RESUMO
BACKGROUND: Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are life-threatening conditions that may present with similar findings to other severe dermatologic diseases. OBJECTIVE: The primary objective of this exploratory study was to explore factors associated with SJS/TEN and develop a model that provides the predicted probability of SJS/TEN for patients for whom the diagnosis of SJS/TEN is considered. METHODS: Retrospective review of consultations for patients with suspected SJS, TEN, or overlap at 4 academic dermatology consultation services. RESULTS: Overall, 208 patients were included; 59 (28.4%) had a final diagnosis of SJS/TEN, and 149 (71.6%) were given a different diagnosis. The most common mimickers were drug hypersensitivity syndrome (n = 21, 10.1%), morbilliform drug eruption (n = 18, 8.7%), erythema multiforme (n = 15, 7.2%), and acute generalized exanthematous pustulosis (n = 13, 6.2%). Nikolsky sign, atypical targets, fever, and lymphopenia were included in a model for predicting the probability of SJS/TEN. LIMITATIONS: All cases were obtained from academic centers, which may limit the generalization of findings to community-based settings. This was an exploratory study with a small number of cases, and external validation of the model performance is needed. CONCLUSION: Early dermatologic evaluation of patients with suspected SJS/TEN is key to separating patients with this condition from those who ultimately receive diagnoses of other serious skin diseases.
Assuntos
Modelos Biológicos , Encaminhamento e Consulta , Síndrome de Stevens-Johnson/diagnóstico , Adulto , Idoso , Diagnóstico Diferencial , Feminino , Hospitalização , Humanos , Masculino , Pessoa de Meia-Idade , Prognóstico , Estudos Retrospectivos , Medição de Risco/métodosRESUMO
BACKGROUND: Few reports have described vitiligo developing in patients with cutaneous T-cell lymphoma (CTCL). OBJECTIVE: We sought to identify possible factors that might predispose patients with CTCL to vitiligo. METHODS: Patient demographics, CTCL disease characteristics and treatments were analyzed in 25 patients with CTCL who developed vitiligo. Cox proportional hazards modeling was used to identify associations of risk factors with the development of vitiligo. RESULTS: Younger age, later CTCL disease stage (stages IIB-IV) and presence of a CD8+CD4- mycosis fungoides phenotype were associated with the development of vitiligo. After adjusting for disease stage, increased risk of vitiligo was associated with methotrexate and CD4 antibody therapies (although the total number of patients with these was small), while decreased risk was associated with nitrogen mustard and PUVA therapies. CONCLUSIONS: No single feature was common to all of our patients, suggesting that multiple factors may contribute to the development of vitiligo in a patient-specific fashion.
Assuntos
Melanócitos/patologia , Micose Fungoide/complicações , Vitiligo/etiologia , Adolescente , Adulto , Fatores Etários , Idoso , Idoso de 80 Anos ou mais , Antimetabólitos Antineoplásicos/uso terapêutico , Antineoplásicos Alquilantes/uso terapêutico , Antígenos CD4/análise , Antígenos CD8/análise , Feminino , Humanos , Masculino , Mecloretamina/uso terapêutico , Metotrexato/uso terapêutico , Pessoa de Meia-Idade , Micose Fungoide/patologia , Micose Fungoide/terapia , Estadiamento de Neoplasias , Terapia PUVA , Fatores de Risco , Linfócitos T/química , Vitiligo/epidemiologiaRESUMO
We report 3 unusual cases of atypical exophytic cutaneous herpes simplex virus (HSV) type 2 with concurrent cytomegalovirus (CMV) infection in immunosuppressed patients and raise awareness to the significant clinical and pathologic challenges in establishing the correct diagnosis. In all the 3 cases, the lesions presented as fungating plaques and nodules with areas of superficial erosion. Initial clinical differential included genital warts, syphilis, versus cutaneous malignancy. All the 3 patients were referred to the dermatology clinic where a combination of cutaneous biopsies, viral cultures of the lesions, polymerase chain reaction, CMV antigenemia, and immunoperoxidase stains for CMV and HSV confirmed the diagnosis of HSV type 2 with concurrent CMV infection. All the 3 patients were treated with oral valganciclovir with significant improvement noted at the follow-up visit. In addition, we review the previously reported HSV/CMV cutaneous coinfection cases.
Assuntos
Coinfecção , Infecções por Citomegalovirus/diagnóstico , Herpes Simples/diagnóstico , Herpesvirus Humano 2/isolamento & purificação , Hospedeiro Imunocomprometido , Pele/virologia , Administração Oral , Adulto , Antivirais/uso terapêutico , Biópsia , Infecções por Citomegalovirus/complicações , Infecções por Citomegalovirus/tratamento farmacológico , Infecções por Citomegalovirus/imunologia , Diagnóstico Diferencial , Feminino , Herpes Simples/complicações , Herpes Simples/tratamento farmacológico , Herpes Simples/imunologia , Herpes Simples/virologia , Humanos , Masculino , Pessoa de Meia-Idade , Valor Preditivo dos Testes , Pele/imunologia , Pele/patologia , Resultado do TratamentoRESUMO
We present the case of an infant with presumed Stevens-Johnson syndrome. Through a history, physical, and histopathology, we were able to diagnose the patient with kwashiorkor. Physicians should be aware of this disorder, which is commonly thought of as a developing world problem, because it is increasing in incidence in industrialized nations because of changing dietary habits.
Assuntos
Vesícula/diagnóstico , Países Desenvolvidos , Transtornos da Nutrição do Lactente/diagnóstico , Kwashiorkor/diagnóstico , Síndrome de Stevens-Johnson/diagnóstico , Doença Aguda , Vesícula/dietoterapia , Vesícula/etiologia , Diagnóstico Diferencial , Feminino , Humanos , Lactente , Fórmulas Infantis , Transtornos da Nutrição do Lactente/dietoterapia , Transtornos da Nutrição do Lactente/etiologia , Pacientes Internados , Kwashiorkor/dietoterapia , Kwashiorkor/etiologiaRESUMO
BACKGROUND: Although patients with mycosis fungoides (MF) typically experience an indolent disease course, a minority undergo a process of large-cell transformation (LCT), which often heralds more aggressive disease and shortened survival. Regrettably, most dermatologists are unfamiliar with LCT, and even fewer understand how to recognize it clinically. Because a diagnosis of LCT typically triggers more aggressive therapy and/or referral to cutaneous T-cell lymphoma (CTCL) centers, it is paramount for clinicians to be able to recognize suspect lesions visually. OBJECTIVE: LCT is diagnosed histologically; however, diagnostic biopsy is performed only if transformed lesions are suspected clinically. Because the literature provides little information on what clinical features should lead to suspicion of LCT, we sought to identify and categorize the presentations of LCT to aid in its recognition. METHODS: We identified 14 patients with biopsy-proven LCT confirmed by a board-certified dermatopathologist experienced with this diagnosis. The clinical presentations of LCT, timing of its evolution, and treatment regimens were evaluated by chart and photograph review. RESULTS: We devised 3 categories that clinically represent LCT: (1) LCT occurring as a new, solitary nodule within a classic MF patch or plaque, (2) LCT occurring as abrupt onset of multiple scattered papules and/or nodules without spontaneous resolution, and (3) LCT occurring within new or enlarging tumors. LIMITATIONS: A larger number of reviewed cases might reveal additional clinical presentations of LCT. CONCLUSIONS: Dermatologists may use our categories of clinical indicators to recognize and diagnose LCT earlier, allowing implementation of more aggressive treatment regimens when appropriate.
Assuntos
Transformação Celular Neoplásica/patologia , Micose Fungoide/classificação , Micose Fungoide/patologia , Neoplasias Cutâneas/classificação , Neoplasias Cutâneas/patologia , Adulto , Idoso , Biópsia , Diagnóstico Diferencial , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Micose Fungoide/mortalidade , Estadiamento de Neoplasias/métodos , Estadiamento de Neoplasias/mortalidade , Prognóstico , Fatores de Risco , Pele/patologia , Neoplasias Cutâneas/mortalidade , Úlcera Cutânea/patologiaRESUMO
DRESS (drug rash with eosinophilia and systemic symptoms), also known as drug-induced hypersensitivity syndrome, is a severe, systemic drug reaction most commonly associated with aromatic anticonvulsants and sulfonamides. Patients typically present with fever, facial edema, cervical lymphadenopathy and a morbilliform eruption, which may progress to erythroderma. Hematologic abnormalities are a hallmark of the condition, including eosinophilia and atypical lymphocytosis. Visceral organ involvement typically manifests as hepatic dysfunction but may include lymphadenopathy, nephritis, interstitial pneumonitis, and myocarditis. Five to ten percent of patients with DRESS die from systemic complications, making timely recognition and treatment essential to prevent life-threatening manifestations. Myocarditis is a fatal and under-recognized manifestation of DRESS, which may occur long after the initial diagnosis. We review the literature of previously reported cases of DRESS and myocardial involvement, highlighting the presenting symptoms associated with cardiac involvement, treatments used, and the outcome for each patient. In addition, we offer an algorithm for early diagnosis, treatment, and subsequent monitoring of these patients.
Assuntos
Toxidermias/complicações , Miocardite/etiologia , Algoritmos , Toxidermias/imunologia , Eosinofilia/complicações , Humanos , Miocardite/diagnóstico , Miocardite/terapiaRESUMO
Nonmalignant cutaneous findings associated with the use of vemurafenib have only recently been described in the literature. Patients receiving vemurafenib have exhibited cutaneous reactions including prominent follicular plugging, hand-foot skin reaction, exuberant seborrheic dermatitis-like hyperkeratosis of the face, keratosis pilaris, and diffuse spiny follicular hyperkeratosis. Many of these nonmalignant cutaneous findings are associated with abnormal follicular keratinization thought to be secondary to abnormal signaling of the mitogen-activated protein kinase pathway that occurs with the use of BRAF inhibitors. Whether underlying Ras mutations affect this abnormal signaling as in malignant lesions is still unknown. Different therapeutic options exist for these patients that may result in significant improvement in some of these nonmalignant cutaneous findings. Conservative treatment should focus on topical therapies such as topical retinoids or topical steroids. However, systemic therapies such as concomitant oral retinoids or MEK inhibitors should be considered for more severe or refractory cutaneous findings.
Assuntos
Toxidermias/etiologia , Indóis/efeitos adversos , Melanoma/tratamento farmacológico , Melanoma/secundário , Neoplasias Cutâneas/tratamento farmacológico , Neoplasias Cutâneas/patologia , Sulfonamidas/efeitos adversos , Toxidermias/terapia , Humanos , Indóis/uso terapêutico , Sulfonamidas/uso terapêutico , VemurafenibRESUMO
Approaching the hospitalized patient with skin disease can be daunting. This article focuses on a practical approach to the patient with targetoid lesions. The discussion focuses on differentiating erythema multiforme from Stevens-Johnson syndrome and toxic epidermal necrolysis. In addition, the article offers a concise review of the broader differential diagnosis of targetoid lesions including ecthyma gangrenosum, fixed drug eruption, erythema multiforme-like drug reaction, vasculitis, acute hemorrhagic edema of infancy, erythema chronicum migrans, connective tissue diseases, and blistering diseases.
Assuntos
Eritema Multiforme/diagnóstico , Hospitais , Pacientes Internados , Síndrome de Stevens-Johnson/diagnóstico , Algoritmos , Dermatologia , Diagnóstico Diferencial , Eritema Multiforme/terapia , Humanos , Valor Preditivo dos Testes , Encaminhamento e Consulta , Pele/patologia , Síndrome de Stevens-Johnson/terapiaRESUMO
Scurvy is a well-known disease of vitamin C deficiency that still occurs in industrialized countries. The clinical manifestations of follicular hyperkeratosis, perifollicular petechiae, corkscrew hairs, and easy bruising are due to defective collagen synthesis and can be mistaken for small vessel vasculitis. Populations at risk for development of scurvy include elderly patients, alcohol and drug users, individuals who follow restrictive diets or have eating disorders, patients with malabsorption, and individuals with mental illness. We report an acute case of scurvy presenting in the inpatient/hospital setting with clinical findings initially thought to represent vasculitis. A high index of suspicion for scurvy must be kept in the appropriate clinical context, and a thorough medical history and physical examination are vital to make the diagnosis.
Assuntos
Ácido Ascórbico/sangue , Escorbuto/diagnóstico , Vasculite/diagnóstico , Doença Aguda , Humanos , Masculino , Pessoa de Meia-Idade , Escorbuto/fisiopatologiaAssuntos
Transformação Celular Neoplásica/patologia , Linfoma Anaplásico de Células Grandes/patologia , Micose Fungoide/patologia , Neoplasias Cutâneas/patologia , Biópsia por Agulha , Consenso , Diagnóstico Diferencial , Feminino , Humanos , Imuno-Histoquímica , Linfoma Anaplásico de Células Grandes/fisiopatologia , Linfoma Anaplásico de Células Grandes/terapia , Linfoma Cutâneo de Células T/patologia , Linfoma Cutâneo de Células T/fisiopatologia , Linfoma Cutâneo de Células T/terapia , Masculino , Micose Fungoide/fisiopatologia , Micose Fungoide/terapia , Prognóstico , Medição de Risco , Neoplasias Cutâneas/fisiopatologia , Neoplasias Cutâneas/terapia , Sociedades Médicas , Estados UnidosRESUMO
Cutaneous Hodgkin lymphoma is infrequent and typically occurs after extensive involvement of the lymph nodes. The condition decreased significantly in incidence in the past two decades, likely owing to the new treatment protocols composed of chemotherapy, radiotherapy and stem cell transplantation. Nevertheless, recognition of this uncommon but significant disease manifestation is important from a prognostic and therapeutic perspective. We are sharing a recent case of Hodgkin lymphoma where the primary presentation appeared as a solitary plaque on the left side of the occipital scalp, clinically suspected to represent a ruptured follicular cyst. The patient underwent excisional biopsy. Histological assessment revealed Hodgkin lymphoma affecting the skin. Radiological studies showed no regional lymphadenopathy. However, two enlarged lymph nodes were identified in the mediastinum and were positron emission tomography avid. The patient underwent systemic treatment without further histopathological examination of these two lymph nodes. Not being clear if these enlarged two lymph nodes were related to his cutaneous disease or not, we cannot be sure if the patient was afflicted either by primary cutaneous Hodgkin lymphoma or by secondary cutaneous involvement because of hematogenous spread. In either case, primary or secondary cutaneous Hodgkin disease is an extreme rarity. The literature is critically reviewed.
Assuntos
Doença de Hodgkin/patologia , Couro Cabeludo/patologia , Neoplasias Cutâneas/patologia , Idoso , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Doença de Hodgkin/metabolismo , Doença de Hodgkin/terapia , Humanos , Imuno-Histoquímica , Masculino , Couro Cabeludo/cirurgia , Neoplasias Cutâneas/metabolismo , Neoplasias Cutâneas/cirurgiaRESUMO
Importance: Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN) is a spectrum of severe mucocutaneous drug reaction associated with significant morbidity and mortality. A previously developed SJS/TEN-specific severity-of-illness model (Score of Toxic Epidermal Necrolysis [SCORTEN]) has been reported to overestimate and underestimate SJS/TEN-related in-hospital mortality in various populations. Objective: To derive a risk prediction model for in-hospital mortality among patients with SJS/TEN and to compare prognostic accuracy with the SCORTEN model in a multi-institutional cohort of patients in the United States. Design, Setting, and Participants: Data from a multicenter cohort of patients 18 years and older treated for SJS/TEN between January 1, 2000, and June 1, 2015, were obtained from inpatient consult databases and electronic medical record systems at 18 medical centers in the United States as part of the Society for Dermatology Hospitalists. A risk model was derived based on data from 370 of these patients. Model discrimination (calculated as area under the receiver operating characteristic curve [AUC]) and calibration (calculated as predicted vs observed mortality, and examined using the Hosmer-Lemeshow goodness-of-fit statistic) were assessed, and the predictive accuracy was compared with that of SCORTEN. All analysis took place between December 2016 and April 2018. Main Outcomes and Measures: In-hospital mortality. Results: Among 370 patients (mean [SD] age 49.0 [19.1] years; 195 [52.7%] women), 54 (15.14%) did not survive to hospital discharge. Five covariates, measured at the time of admission, were independent predictors of in-hospital mortality: age in years (odds ratio [OR], 1.05; 95% CI, 1.02-1.07), body surface area (BSA) in percentage of epidermal detachment (OR, 1.02; 95% CI, 1.01-1.04), serum bicarbonate level below 20 mmol/L (OR, 2.90; 95% CI, 1.43-5.88), active cancer (OR, 4.40; 95% CI, 1.82-10.61), and dialysis prior to admission (OR, 15.94; 95% CI, 3.38-66.30). A severity-of-illness score was calculated by taking the sum of 1 point each for age 50 years or older, epidermal detachment greater than 10% of BSA, and serum bicarbonate level below 20 mmol/L; 2 points for the presence of active cancer; and 3 points for dialysis prior to admission. The score was named ABCD-10 (age, bicarbonate, cancer, dialysis, 10% BSA). The ABCD-10 model showed good discrimination (AUC, 0.816; 95% CI, 0.759-0.872) and calibration (Hosmer-Lemeshow goodness of fit test, P = .30). For SCORTEN, on admission, the AUC was 0.827 (95% CI, 0.774-0.879) and was not significantly different from that of the ABCD-10 model (P = .72). Conclusions and Relevance: In this cohort of patients with SJS/TEN, ABCD-10 accurately predicted in-hospital mortality, with discrimination that was not significantly different from SCORTEN. Additional research is needed to validate ABCD-10 in other populations. Future use of a new mortality prediction model may provide improved prognostic information for contemporary patients, including those enrolled in observational studies and therapeutic trials.
Assuntos
Mortalidade Hospitalar , Modelos Teóricos , Síndrome de Stevens-Johnson/mortalidade , Adulto , Idoso , Estudos de Coortes , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Prognóstico , Fatores de Risco , Índice de Gravidade de Doença , Síndrome de Stevens-Johnson/fisiopatologia , Estados UnidosRESUMO
Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN) is a rare, severe mucocutaneous reaction with few large cohorts reported. This multicenter retrospective study included patients with SJS/TEN seen by inpatient consultative dermatologists at 18 academic medical centers in the United States. A total of 377 adult patients with SJS/TEN between January 1, 2000 and June 1, 2015 were entered, including 260 of 377 (69%) from 2010 onward. The most frequent cause of SJS/TEN was medication reaction in 338 of 377 (89.7%), most often to trimethoprim/sulfamethoxazole (89/338; 26.3%). Most patients were managed in an intensive care (100/368; 27.2%) or burn unit (151/368; 41.0%). Most received pharmacologic therapy (266/376; 70.7%) versus supportive care alone (110/376; 29.3%)-typically corticosteroids (113/266; 42.5%), intravenous immunoglobulin (94/266; 35.3%), or both therapies (54/266; 20.3%). Based on day 1 SCORTEN predicted mortality, approximately 78 in-hospital deaths were expected (77.7/368; 21%), but the observed mortality of 54 patients (54/368; 14.7%) was significantly lower (standardized mortality ratio = 0.70; 95% confidence interval = 0.58-0.79). Stratified by therapy received, the standardized mortality ratio was lowest among those receiving both steroids and intravenous immunoglobulin (standardized mortality ratio = 0.52; 95% confidence interval 0.21-0.79). This large cohort provides contemporary information regarding US patients with SJS/TEN. Mortality, although substantial, was significantly lower than predicted. Although the precise role of pharmacotherapy remains unclear, co-administration of corticosteroids and intravenous immunoglobulin, among other therapies, may warrant further study.
Assuntos
Corticosteroides/uso terapêutico , Imunoglobulinas Intravenosas/uso terapêutico , Síndrome de Stevens-Johnson/epidemiologia , Sulfametoxazol/efeitos adversos , Trimetoprima/efeitos adversos , Adulto , Idoso , Estudos de Coortes , Cuidados Críticos , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Síndrome de Stevens-Johnson/tratamento farmacológico , Síndrome de Stevens-Johnson/mortalidade , Análise de Sobrevida , Estados Unidos/epidemiologiaAssuntos
Transplante de Medula Óssea , Mucinose Folicular/etiologia , Micose Fungoide/complicações , Adulto , Anticarcinógenos/administração & dosagem , Bexaroteno , Evolução Fatal , Feminino , Humanos , Leucemia Mieloide Aguda/cirurgia , Micose Fungoide/patologia , Tetra-Hidronaftalenos/administração & dosagemRESUMO
The emergency department presents difficult cases for the consulting dermatologist. One of the most challenging is the patient who presents with fever and an erythematous rash. The differential diagnosis of the erythrodermic patient in the emergency room can include drug eruptions (DRESS syndrome), Staphylococcal scalded skin syndrome, toxic shock syndrome, Kawasaki disease and Kawasaki disease-like Syndrome, and recalcitrant erythematous desquamating disorder. Evaluating these patients can be frustrating and requires patience and thoroughness to arrive at the correct diagnosis.
Assuntos
Infecções Oportunistas Relacionadas com a AIDS/diagnóstico , Dermatite Esfoliativa , Febre/etiologia , Imunoglobulinas Intravenosas/uso terapêutico , Síndrome de Linfonodos Mucocutâneos , Infecções Oportunistas Relacionadas com a AIDS/imunologia , Adulto , Dermatite Esfoliativa/diagnóstico , Dermatite Esfoliativa/etiologia , Dermatite Esfoliativa/terapia , Diagnóstico Diferencial , Serviço Hospitalar de Emergência , Humanos , Masculino , Síndrome de Linfonodos Mucocutâneos/diagnóstico , Síndrome de Linfonodos Mucocutâneos/terapia , Síndrome da Pele Escaldada Estafilocócica/diagnóstico , Síndrome de Stevens-Johnson/diagnóstico , SíndromeAssuntos
Neoplasias da Mama/complicações , Carcinoma Ductal de Mama/complicações , Dermatomiosite/etiologia , Miastenia Gravis/etiologia , Síndromes Paraneoplásicas/etiologia , Idoso , Neoplasias da Mama/diagnóstico , Carcinoma Ductal de Mama/diagnóstico , Dermatomiosite/diagnóstico , Feminino , Humanos , Miastenia Gravis/diagnóstico , Síndromes Paraneoplásicas/imunologia , Síndromes Paraneoplásicas do Sistema Nervoso/diagnóstico , Síndromes Paraneoplásicas do Sistema Nervoso/etiologiaRESUMO
Primary cutaneous CD30⺠lymphoproliferative disorders (LPDs) account for approximately 25% of cutaneous lymphomas. Although these LPDs are clinically heterogeneous, they can be indistinguishable histologically. Lymphomatoid papulosis rarely requires systemic treatment; however, multifocal primary cutaneous anaplastic large cell cutaneous lymphoma and large cell transformation of mycosis fungoides are typically treated systemically. As CD30⺠LPDs are rare, there is little published evidence to support a specific treatment algorithm. Most studies are case reports, small case series, or retrospective reviews. This article discusses various treatment choices for each of the CD30⺠disorders and offers practical pearls to aid in choosing an appropriate regimen.