ABSTRACT
Background: Stevens-Johnson Syndrome (SSJ) and Toxic Epidermal Necrolysis (NET) are infrequent and life-threatening mucocutaneous diseases, which occur predominantly as adverse drug reactions. Aim: To describe the frequency of SSJ and NET diagnoses at a national level, estimate their incidence and describe their distribution among the different regions of the country. Material and Methods: Analysis of hospital discharge databases available at the website of the Chilean Ministry of Health searching for the tenth version of the International Classification of Diseases (ICD 10) codes for SSJ or NET, between 2001 and 2015. Results: We analyzed 24,521,796 hospital discharges nationwide. SSJ caused 855 discharges, with a lethality of 2%. NET caused 128 discharges with a lethality of 16%. The global cumulative incidence was 3.87 cases per million inhabitants per year nationwide, with a trend line to increase incidence towards the regions of higher latitude. Conclusions: SSJ and NET are dermatological emergencies with high mortality. The increase in incidence towards regions at higher latitudes may suggest an association between these conditions and lower levels of vitamin D, correlated with latitude and exposure to UV radiation.
Subject(s)
Humans , Patient Discharge/statistics & numerical data , Stevens-Johnson Syndrome/epidemiology , Chile/epidemiology , Databases, Factual , Hospital Information SystemsABSTRACT
El látex o caucho natural es un producto vegetal que se obtiene de la savia de la Hevea brasiliensis. Ampliamente utilizado para la fabricación de productos de uso doméstico (guantes, preservativos, globos, etc.) y del área de la salud, donde se estima que el 50% de los artículos médicos contienen látex. La alergia al látex es un problema de salud mundial. Se estima que la prevalencia de sensibilización al látex en población general es del 1%. Clínicamente, puede generar una reacción de hipersensibilidad tipo I, IgE mediada, en respuesta a las proteínas del látex que se manifiesta como una urticaria hasta la anafilaxia. También se describe dermatitis de contacto irritativa y la dermatitis de contacto alérgica, 12 a 96 horas luego del contacto, por una reacción de hipersensibilidad retardada tipo IV, secundaria a los aditivos del látex. El diagnóstico se basa en la sospecha clínica, confirmado con pruebas cutáneas y medición de IgEs específicas. Realizar una anamnesis que permita sospechar una alergia al látex y si se determina que el paciente tiene riesgo, realizar el estudio complementario para confirmar el diagnóstico. El enfrentamiento se basa en la prevención de la exposición y el tratamiento de la reacción aguda. Es necesario conocer los alérgenos y procesos implicados en la alergia al látex pues de este modo se podrá sospechar e identificar a pacientes o funcionarios que puedan sufrir o estar en riesgo de presentarla, disminuyendo de este modo las posibles complicaciones.
Latex or natural rubber is a vegetable product which is obtained from the sap of the Hevea brasiliensis. It is widely used to manufacture domestic products (gloves, condoms, balloons, etc.) and used in the health area, where it is estimated that 50% of medical articles contain latex. Latex allergy is a global health problem. It is estimated that the prevalence of latex sensitization is 1% in the general population. The clinical presentation can be a type I hypersensitivity reaction, IgE mediated, in response to latex proteins, manifested as a rash or even as an anaphylaxis. Irritant contact dermatitis and allergic contact dermatitis, 12 to 96 hours after contact, is also described, due to a delayed type IV hypersensitivity reaction, secondary to latex additives. The diagnosis of latex allergy is based on clinical suspicion and confirmed with skin tests measuring specific IgEs. Management is based on the exposure prevention and treatment of the acute reaction. A detailed anamnesis must be made to suspect a latex allergy and if the patient is at risk of presenting it, perform additional study to confirm the diagnosis. It is necessary to know the allergens and processes involved in latex allergy, to be able to suspect and identify patients or staff who may suffer or be at risk of presenting it, thus reducing potential complications.
Subject(s)
Humans , Latex Hypersensitivity/diagnosis , Latex Hypersensitivity/therapy , Latex/adverse effects , Skin Tests , Risk FactorsABSTRACT
La hidroxiurea es un agente citostático que inhibe la síntesis de ADN. Se considera el tratamiento de primera línea para algunos trastornos mieloproliferativos, enfermedad de células falciformes, casos severos de psoriasis refractaria y como adyuvante en la terapia de VIH. Se ha informado de que algunos pacientes tratados con hidroxiurea pueden tener úlceras en las extremidades inferiores. Paciente femenino de 67 años de edad con antecedentes de policitemia vera tratada con hidroxiurea durante un año, se deriva a dermatología por presentar úlceras bilaterales en extremidades inferiores. Al examen físico se evidencian dos lesiones ulceradas en la región calcánea. Se realiza una biopsia de piel, y muestra signos no específicos de inflamación. Se decide la interrupción de la hidroxiurea y se inicia la terapia adyuvante con pentoxifilina. Las lesiones se resolvieron en dos meses, dejando una pequeña cicatriz residual. Es importante recordar esta rara complicación inducida por el uso prolongado de la hidroxiurea y, de esta manera, realizar un diagnóstico precoz y tratamiento adecuado, que hasta el momento es básicamente la suspensión de la hidroxiurea.
Hydroxyurea is a cytostatic agent that inhibits DNA synthesis. It is considered the first line treatment for some myeloproliferative disorders, sickle cell disease, severe cases of refractory psoriasis and as adjuvant in VIH therapy. It has been reported that some patients treated with hydroxyurea may have leg ulcers. A 67 year old female patient with a history of polycythemia vera treated with hydroxyurea for a year, is derived to dermatology for presenting bilateral lower extremity ulcers. Physical examination demonstrated two ulcerated lesions in the calcaneal region. A skin biopsy is performed, and it shows non-specific signs of inflammation. Discontinuation of hydroxyurea is decided and initiate adjuvant therapy with pentoxifylline. These ulcerative lesions were resolved within two months, leaving a small residual scar. It is important to remember this rare complication induced by prolonged use of hydroxyurea and thus, early diagnosis and appropriate treatment can be made, which so far is basically the suspension of hydroxyurea.
Subject(s)
Humans , Female , Aged , Polycythemia Vera/drug therapy , Skin Ulcer/chemically induced , Drug Eruptions/diagnosis , Hydroxyurea/adverse effects , Antisickling Agents/adverse effects , Physical Examination , Biopsy , Hydroxyurea/therapeutic use , Antisickling Agents/therapeutic useABSTRACT
La telangiectasia hemorrágica hereditaria (THH) o síndrome Rendu Osler Weber es un trastorno autosómico dominante que lleva a la formación anormal de vasos sanguíneos y se manifiesta como telangiectasias y malformaciones arteriovenosas en piel y órganos internos. Mujer de 72 años con antecedentes de hipertensión arterial, anemia ferropénica e hipertensión pulmonar en tratamiento. Es derivada a dermatología por la presencia durante años de lesiones asintomáticas en cara y manos. Refiere 4 episodios de epistaxis durante su vida y no tiene historia familiar de patologías dermatológicas. Al examen se observan múltiples máculas eritemato-violáceas, con vitropresión positiva, algunas conformadas por telangiectasias, localizadas en la región malar, frente, lengua y ambas palmas. Se excluyó compromiso hepático, pero se encontraron lesiones vasculares en estómago y duodeno. Con estos antecedentes, se confirmó el diagnóstico de THH. La THH es un diagnóstico clínico basado en los criterios de Curaçao: epistaxis, telangiectasias, lesiones viscerales e historia familiar. Desde el punto de vista dermatológico, se presenta con telangiectasias en palmas, dedos, labios y lengua. Aunque la epistaxis u otras presentaciones pueden ser las manifestaciones más incapacitantes o peligrosas, las telangiectasias extranasales pueden ser más importantes para el paciente, llevándolo a consultar a dermatología. Los dermatólogos deben considerar este síndrome, a pesar de su baja incidencia reportada, debido a sus posibles complicaciones. El tratamiento es sólo paliativo, sin consenso sobre la mejor opción de manejo. Es esencial promover un control a largo plazo de la enfermedad.
Hereditary hemorrhagic telangiectasia (HHT) or Rendu Osler Weber syndrome is an autosomal dominant disorder that leads to abnormal blood vessels formation. It manifests as telangiectasias and arteriovenous malformations in the skin and internal organs. A 72-year-old female patient with previous medical history of hypertension, iron deficiency anemia, and pulmonary hypertension in treatment was referred to our clinic due to the presence of asymptomatic acral lesions. She reported only four epistaxis events throughout her life, and had no family history bleeding. Examination showed multiple, blanching, erythematous-violaceous macules. On the malar region, forehead, tongue and palms, some telangiectasias were grouped. No hepatic lesions were found, however, stomach and duodenum vascular malformations were found after workup; prompting the diagnosis of HHT . HHT diagnosis is made clinically based on the Curaçao criteria: epistaxis, telangiectasias, visceral lesions and family history. From a dermatological point of view, it is presented with telangiectases in palms, fingers, lips and tongue. However, epistaxis or other vascular malformations may be life-threatening. Dermatologists should be aware of the existence of HHT, despite its low reported incidence, due to its frequent cutaneous manifestations and potential complications. Treatment is only palliative, with no consensus on the best management option. It is essential to promote long-term control of the disease.