Subject(s)
Humans , Female , Infant , Hamartoma/diagnosis , Hamartoma/pathology , Biopsy , Magnetic Resonance Imaging , Ultrasonography , Diagnosis, DifferentialSubject(s)
Humans , Female , Infant , Psoriasis/diagnosis , Psoriasis/therapy , Dermatitis/diagnosis , Skin Diseases/diagnosis , Diagnosis, DifferentialSubject(s)
Humans , Female , Child , Focal Epithelial Hyperplasia/drug therapy , Lip Diseases , Mouth Mucosa/injuries , Scleromyxedema , Skin/injuriesABSTRACT
Mycosis fungoides (MF), the most common primary cutaneous T-cell lymphoma, is unusual in children. OBJECTIVES: We aimed to describe the epidemiologic, clinical, histopathologic, and immunophenotypic characteristics of MF as well as treatments and course of disease in a pediatric case series. MATERIAL AND METHOD: Data for all patients admitted to our pediatric hospital (Hospital Dr. J. P. Garrahan) in Argentina with a clinical and histopathologic diagnosis of MF between August 1988 and July 2014 were included. RESULTS: A total of 14 patients were diagnosed with MF. The ratio of boys to girls was 1:1.33. The mean age at diagnosis was 11.23 years (range, 8-15 years). The mean time between onset and diagnosis was 3.5 years (range, 4 months-7 years). All patients had hypopigmented MF and 42% also presented the features of classic MF. Seven (50%) had the CD8+ immunophenotype exclusively. Seventy-eight percent were in stage IB at presentation. Phototherapy was the treatment of choice. Four patients relapsed at least once and skin lesions progressed in 3 patients. All patients improved. CONCLUSIONS: MF is unusual in children. The hypopigmented form is the most common. Diagnosis is delayed because the condition is similar to other hypopigmented diseases seen more often in childhood. Although prognosis is good, the rate of recurrence is high, so long-term follow-up is necessary.
Subject(s)
Hospitals, Pediatric , Mycosis Fungoides/epidemiology , Skin Neoplasms/epidemiology , Adolescent , Adrenal Cortex Hormones/therapeutic use , Age of Onset , Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Argentina/epidemiology , Child , Cross-Sectional Studies , Delayed Diagnosis , Diagnostic Errors , Female , Hospitals, Pediatric/statistics & numerical data , Humans , Hypopigmentation/etiology , Male , Mycosis Fungoides/drug therapy , Mycosis Fungoides/pathology , Mycosis Fungoides/radiotherapy , PUVA Therapy , Recurrence , Retrospective Studies , Skin Diseases/diagnosis , Skin Neoplasms/drug therapy , Skin Neoplasms/pathology , Skin Neoplasms/radiotherapy , Ultraviolet TherapyABSTRACT
Nevoid basal cell carcinoma (BCC) syndrome, or Gorlin syndrome, is a rare autosomal dominant disorder associated with mutations in the patched 1 gene, PTCH1. It is characterized by the presence of multiple BCCs in association with disorders affecting the bones, the skin, the eyes, and the nervous system. We describe 6 cases of nevoid BCC syndrome evaluated in our department. Palmoplantar pitting was observed in all 6 patients, multiple BCCs in 5 patients (83%), skeletal anomalies in 3 patients (50%), and odontogenic keratocysts in 1 patient (17%). We would like to stress the importance of early diagnosis and treatment in nevoid BCC syndrome and the need for continuous, long-term follow-up by a multidisciplinary team.
Subject(s)
Basal Cell Nevus Syndrome , Skin Neoplasms , Adolescent , Basal Cell Nevus Syndrome/diagnosis , Child , Child, Preschool , Female , Hospitals, Pediatric , Humans , Male , Skin Neoplasms/diagnosisABSTRACT
Paraneoplastic pemphigus is an autoimmune blistering disease associated with an occult or previously diagnosed tumor. Its clinical, histological, and immunological features have been clearly defined. It is characterized by the presence of polymorphic skin lesions and by erosions of the oral and genital mucosas that are refractory to conventional treatments. The histology can be variable and includes acantholysis or lichenoid dermatitis. Circulating autoantibodies are a constant feature and confirm the diagnosis. We describe 2 girls with paraneoplastic pemphigus associated with Hodgkin lymphoma in one and Castelman disease in the other. Both children had oral and genital lesions that did not respond to conventional treatments. Biopsy revealed acantholysis in one and a lichenoid reaction in the other, and immunoassays confirmed the diagnosis. Chemotherapeutic treatment of the underlying disease was performed in both cases, together with high-dose corticosteroids for the skin and mucosal lesions. Both patients died due to respiratory failure. We suggest that paraneoplastic pemphigus, although rare in childhood and adolescence, should be included in the differential diagnosis of periorificial erosive dermatitis; this may assist in the detection of an occult neoplasm.
Subject(s)
Autoimmune Diseases/pathology , Paraneoplastic Syndromes/pathology , Pemphigus/pathology , Child , Fatal Outcome , Female , HumansSubject(s)
Humans , Infant , Child, Preschool , Child , Adolescent , Dermatitis, Atopic/diagnosis , Dermatitis, Atopic/etiology , Dermatitis, Atopic/physiopathology , Dermatitis, Atopic/drug therapy , Dermatitis, Atopic/therapy , Chronic Disease , Diagnosis, Differential , Immunosuppressive Agents/therapeutic useABSTRACT
El calcipotriol, un análogo de la vitamina D, es un tratamiento eficaz y seguro para la psoriasis en placas de carácter leve a moderado en pacientes adultos. Objetivos: Evaluar la eficacia y tolerancia del ungüento de calcipotriol en el tratamiento tópico de la psoriasis en niños. Determinar la influencia del mismo sobre el metabolismo cálcico y la presencia de efectos adversos. Materiales y métodos: 14 niños con psoriasis vulgar con menos del 30% de superficie corporal comprometida fueron tratados durante 8 semanas con calcipotriol ungüento 2 veces por día. Se evaluaron clínicamente a través del PASI la extensión y severidad de la enfermedad la eficacia clínica global y la tolerancia de la medicación. Se realizaron estudios de laboratorio (hematología, función renal, hepática, calcio sérico y urinario, fósforo, PTH y 25 hidroxivitamina D, antes de iniciar el tratamiento y las cuatro semanas. Resultados: 14 niños completaron el estudio, 7 mujeres y 7 varones. La edad promedio fue de 9 años. El PASI diminuyó en el 71%. No se detectaron efectos adversos a excepción de un leve ardor en dos pacientes. No hubo alteraciones de los parámetros de laboratorio incluyendo aquellos relacionados con la homeostasis del calcio. La tolerancia de la medicación fue excelente. Conclusiones: El ungüento de calcipotriol es un tratamiento seguro y eficaz para la psoriasis infantil. Constituye una terapéutica aceptable y útil junto a otros tratamientos antipsoriáticos furante la infancia.
Subject(s)
Child, Preschool , Child , Adolescent , Ointments/therapeutic use , Psoriasis/diagnosis , Psoriasis/therapy , Vitamin D/therapeutic useABSTRACT
La enfermedad de Kawasaki es una vasculitis multisistémica febril aguda, que afecta generalmente a niños menores de 5 años, de curso limitado. Se caracteriza por fiebre prolongada, alteraciones cutáneas y mucosas, adenitis cervical y manifestaciones sistémicas, siendo las cardiovasculares las que determinan la morbimortalidad de la enfermedad. Objetivo: Describir los hallazgos clínicos, de laboratorio y resultados del tratamiento en pacientes con diagnóstico de enfermedad de kawasaki evaluados en el Hospital de Pediatría Juan P. Garrahan entre agosto de de 1988 y diciembre de 1999. Materiales y métodos: Se hizo un análisis retrospectivo de los datos consignados en las historias clínicas de pacientes con diagnóstico de enfermedad de kawasaki, internados en nuestro hospital. Se analizaron las características y la frecuencia de los criterios diagnósticos, los hallazgos clínicos asociados, las alteraciones de los exámenes de laboratorio y estudios ecográficos y la respuestas al tratamiento. Resultados: Se evaluaron 100 pacientes, siendo la relación varón/mujer de 1,38/1. La media de edad de los niños estudiados fue de 23 meses (rango 3 meses a 9 años). En cuanto a los criterios diagnósticos, se constato fiebre en el 100% de los pacientes, inyección conjuntival en el 77%, compromiso de la mucosa orofaríngea en el 88%, compromiso de las extremidades en el 89%, exantema en el 95% y compromiso ganglionar en el 68%. Con respectos a las manifestaciones clínicas asociadas se observó compromiso cardiovascular en 39%; gastrointestinal en el 25%; osteoarticular en el 14%; genitourinario en el 12% y neurológico en el 90%. Las alteraciones del laboratorio encontradas incluyeron anemia en el 69% , leucocitosis en el 79% y trombocitosis en el 87%. El 97% de los pacientes recibió tratamiento, gammaglobulina intravenosa y ácido acetil salicílico (vía oral) 66 pacientes, ácido acetil salicílico solamente 29 pacientes y gammaglobulina intravenosa solamente 2 pacientes.
