Safety in Dermatologic Procedures: Anaphylaxis, Vasovagal Reaction, and Hyperventilation. / Seguridad en procedimientos dermatológicos: anafilaxia, reacción vasovagal e hiperventilación.

This article, part of a the series on safety in dermatologic procedures, covers the diagnosis, prevention, management, and treatment of 3 situations or conditions. The first condition we address is anaphylaxis, an uncommon but severe and potentially fatal reaction that must be recognized quickly so that urgent management coordinated with an anesthesiologist can commence. The second is the vasovagal reaction, which is the most common complication in dermatologic surgery. This event, which occurs in 1 out of every 160 procedures, usually follows a benign course and resolves on its own. However, in patients susceptible to vasovagal reactions, syncope may lead to asystole and cardiac arrest. The third is acute hyperventilation syndrome, which is an anomalous anxiety-related increase in breathing rate beyond metabolic requirements. Brief practical recommendations for managing all 3 events are included.

Urticaria multiforme, una entidad infradiagnosticada / Urticaria multiforme, a cutaneous disorder infradiagnosticated

La urticaria multiforme es un trastorno cutáneo, propio de lactantes y niños pequeños, caracterizado por lesiones habonosas de centro violáceo y morfología anular. Presenta similitud con el eritema multiforme, pero a diferencia de este, tiene un carácter benigno y autolimitado. Su etiología es desconocida y el tratamiento, sintomático. Su conocimiento y diagnóstico diferencial es importante para evitar pruebas diagnósticas innecesarias, realizar un manejo correcto y proporcionar información adecuada a los padres

Urticaria multiforme is a cutaneous disorder, which mainly affects infants and young children. It seems like erythema multiforme, but it has a benign and self-limiting course. Its etiology is unknown and treatment, symptomatic. Their knowledge and differential diagnosis is important to avoid unnecessary diagnostic procedures, correct management and provide adequate information to parents

Micosis fungoide hipocrómica en la infancia / Hypopigmented mycosis fungoides in childhood

Describimos una niña de 9 años con hepatopatía crónica por atresia de vías biliares congénita. Mostró a los 4 años de edad lesiones maculosas hipocrómicasen glúteos y extremidades inferiores, algunas de ellas con componente purpúrico. Fue biopsiada a los 7 y 8 años de edad con estudio inmunocitoquímicoque confirmó micosis fungoide hipocrómica. Se discute la micosis fungoide en la infancia, su pronóstico y tratamiento. Concluimos,que ante lesiones hipopigmentadas refractarias al tratamiento, conviene realizar biopsia cutánea para descartar micosis fungoide hipocrómica

A 9 year old girl with chronic hepatic disease by congenital byliary atresia is reported. When she was 4 years old presented a hypopigmented macularlesions in the buttocks and lower limbs, some of them with purpuric component. Two biopsies with immunohistochemical study confirmed hypopigmentedmycosis fungoides. We discuss mycosis fungoides in childhood, its pronostic and treatment. We conclude, that hypopigmented lesions refractaryto treatment should always undergo biopsy to rule hypopigmented mycosis fungoides

[Familial pemphigus vulgaris: immunogenetic study of HLA class II antigens]. / Pénfigo vulgar familiar: estudio inmunogenético de los antígenos HLA clase II.

INTRODUCTION: Pemphigus vulgaris (PV) is a rare autoimmune bullous disease that affects the skin and mucosae, characterized by the presence of antibodies against desmoglein 3, that causes acantholisis and formation of intraepidermal blisters. Observation of PV cases in several members of the same family suggests the existence of genetic factors that contribute to susceptibility to suffer the disease. However, very few cases of familial PV have been described. Based on its autoimmune nature, many studies have found an association between PV and the HLA class II allele, specifically with the HLA-DRB1*0402 DQB1*0302 and HLA-DRB1*1401 DQB1*0503 haplotypes that bestows a significant risk of disease. OBJECTIVES: Study of three families with PV. PATIENTS AND METHODS: In this study, we present three families, with a total of 7 patients, diagnosed of familial PV. HLA antigens were determined with the PCR (polymerase chain reaction) technique in several members of these families. RESULTS: All the subjects affected were positive for HLA DR4 and HLA DR14. The fact that different families with PV are associated with identical haplotypes and that healthy siblings of the patients have the same haplotype is of special interest. CONCLUSION: These results support the concept of genetic predisposition in this rare disease.

Pénfigo vulgar familiar: estudio inmunogenético de los antígenos HLA clase II / Familial pemphigus vulgaris: immunogenetic study of HLA class II antigens

Introducción. El pénfigo vulgar (PV) es una rara enfermedad ampollosa autoinmune que afecta a la piel y a las mucosas, caracterizada por la presencia de autoanticuerpos dirigidos contra la desmogleína 3, causando acantolisis y formación de ampollas intraepidérmicas. La observación de casos de PV en varios miembros de una familia sugiere la existencia de factores genéticos que contribuyen a una susceptibilidad a padecer la enfermedad; sin embargo, son muy pocos los casos descritos de PV familiar. Basándose en su naturaleza autoinmune, numerosos estudios han determinado una asociación entre el PV y los alelos antígenos de histocompatibilidad (HLA) clase II; en concreto con los haplotipos HLA-DRB1*0402 DQB1*0302 y HLA-DRB1* 1401 DQB1*0503 que confieren un riesgo significativo de enfermedad. Objetivos. Estudio de tres familias con PV. Pacientes y métodos. En este estudio presentamos tres familias diagnosticadas de PV familiar, con 7 pacientes. Determinamos los antígenos HLA mediante la técnica de PCR (reacción en cadena de la polimerasa) en varios miembros de estas familias. Resultados. Todos los individuos afectos fueron positivos para HLA DR4 y HLA DR14. Es de especial interés el hecho de que diferentes familias con PV se asocien con haplotipos idénticos y que hermanos sanos de los pacientes tengan el mismo haplotipo. Conclusiones. Estos resultados apoyan el concepto de la predisposición genética en esta rara enfermedad

Introduction. Pemphigus vulgaris (PV) is a rare autoimmune bullous disease that affects the skin and mucosae, characterized by the presence of antibodies against desmoglein 3, that causes acantholisis and formation of intraepidermal blisters. Observation of PV cases in several members of the same family suggests the existence of genetic factors that contribute to susceptibility to suffer the disease. However, very few cases of familial PV have been described. Based on its autoimmune nature, many studies have found an association between PV and the HLA class II allele, specifically with the HLA-DRB1*0402 DQB1*0302 and HLA-DRB1*1401 DQB1*0503 haplotypes that bestows a significant risk of disease. Objectives. Study of three families with PV. Patients and methods. In this study, we present three families, with a total of 7 patients, diagnosed of familial PV. HLA antigens were determined with the PCR (polymerase chain reaction) technique in several members of these families. Results: All the subjects affected were positive for HLA DR4 and HLA DR14. The fact that different families with PV are associated with identical haplotypes and that healthy siblings of the patients have the same haplotype is of special interest. Conclusion. These results support the concept of genetic predisposition in this rare disease

Síndrome de Stevens-Johnson/necrolisis epidérmica tóxica tratado con inmunoglobulinas intravenosas / Stevens-Johnson Syndrome/Toxic epidermal necrolysis treated with intravenous immunoglobulins

El síndrome de Stevens-Johnson (SSJ) y la necrolisis epidérmica tóxica (NET) se engloban dentro de un mismo espectro de enfermedad, pues sólo se diferencian por el grado de despegamiento cutáneo. En la NET se ve afectada más del 30 % de la superficie corporal, por lo que se convierte en un proceso grave cuya frecuencia se estima en 1,2-6 casos por millón de habitantes y año. Se describe a un varón de 75 años que sufrió un SSJ con evolución a NET, probablemente por la ingesta de extracto de ginkgo biloba. Fue tratado con inmunoglobulinas intravenosas, con dosis de 0,5 g/kg/día durante 5 días consecutivos, con evolución favorable y sin efectos secundarios relevantes. Es evidente que casos aislados no justifican el uso sistemático de este tratamiento, pero pueden contribuir a una mayor experiencia

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are part of the same disease spectrum, but are differentiated by the degree of skin detachment. In TEN, more than 30 % of the body's surface area is affected; thus, it is a serious process, whose frequency is estimated at 1.2-6 cases per million population/year. We describe the case of a 75-year-old male who suffered from SJS which evolved into TEN, probably because of the ingestion of ginkgo biloba extract. He was treated with intravenous immunoglobulins (Ig IV) at a dose of 0.5 g/kg/day for five consecutive days, with favorable progress and no significant side effects. It is evident that isolated cases do not justify the systematic use of this treatment, but they may help build up experience

Hipertricosis lanuginosa adquirida paraneoplásica / Acquired paraneoplastic hypertrichosis lanuginosa

La hipertricosis lanuginosa adquirida, un proceso paraneoplásico poco frecuente, consiste en el desarrollo rápido de pelo tipo lanugo, fundamentalmente en cara. Se describe una mujer de 50 años con hipertricosis en cara y porción superior del cuerpo de 4 meses de evolución y pérdida de peso en los últimos 6 meses, en la que se detectó la presencia de un carcinoma epidermoide de cuello uterino inoperable. La hipertricosis lanuginosa es un indicador de mal pronóstico. Se asocia a tumores de cualquier localización, pero con mayor frecuencia, pulmonares y colorrectales. Este caso es el primero asociado a carcinoma epidermoide de cuello uterino

Acquired hypertrichosis lanuginosa, an infrequent, paraneoplastic process, consists of the rapid development of lanugo-type hair, primarily on the face. We describe a 50-year-old woman with a 4-month case of hypertrichosis on the face and upper body, and weight loss over the last 6 months, in whom an inoperable squamous cell carcinoma of the cervix was discovered. Hypertrichosis lanuginosa is an indicator of a poor prognosis. It is associated with tumors in any location, but most often with lung and colorectal tumors. This is the first case associated with squamous cell carcinoma of the cervix

[Acquired paraneoplastic hypertrichosis lanuginosa]. / Hipertricosis lanuginosa adquirida paraneoplásica.

Acquired hypertrichosis lanuginosa, an infrequent, paraneoplastic process, consists of the rapid development of lanugo-type hair, primarily on the face. We describe a 50-year-old woman with a 4-month case of hypertrichosis on the face and upper body, and weight loss over the last 6 months, in whom an inoperable squamous cell carcinoma of the cervix was discovered. Hypertrichosis lanuginosa is an indicator of a poor prognosis. It is associated with tumors in any location, but most often with lung and colorectal tumors. This is the first case associated with squamous cell carcinoma of the cervix.

[Stevens-Johnson syndrome/toxic epidermal necrolysis treated with intravenous immunoglobulins]. / Síndrome de Stevens-Johnson/necrolisis epidérmica tóxica tratado con inmunoglobulinas intravenosas.

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are part of the same disease spectrum, but are differentiated by the degree of skin detachment. In TEN, more than 30 % of the body's surface area is affected; thus, it is a serious process, whose frequency is estimated at 1.2-6 cases per million population/year. We describe the case of a 75-year-old male who suffered from SJS which evolved into TEN, probably because of the ingestion of ginkgo biloba extract. He was treated with intravenous immunoglobulins (Ig IV) at a dose of 0.5 g/kg/day for five consecutive days, with favorable progress and no significant side effects. It is evident that isolated cases do not justify the systematic use of this treatment, but they may help build up experience.