Eritrodermia ictiosiforme congénita ampollosa: a propósito de un caso / Congenital bullous ichthyosiform erythroderma

La eritrodermia ictiosiforme congénita ampollosa es una entidad poco frecuente, con herencia autosómica dominante, pero un 50% se presenta por mutaciones de novo; se caracteriza en su fase inicial por eritrodermia y aparición espontánea de ampollas. Posteriormente las ampollas disminuyen en frecuencia y aparece una descamación que compromete toda la superficie corporal. No suele haber afectación palmoplantar ni de las mucosas. Su tratamiento de primera línea son los retinoides sistémicos junto a los cuidados de la piel

Congenital bullous ichthyosiform erythroderma is a rare entity, with autosomal dominant inheritance, but in 50% of cases it is presented by de novo mutations; is characterized in its initial phase by erythroderma and spontaneous appearance of blisters. Subsequently the blisters decrease in frequency and appears a scaling that compromises the entire body surface. There is usually no palmoplantar or mucosal involvement. Its first-line treatment is systemic retinoids along with skin care

Micosis fungoide en niños y adolescentes: descripción de una serie de 23 casos / Mycosis Fungoides in Children and Adolescents: A Series of 23 Cases

ANTECEDENTES: La micosis fungoide (MF) es el tipo más común de linfoma cutáneo de células T tanto en adultos como en niños. En algunos países se ha observado un aumento de la prevalencia de MF en niños. Hasta la fecha, no existen estudios descriptivos de MF en la población pediátrica colombiana. MÉTODOS: En una revisión ambispectiva de 128 pacientes con el diagnóstico confirmado de MF en la Sección de Dermatología y del Laboratorio de Dermatopatología de la Universidad de Antioquia entre los años 2008-2017, se describen las variantes clínicas e histopatológicas, la respuesta al tratamiento y la progresión de la enfermedad de 23 pacientes menores de 18 años de edad. RESULTADOS: Los casos de MF en niños constituyeron el 18% del total. La mediana de edad de inicio de las lesiones fue de 9 años; la mediana de edad al momento del diagnóstico fue de 11 años y la mediana del tiempo promedio entre el inicio de las lesiones y el diagnóstico fue de 2 años. Todos los pacientes se encontraban en estadios tempranos de la enfermedad. La MF hipopigmentada fue la presentación clínica más frecuente (52,2%), seguida por la MF clásica (30,4%). La MF foliculotropa se presentó en el 17,4%. Todos los pacientes recibieron terapias dirigidas a la piel con esteroides tópicos y fototerapia, uno recibió quimioterapia, aún en estadio temprano. El 59,1% obtuvieron remisión completa y el 40,9% respuesta parcial. Solo 2 casos permanecieron asintomáticos durante 5 años. CONCLUSIÓN: En nuestra experiencia, la MF hipopigmentada fue la presentación clínica más común en pacientes menores de 18 años. No se presentaron progresiones a estadios avanzados. Sin embargo, las recurrencias después de la interrupción del tratamiento fueron comunes

BACKGROUND: Mycosis fungoides (MF) is the most common type of cutaneous T-cell lymphoma in adults and children. The prevalence has increased in some countries, but no descriptive studies of MF in the pediatric population have been done in Colombia to date. METHODS: A combined prospective-retrospective study of 128 patients with a diagnosis of MF confirmed by the dermatology department and dermatopathology laboratory of Universidad de Antioquia between 2008 and 2017. We describe the clinical and histopathologic variants, response to treatment, and progression of the disease in 23 patients under 18 years of age. RESULTS: The pediatric cases of MF accounted for 18% of all the cases on record. The median age of onset of lesions was 9 years, the median age at diagnosis was 11 years, and the median time between onset of lesions and diagnosis was 2 years. All patients were in early stages of the disease. Hypopigmented MF was the most common clinical presentation (in 52.2%), followed by classical MF (in 30.4%). Folliculotropic MF was identified in 17.4%. All patients were treated with topical corticosteroids and phototherapy. One patient received chemotherapy while still in the early stage of disease.complete remission was achieved in 59.1% and a partial response in 40.9%. Only 2 patients remained asymptomatic for 5 years. CONCLUSIÓN: We found hypopigmented MF to be the most common clinical presentation in patients under 18 years of age. The disease did not progress to advanced stages in any of the patients, although recurrence after treatment interruption was common

Human papillomavirus (HPV) viral load and HPV type in the clinical outcome of HIV-positive patients treated with imiquimod for anogenital warts and anal intraepithelial neoplasia.

OBJECTIVE: To evaluate the efficacy of 5% imiquimod in HIV-positive male patients with anogenital warts or anal intraepithelial neoplasia (AIN), and to elucidate whether human papillomavirus (HPV) type and viral load were important for clinical outcome and recurrences. METHODS: Thirty-seven patients with histologically proven anogenital warts or AIN were enrolled. Topical 5% imiquimod was applied three times per week for more than 8 h overnight for 16 weeks, although patients were allowed to continue therapy for 4 more weeks if they did not have complete clearance of lesions. RESULTS: Mean age was 34 years. The perianal area was the main lesion location. Thirty-three patients had CD4 counts of < 500 cells/mm(3). Eighteen patients had a histopathological diagnosis of AIN-1. Main HPV types detected corresponded to low-risk HPV types. At 20 weeks of therapy, 46% patients achieved total clearance whereas 14 patients had > 50% clearance. Recurrence was observed in 5 of 17 patients who cleared. Clearance was not influenced by patients' CD4 counts, wart location, HIV viral load or HPV viral load. CONCLUSIONS: The assumption that visible perianal warts are benign lesions in HIV-positive patients has to be reevaluated since an important number of such lesions could correspond to low-grade anal disease, which in turn could progress to high-grade anal disease or cancer. In addition, our results in this preliminary study indicate that imiquimod appears to be effective in treating AIN in HIV-positive patients. Further studies are needed to document its utility to prevent high-grade dysplasia and/or anal cancer.