJ/NET) diez días después del agregado de lamotrigina a su tratamiento para la epilepsia iniciado con ácido valproico. La paciente fue internada en clínica médica, la relación causal para lamotrigina fue definida y el cuadro cursó una evolución favorable con la suspensión del fármaco y la instauración temprana de las medidas de sostén...

Eritema multifome - atualizações / Erythema multiforme - an update

O eritema multiforme ou polimorfo é um processo inflamatório agudo que apresenta lesões cutâneo-mucosas características. Representa uma enfermidade de interesse para Odontologia, uma vez que, por vezes, a boca pode ser a única área afetada. O objetivo deste trabalho é apresentar uma revisão da literatura destacando os aspectos relacionados à etiologia, características clínicas e histológicas, diagnóstico, tratamento e prognóstico da referida condição patológica

Lúpus eritomatoso sistêmico e síndrome de Lyell: necrólise epidérmica tóxica / Systemic lupus erythematosus and Lyell Syndrome: toxic epidermal necrosis

Os autores relatam a ocorrência da Síndrome de Lyell (SL) ou Necrólise Epidérmica Tóxica (NET) em 3 (três) pacientes portadores de Lúpus Eritomatoso Sistêmico (LES). Todos estavam em atividade da doença lúpica e apresentavam manifestaçöes sistêmicas graves. Os prováveis agentes desencadeantes foram: dipirona, trimetoprim-sulfametoxazol e cefalotina. A evoluçåo foi muito boa em um paciente, que recebeu alta sem sequelas; outra evoluiu com síndrome seca e perda da visåo; e uma terceira foi ao êxito letal, por hemorragia digestiva alta incontrolável. É feita revisåo da literatura e discutidos os mecanismos histopatológicos da NET e sua relaçåo com a Doença Enxerto Versus Hospedeiro (DEVH) e Síndrome Seca (SS). Sugere-se ainda que o LES poderia funcionar como co-fator para o desencadeamento da NET, que por sua vez seria um sinal de agressividade da doença lúpica

Caso anatomoclínico del Instituto Nacional de Cancerología / Anatomoclinic case of tha Instituto Nacional of Cancerologia

Se describe el caso de una mujer de 77 años que presentó datos cerebrales de un probable carcinoma con primario de origen desconocido. En el estadio final de su evolución clínica presentó datos de abdomen agudo. Se efectúan consideraciones acerca del manejo de las neoplasias de origen desconocido que eventualmente dan metástasis a sistema nervioso central. Así mismo se presenta una revisión bibliográfica de las diferentes extirpes histológicas que metastatizan a cerebro y se comenta acerca de su pronóstico. Finalmente, se efectúan comentarios de los hallazgos histopatológicos.

Toxic epidermal necrolysis due to nimorazole

Toxic epidermal necrolysis [TEN] first described by Lyell is usually caused by drugs. There are, however, other causes of TEN which have been enumerated. A patient is being reported for the first time in whom nimorazole was the offending drug for TEN. The drugs reported to have caused TEN have been enumerated. The subject is being reviewed briefly

Síndrome de Stevens-Johnson y embarazo / Stevens-Johnson's syndrome and pregnancy

Se presenta un caso de sindrome de Stevens-Johnson asociado a embarazo. Se afirma dicha etiología descartando, con los métodos a nuestro alcance, otras patologías que podrían provocarlo y valorizando el antecedente de haber padecido síntomas mínimos del mismo en el embarazo anterior. Se hacen consideraciones sobre la gravedad de esta patología y lo infrecuente de la misma en relación con el embarazo, tanto en el ambiente dermatològico como obstétrico.