Síndrome de BOD / BOD syndrome

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Manejo clínico de la epidermolisis ampollosa distrófica hereditaria: a propósito de un caso / Clinical handling of hereditary dystrophic epidermolysis bullosa: a case report

Las epidermolisis ampollosas son enfermedades genéticamente determinadas, caracterizadas por una fragilidad excesiva de la piel a las fuerzas de fricción. Esto ocasiona erosiones y ampollas, espontáneamente o ante mínimos traumatismos. Todas ellas se producen por alteraciones, hoy conocidas, en las proteínas de la unión dermoepidérmica. Los pacientes afectados requieren una atención adecuada para mantener una buena calidad de vida, mediante un manejo clínico muy cuidadoso, que incluya la prevención y el tratamiento oportuno de las múltiples complicaciones asociadas, algunas de las cuales pueden llegar a condicionar su supervivencia. Presentamos un caso de epidermolisis ampollosa distrófica con inicio en el nacimiento, confirmada con estudios histológicos y ultraestructurales(AU)

The epidermolysis bullosa constitute a group of determined genetically illnesses characterized by an excessive fragility of the skin to the friction forces. All of them take place for protein alterations, today known, that intervene in the union of the epidermis with the dermis. This leads to the formation of erosion and blisters, spontaneously or before a minimal traumatism. The patients suffering from this type of pathologies need a very careful clinical handling, which propitiates them the attention adapted to support a good quality of life, as well as the prevention and opportune treatment of the multiple complications that they can present, some of which can go so far as to threaten their life. We present of dystrophic epidermolysis bullosa case at birth, confirmed throughout histological and ultrastructural studies(AU)

Hipoplasia ungueal bilateral y simétrica de las manos / Bilateral Symmetrical Nail Hypoplasia of the Hands

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Pitiriasis rosada: una presentación atípica / Pityriasis rosea: an atypical presentation

En este artículo mostramos una presentación atípica de pitiriasis rosada en una paciente diagnosticada inicialmente de tiña corporis. La pitiriasis rosada es una dermatosis papuloescamosa aguda y autolimitada que afecta fundamentalmente a niños y adultos jóvenes sin predominancia de sexo. Se postula una etiología infecciosa, fundamentalmente vírica, aunque no ha podido ser confirmada. Esta entidad puede manifestarse con formas atípicas que dificultan el diagnóstico, y además es una dermatosis que se irrita con extremada facilidad variando su aspecto clínico (AU)

In this article, we present an atypical presentation of pityriasis rosea in a patient initially diagnosed of tinea corporia. Pityriasis rosea is an acute and self-limited papulosquamous dermatosis that fundamentally affects children and young adults with no gender predominance. It is hypothesized that its etiology is infectious, basically viral, although this has not been confirmed. This condition may occur in atypical forms that hinder its diagnosis and is a dermatosis that becomes irritated very easily, varying its clinical aspect (AU)

Metástasis cutáneas de neoplasias internas / Cutaneous metastases of internal neoplasm

Las metástasis cutáneas son un evento raro en dermatología, hay que tener en cuenta que pueden ser el primer marcador de la enfermedad y que asímismo pueden ser la primera señal de progresión. Generalmente las cutánides tienen la misma distribución por sexos que los primarios, siendo lascutánides más frecuentes en mujeres las procedentes de la mama, y en el hombre las procedentes de pulmón.Es importante reconocer estas lesiones para no diferir un diagnóstico correcto y por sus implicaciones con respecto al tratamiento. En aquellos pacientescon antecedentes de neoplasias internas, las metástasis cutáneas siempre hay que tenerlas dentro del diagnóstico diferencial de las lesiones cutáneas (AU)

Cutaneous metastases are infrequent in dermatology; they may be the first sign of disease and the first signal of progression. Cutaneous metastasesusually have the same distribution by sex than primary neoplasms. Breast cutaneous metastases are the most frequent in women and lung cutaneousmetastases are the most frequent in men.It is important to recognize these lesions not to defer a correct diagnosis and because their implications with regard to the treatment. In patients whohave a history of an internal neoplasm, cutaneous metastases always must be taken in mind in the differential diagnosis of cutaneous pathologies (AU)

[Allergic contact dermatitis to cosmetics]. / Dermatitis alérgica de contacto por cosméticos.

INTRODUCTION: Contact dermatitis to cosmetics is a common problem in the general population, although its prevalence appears to be underestimated. We reviewed cases of allergic contact dermatitis to cosmetics diagnosed in our dermatology department over a 7-year period with a view to identifying the allergens responsible, the frequency of occurrence of these allergens, and the cosmetic products implicated. METHODS: Using the database of the skin allergy department, we undertook a search of all cases of allergic contact dermatitis to cosmetics diagnosed in our department from January 2000 through October 2007. RESULTS: In this period, patch tests were carried out on 2,485 patients, of whom 740 were diagnosed with allergic contact dermatitis and the cause was cosmetics in 202 of these patients (170 women and 32 men), who accounted for 27.3 % of all cases. A total of 315 positive results were found for 46 different allergens. Allergens most often responsible for contact dermatitis in a cosmetics user were methylisothiazolinone (19 %), paraphenylenediamine (15.2 %), and fragrance mixtures (7.8 %). Acrylates were the most common allergens in cases of occupational disease. Half of the positive results were obtained with the standard battery of the Spanish Group for Research Into Dermatitis and Skin Allergies (GEIDAC). The cosmetic products most often implicated among cosmetics users were hair dyes (18.5 %), gels/soaps (15.7 %), and moisturizers (12.7 %). CONCLUSION: Most patients affected were women. Preservatives, paraphenylenediamine, and fragrances were the most frequently detected cosmetic allergens, in line with previous reports in the literature. Finally, in order to detect new cosmetic allergens, cooperation between physicians and cosmetics producers is needed.

Dermatitis alérgica de contacto por cosméticos / Allergic Contact Dermatitis to Cosmetics

Introducción: La dermatitis de contacto por cosméticos es un problema frecuente entre la población general, sin embargo, parece ser que su prevalencia está infraestimada. Revisamos en este trabajo los casos de dermatitis de contacto alérgica por cosméticos diagnosticados en el Departamento de Dermatología en un periodo de 7 años con el objetivo de detectar los alergenos responsables, la frecuencia de los mismos, así como los productos cosméticos implicados. Métodos: Utilizando la base de datos de la sección de Alergia Cutánea se realiza una búsqueda de todos los casos de dermatitis de contacto alérgica por cosméticos diagnosticados en nuestro departamento entre enero de 2000 y octubre de 2007. Resultados: Durante este periodo se realizaron pruebas epicutáneas a 2.485 pacientes. De todos ellos, 740 fueron diagnosticados de una dermatitis de contacto alérgica, 202 pacientes (170 mujeres/32 varones), es decir, el 27,3 % lo fueron por cosméticos. Se detectaron un total de 315 parches positivos y 46 alergenos diferentes. Los alergenos que con más frecuencia produjeron una dermatitis de contacto en el usuario fueron las metilisotiazolinonas (19 %), la parafenilendiamina (15,2 %) y la mezcla de perfumes (7,8 %). Los acrilatos fueron los alergenos más frecuentes en aquellos casos que tenían un origen laboral. Con la batería estándar del Grupo Español en Investigación en Dermatitis y Alergia Cutánea (GEIDAC) se detectaron la mitad de las pruebas positivas. Los productos cosméticos implicados con mayor frecuencia en el usuario fueron los tintes capilares (18,5 %), los geles/jabones (15,7 %) y las cremas hidratantes (12,7 %). Conclusión: La mayoría de los pacientes afectados fueron mujeres. Los conservantes, la parafenilendiamina y los perfumes fueron los alergenos cosméticos más frecuentes, tal y como había sido publicado previamente en la literatura. Finalmente, con el objetivo de detectar nuevos alergenos cosméticos debe existir colaboración entre los facultativos y las casas comerciales (AU)

Introduction: Contact dermatitis to cosmetics is a common problem in the general population, although its prevalence appears to be underestimated. We reviewed cases of allergic contact dermatitis to cosmetics diagnosed in our dermatology department over a 7-year period with a view to identifying the allergens responsible, the frequency of occurrence of these allergens, and the cosmetic products implicated. Methods: Using the database of the skin allergy department, we undertook a search of all cases of allergic contact dermatitis to cosmetics diagnosed in our department from January 2000 through October 2007. Results: In this period, patch tests were carried out on 2485 patients, of whom 740 were diagnosed with allergic contact dermatitis and the cause was cosmetics in 202 of these patients (170 women and 32 men), who accounted for 27.3% of all cases. A total of 315 positive results were found for 46 different allergens. Allergens most often responsible for contact dermatitis in a cosmetics user were methylisothiazolinone (19%), paraphenylenediamine (15.2%), and fragrance mixtures (7.8%). Acrylates were the most common allergens in cases of occupational disease. Half of the positive results were obtained with the standard battery of the Spanish Group for Research Into Dermatitis and Skin Allergies (GEIDAC). The cosmetic products most often implicated among cosmetics users were hair dyes (18.5%), gels/soaps (15.7%), and moisturizing creams (12.7%). Conclusion: Most patients affected were women. Preservatives, paraphenylenediamine, and fragrances were the most frequently detected cosmetic allergens, in line with previous reports in the literature. Finally, in order to detect new cosmetic allergens, cooperation between physicians and cosmetics producers is needed (AU)

Pápulas hiperqueratósicas en las manos / Hyperkeratotic Papules on the Hands

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[Long-term control of psoriasis is necessary]. / Es necesario el control de la psoriasis a largo plazo.

Psoriasis is an immune mediated, inflammatory skin condition affecting approximately 1.43% of Spanish population. In clinical practice, physicians use PASI index to assess the severity. Psoriasis causes physical and mental disability comparable to other chronic diseases and affects seriously the patients quality of life. For treatment we have different options. Conventional systemic treatment such as methotrexate, cyclosporine and acitretin may be associated with relevant side effects, and organ toxicity that avoid long term therapy. Several psoriasis patients have other comorbid disorders like obesity, diabetes, dyslipemia, hypertension and an increased rate of cardiovascular disease and metabolic syndrome and these patients need safer treatments. The accumulating clinical experience with new therapies consistent on biological agents like efalizumab indicates that are effective therapies, with safety profile and no evidence of cumulative toxicity that allows a long term use. A better control of psoriasis improves patients quality of life.

Leucemia/linfoma de células dendríticas plasmocitoides precoces / No disponible

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