[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)

Bcl-2 and CD10 expression in the differential diagnosis of trichoblastoma, basal cell carcinoma, and basal cell carcinoma with follicular differentiation.

BACKGROUND: Both trichoblastoma and basal cell carcinoma (BCC) of the skin are characterized morphologically by the proliferation of basaloid cells; however, BCCs are clinically associated with a more aggressive behavior. An accurate diagnosis of these lesions is essential for effective, timely treatment and appropriate therapeutic decisions. METHODS: This study includes 40 lesions. Bcl-2 and CD10 immunohistochemistry were performed in all cases and the patterns of expression were analyzed. RESULTS: Bcl-2 is useful for the detection of BCC with diffuse expression in nests of basaloid cells, but cannot distinguish between BCC with follicular differentiation and trichoblastoma, as both lesions show the same pattern with positive and negative areas. Conversely, CD10 expression can distinguish between trichoblastomas with peritumoral stromal staining and BCCs with epithelial staining. If both stromal and epithelial areas are stained, these cases are classified as BCC with follicular differentiation. CONCLUSIONS: CD10 is useful for distinguishing between BCC with widespread follicular differentiation and trichoblastomas.

[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