[Pediatric genital lichen sclerosus: a case series of 11 girls].

BACKGROUND: Genital lichen sclerosus decreases the quality of life of women; 10-15% of cases occur in prepubertal girls. METHODS: Retrospective and descriptive study on the characteristics of girls diagnosed with genital lichen sclerosus at the Hospital Universitario de Navarra (Pamplona, Spain) between 2019 and 2022. RESULTS: Eleven girls aged between 4 and 14 year-old were diagnosed. Frequently, diagnostic delays were up to two years after the appearance of the lesions; the girl with a four-year delay showed a significant vulvar architectural alteration. All cases showed the typical sclerotic lesions on the genital area, and two of them also on the back. While six patients were asymptomatic, the rest reported pruritus and/or pain. Treatment with high/very high potency topical cortico-steroids achieved a good partial response, without complete remission of the lesions. CONCLUSION: Early diagnosis of genital lichen sclerosus is key to start early treatment, avoiding ireversible genital structural alteration.

Liquen escleroso genital pediátrico en niñas: serie de 11 casos / Pediatric genital lichen sclerosus: a case series of 11 girls

Fundamento: El liquen escleroso genital disminuye la calidad de vida de las mujeres; el 10-15% de los casos se dan en prepúberes. Métodos: Estudio descriptivo retrospectivo de las características de las niñas diagnosticadas con liquen escleroso genital en el Hospital Universitario de Navarra (España) entre 2019 y 2022. Resultados: Se diagnosticaron once niñas entre 4 y 14 años. Fue habitual un retraso diagnóstico tras la aparición de las lesiones de hasta dos años; la niña con cuatro años de retraso presentaba una importante alteración arquitectural vulvar. Todas presentaban lesiones escleróticas típicas en genitales, y dos de ellas también tenían afectación en espalda. Seis pacientes eran asintomáticas, el resto refería prurito y/o dolor. El tratamiento con corticoides tópicos de alta/muy alta potencia logró una buena respuesta parcial, sin remisión completa de las lesiones. Conclusión: El diagnóstico precoz de liquen escleroso genital es clave para para iniciar el tratamiento lo antes posible y evitar una alteración estructural genital irreversible.

Background. Genital lichen sclerosus decreases the quality of life of women; 10-15% of cases occur in prepubertal girls. Methods: Retrospective and descriptive study on the characteristics of girls diagnosed with genital lichen sclerosus at the Hospital Universitario de Navarra (Pamplona, Spain) between 2019 and 2022. Results: Eleven girls aged between 4 and 14 year-old were diagnosed. Frequently, diagnostic delays were up to two years after the appearance of the lesions; the girl with a four-year delay showed a significant vulvar architectural alteration. All cases showed the typical sclerotic lesions on the genital area, and two of them also on the back. While six patients were asymptomatic, the rest reported pruritus and/or pain. Treatment with high/very high potency topical corticosteroids achieved a good partial response, without complete remission of the lesions. Conclusion: Early diagnosis of genital lichen sclerosus is key to start early treatment, avoiding irreversible genital structural alteration.(AU)

Livedo Racemosa, Reticulated Ulcerations, Panniculitis and Violaceous Plaques in a 46-year-old Woman.

Clinically amyopathic dermatomyositis (CADM) is a subset of dermatomyositis (DM) that has conventional cutaneous manifestations of DM, but paradoxically, little or no muscle involvement. In 2005, a novel antibody was described in association with CADM - anti-melanoma differentiation-associated gene 5 (anti-MDA5). Patients with this serologic marker have a characteristic mucocutaneous phenotype consisting of skin ulceration among other signs. We describe the case of a 46-year-old woman with CADM, elevated anti-MDA5 autoantibodies, and unusual clinical features (livedo racemosa, florid acral edema) among the classical phenotype of MDA5 DM (arthralgias, ulcerations, panniculitis) and classical DM lesions (Gottron papules, heliotrope rash). The patients did not develop interstitial lung disease or internal malignancies and experienced a rapid response to prednisolone and intravenous immunoglobulins. After 2 years, she has no relapse of her cutaneous disease and continues 5 mg prednisolone and 2 g/kg kilogram of intravenous immunoglobulin every 3 months for maintenance. Our case highlights the clinical heterogeneity of CADM and underscores the importance of a comprehensive approach to DM patients. It was previously postulated that anti-MDA5 antibody could target vascular cells and compromise vascular function, the presence of livedo racemosa lesions, and MDA5 antibodies in a patient with negative thrombophilia workup, reinforce this idea. This is the first case, to our knowledge, of CADM with acral panniculitis and livedo racemosa.

A New Equation for Predicting Evolution of Oral Pain in Orthodontic Treatment: A Longitudinal, Prospective Cohort Study.

AIMS: To develop an equation capable of relating the evolution of oral pain to the time elapsed, measured from the moment of dental archwire fitting and identifying when pain begins, peaks, and ends; and secondly, to compare pain during orthodontic treatment in relation to archwire material (steel or nickel-titanium [Ni-Ti]) and position (maxillary or mandibular) and patient age (child, teenager, or adult) and gender (male or female). METHODS: A longitudinal prospective cohort study was conducted of 112 patients who filled in a scale to evaluate pain, noting the times when the pain occurred. The total sample consisted of 60 males and 52 females with a mean (± standard deviation [SD]) age of 19.8 ± 6.2 years. The sample was divided into five groups depending on archwire material and position, and patient age and gender. A univariate four-way ANOVA model was performed to compare mean pain levels between groups. Bonferroni test was used for multiple comparisons. A univariate nonlinear regression model was carried out for pain level, 95% confidence intervals (95% CI) were calculated, and the statistic R² was used. RESULTS: An equation was developed based on pain levels in relation to time elapsed, measured from the moment when the archwire had been fitted in the mouth. The equation had three coefficients related to mean pain values: overall pain, peak pain, and how pain decreased. It fitted all study groups with a correlation coefficient > 0.9. The model showed that pain levels were influenced by archwire material and patient gender and age, but not archwire position. CONCLUSION: The equation reproduced the data registered and can be applied to studies of pain derived from archwires, and this methodology could be used for other external agents fitted in the mouth. Patients receiving dental treatment involving external agents can be made aware of the pain they can expect to experience. This will enable them to distinguish expected pain from other pain, which will help them identify other pathologies requiring medical attention and to approach treatment with better motivation since the pattern of pain evolution is known in advance.

[Contact allergy to 3-dimethylaminopropylamine and cocamidopropyl betaine]. / Alergia de contacto a 3-dimetilaminopropilamina y cocamidopropil betaína.

It has been discovered that all individuals who are allergic to cocamidopropyl betaine (CAPB) are sensitized to 3-dimethylaminopropylamine (DMAPA) and to amidoamine, molecules which are intermediaries in CAPB synthesis, and which persist as impurities in the material that is sold; the amounts vary, depending on the quality of the CAPB in the end product. We present three cases of allergic contact dermatitis to DMAPA. In all three cases, the skin tests for DMAPA were positive, while there was no reaction to CAPB. The current relevance of these tests was confirmed by the patients' re-exposure to the suspect products themselves, which contained CAPB. Contact allergy to CAPB is now infrequent, partly because of the increasing use of new non-irritating surfactants that have been introduced on the market in the last decade. However, cases of patients allergic to commercial CAPB who only react to DMAPA -and not to CAPB- when they are patch tested are still being reported. DMAPA itself, and other molecules like amidoamine, would be the true allergens, and some cases of CAPB allergy are therefore being overlooked because DMAPA is not always included in the cosmetics series. CAPB may no longer be necessary in patch tests, as DMAPA seems to be the principal allergenic fraction in this surfactant, and also because manufacturers of skin allergens currently prepare CAPB extracts that are so pure that they are no longer a good screening tool for contact allergy to commercial CAPB.

Alergia de contacto a 3-dimetilaminopopilamina y cocamidopropil betaína / Contact allergy to 3-dimethylaminopropylamine and cocamidopropyl betaine

Se ha descubierto que todos los individuos con alergia a la cocamidopropil betaína (CAPB) se encuentran sensibilizados a la 3-dimetilaminopropilamina (DMAPA) y a la amidoamina, moléculas que son intermediarias en la síntesis de CAPB, y que persisten como impurezas en el material comercializado, en cantidades variables dependiendo de la calidad de la CAPB en el producto final. Se presentan 3 casos de dermatitis de contacto alérgica a DMAPA. En los tres las pruebas epicutáneas para DMAPA fueron positivas, mientras que no hubo reacción a la CAPB. La relevancia actual de estas pruebas fue confirmada por la reexposición de los pacientes a los productos propios sospechados, que contenían CAPB. La alergia de contacto a la CAPB es ahora poco frecuente, en parte por el creciente uso de nuevos agentes tensoactivos no irritantes introducidos en el mercado en la última década. Sin embargo, siguen dándose a conocer casos de pacientes alérgicos a la CAPB comercial que sólo reaccionan a la DMAPA ­y no a la CAPB­ cuando son parcheados. La propia DMAPA u otras moléculas como la amidoamina serían los alérgenos verdaderos y, por tanto, algunos casos de alergia a la CAPB se estarían pasando por alto debido a que la DMAPA no siempre está incluida en la serie de cosméticos. La CAPB podría no ser ya necesaria en las pruebas del parche, puesto que la DMAPA parece ser la principal fracción alergénica en este agente tensoactivo, y también porque los fabricantes de alérgenos epicutáneos elaboran unos extractos de CAPB tan puros que no son ya una buena herramienta de cribado de la alergia de contacto a la CAPB comercial

It has been discovered that all individuals who are allergic to cocamidopropyl betaine (CAPB) are sensitized to 3-dimethylaminopropylamine (DMAPA) and to amidoamine, molecules which are intermediaries in CAPB synthesis, and which persist as impurities in the material that is sold; the amounts vary, depending on the quality of the CAPB in the end product. We present three cases of allergic contact dermatitis to DMAPA. In all three cases, the skin tests for DMAPA were positive, while there was no reaction to CAPB. The current relevance of these tests was confirmed by the patients' re-exposure to the suspect products themselves, which contained CAPB. Contact allergy to CAPB is now infrequent, partly because of the increasing use of new non-irritating surfactants that have been introduced on the market in the last decade. However, cases of patients allergic to commercial CAPB who only react to DMAPA ­and not to CAPB­ when they are patch tested are still being reported. DMAPA itself, and other molecules like amidoamine, would be the true allergens, and some cases of CAPB allergy are therefore being overlooked because DMAPA is not always included in the cosmetics series. CAPB may no longer be necessary in patch tests, as DMAPA seems to be the principal allergenic fraction in this surfactant, and also because manufacturers of skin allergens currently prepare CAPB extracts that are so pure that they are no longer a good screening tool for contact allergy to commercial CAPB

Manifestaciones cutáneas del síndrome de Hunter / Cutaneous manifestations of hunter syndrome

Las mucopolisacaridosis son un grupo de enfermedades de depósito en las cuales el déficit de una determinada enzima lisosomal produce la acumulación de mucopolisacáridos en los lisosomas de las células de varios órganos, incluyendo la piel. El síndrome de Hunter o mucopolisacaridosis tipo II se debe al déficit de la iduronato 2 sulfatasa, que da lugar al depósito y aumento de la excreción urinaria de dermatán y heparán sulfato. Las manifestaciones del síndrome de Hunter incluyen disostosis múltiples, organomegalia, facies tosca, ausencia de opacidad corneal y retraso mental con deterioro neurológico progresivo en la forma grave. Cambios cutáneos no específicos que pueden verse en todas las mucopolisacaridosis son piel engrosada con pérdida de elasticidad en codos y rodillas, pelo áspero e hipertricosis. La presencia de pápulas de color marfil sobre la región escapular y las caras laterales externas de brazos y muslos son típicas y casi patognomónicas del síndrome de Hunter. Se presenta el caso de un niño con la forma moderada del síndrome de Hunter que presenta lesiones cutáneas características de este síndrome (AU)

Autocontrol: variables cognitivas y sociopersonales en niños pre-escolares / Self-control: cognitive and social variables in preschool children

El tema del autocontrol se relaciona con la determinación de conductas orientadas hacia metas futuras. Los sujetos con alto nivel de autocontrol son capaces de superar la tentación de caer en gratificaciones inmediatas, y por ende, de persistir en esfuerzos de progresos, lo que implica recompensas tardías en el tiempo. El autocontrol definido como la habilidad para anticipar consecuencias futuras, tomar decisiones y ejecutar planes de acuerdo a esas consecuencias se relaciona directamente con las competencias cognitivas y sociales de los individuos. El objetivo del presente trabajo fue el estudio de las variables cognitivas, sociopersonales que están relacionadas con el autocontrol en 48 niños preescolares. Las variables cognitivas estudiadas fueron las de lenguaje y las estrategias congitivas usadas por los niños durante el tiempo de espera. La variable sociopersonal considerada fue la confianza del niño hacia los adultos significativos. Los resultados mostraron que a mayor nivel de lenguaje hay una mayor capacidad de autocontrol. En cuanto a las estrategias cognitivas empleadas por los preescolares, se observó que sólo la pregunta referida a la creencia de que el refuerzo destapado (materialmente presente) ayudaría la espera, se correlacionaba positivamente con la capacidad de autocontrol. La variable confianza, medida a través del test de expectativas generalizada de confianza (Borelli y Aber, 1986) y la manipulación experimental de la confianza, no mostraron efectos significativos sobre la capacidad de autocontrol de los preescolares. Sin embargo, las dificultades encontradas en las mediciones de esta variable no permiten concluir confiablemente sobre su efecto en el autocontrol

Aspectos psiconeurofisiológicos de la involución senil / Psychoneurophysiological aspects in senile involution

Se estudian las modificaciones que experimenta el electroencefalograma en la involución senil y se plantea la importancia que tienen los procesos adaptativos en la senectud