Pigmented onychomatricoma: A clinical simulator that could not mislead ultrasound.

INTRODUCTION: Onychomatricoma is a rare subungual tumor composed of stroma and nail matrix-like epithelium. Only seven cases of the pigmented variant have been reported in the literature. CASE REPORT: We present a 62-year-old male patient with a 2-years history of a single fingernail thickening, deformation, and hyperpigmentation. He had been treated for onychomycosis, without improvement. Due to the increasing hyperpigmentation, and to rule out tumoral entities, including melanoma, a color Doppler ultrasound examination of the nail was requested, which showed a hypoechoic tumor of the nail matrix and bed, with hyperechogenic linear images inside, suggesting the diagnosis of onychomatricoma. Onychectomy and partial matricectomy were performed. Histological evaluation confirmed the diagnosis of onychomatricoma. Discusion and conclusion: Histological evaluation is the gold standard for the diagnosis of onychomatricoma. However, ultrasonography represents an easily accessible, non-invasive diagnostic tool for the initial evaluation of these tumors, especially in unsuspected atypical clinical presentations.

Manifestaciones cutáneas en niños con cáncer en tratamiento con quimioterapia en comparación con niños sanos / Cutaneous manifestations in children with cancer in chemotherapy treatment compared to children healthy

Introducción: Los niños con cáncer pueden presentar múltiples dermatosis derivadas del cáncer mismo o secundarias a su terapéutica. El objetivo es conocer las manifestaciones cutáneas e niños con cáncer en quimioterapia, y compararlas con niños controles sanos. Este es el primer estudio chileno que las describe. Pacientes y método: Se realizó un estudio analítico descriptivo transversal, mediante examen físico y registro en ficha. Se estudiaron 82 niños. Los casos fueron 41 niños con cáncer en quimioterapia del Servicio de Oncología Infantil del Hospital Sótero del Río. Los controles, 41 pacientes sanos hospitalizados por patología quirúrgica en el mismo hospital. Ambos grupos fueron pareados por sexo, edad, estado nutricional, fenotipo y tipo de exposición solar en relación 1:1. Los datos se analizaron con software SPSS. Resultados: La xerosis fue la manifestación cutánea más frecuente en ambos grupos 73,2% (n=60). En niños con cáncer se observó en el 82,4% (34) y en niños sanos 63,4% (26). Ambos grupos, tuvieron igual frecuencia de enfermedades infecciosas 14,6% e inflamatorias 19,5%. En los niños oncológicos predominaron las infecciones virales; dermatitis de contacto; y efectos asociados a la quimioterapia, como la hiperpigmentación cutánea y efluvio anágeno. En los niños controles, se encontraron más frecuentemente las tiñas y prurigo. No se reportaron efectos adversos a medicamentos. En ambos grupos, no hubo diferencia en cuanto al número total de nevi. Conclusión: La manifestación cutánea más frecuente fue la xerosis en ambos grupos y los niños con cáncer no presentaron más dermatosis que los controles sanos, salvo las asociadas a su tratamiento.

Introduction: Children with cancer can present multiple dermatoses derived from the cancer itself or secondary to its therapy. The objective is to know the skin manifestations of children with cancer in chemotherapy, and compare them with healthy controls. This is the first Chilean study that describes them. Patients and method: A transversal descriptive analytical study was carried out. By means of a physical examination and review the medical record, 82 children were studied. The cases were 41 children with cancer in chemotherapy from the Children's Oncology Service of Sótero del Río Hospital. The controls, 41 healthy patients hospitalized for surgical pathology in the same hospital. Both groups were matched by sex, age, nutritional status, phenotype and type of sun exposure in a 1: 1 ratio. The data was analyzed with SPSS software. Results: Xerosis was the most common cutaneous manifestation found in both groups 73.2% (n = 60). In children with cancer was found 82,4% (34) and in cases 63,4% (n = 26). Both groups had the same frequency of inflammatory 14,6%, or infectious dermatosis 19,5%. In oncologyc patients, predominated viral infections; contact dermatitis; and adverse drugs reactions associated with chemotherapy, such as skin hyperpigmentation and anagen effluvium. In the controls, were more found superficial fungal infections, and prurigo. No adverse drugs reactions were observed in this group. In both groups, there was no difference in the total number of nevi. Conclusion: The most frequent cutaneous manifestation was xerosis in both groups and children with cancer did not present more dermatosis than healthy controls, except those associated with their treatment.

Histiocitosis de células de Langerhans: el "camaleón" de la dermatopediatría / Langerhans cell histiocytosis

La Histiocitosis de Células de Langerhans (HCL) es una neoplasia mieloide de las células dendríticas Langerhans (CDL), caracterizada por acúmulos de células dendríticas mieloides CD207+. Corresponden a un espectro de enfermedades, desde sólo cutáneas a variantes multiorgánicas. El objetivo de este reporte es describir el caso clínico de un paciente pediátrico, con diagnóstico de Histiocitosis de Células de Langerhans, enfatizando el algoritmo clínico. Paciente masculino de 1 año y 5 meses, con antecedentes de otorrea persistente, refractaria a tratamiento, de un año de evolución. Consulta en policlínico de dermatología por "dermatitis severa" desde hace 1 año. Al examen físico se constatan lesiones tipo dermatitis seborreica generalizadas en tronco y cuero cabelludo, intertrigo fisurado, pápulas eritemato-costrosas plantares con petequias y pus en conducto auditivo externo bilateral. Presenta Hemoglobina 9,5 mg/dl, Hematocrito31,9%, leucocitos 12.400, linfocitos 33,3%, plaquetas 920.000, VHS 27. Subpoblaciones linfocitarias: CD3: 34,7%, C4: 22,7%, CD8: 9,7%, CD19:47,8%. HTLV negativo, VIH negativo. Acaro-test negativo. Dermatopatología: Denso infiltrado de células linfomonocíticas en dermis papilar, con ensanchamiento de estas y gran epidermotropismo, con abundante citoplasma eosinófilo con núcleos arriñonados, CD1a y langerina positivo. Recomendamos elevar la sospecha diagnóstica ante un cuadro de dermatitis seborreica generalizada que esta fuera del rango etario característico y en casos de dermatitis refractarias, donde a pesar de un adecuado tratamiento médico, el paciente persiste comprometido.

Langerhans Cell Histiocytosis (HCL) is a myeloid neoplasm of Langerhans dendritic cells (CDL), characterized by accumulations of myeloid dendritic cells CD207 +. They correspond to a spectrum of diseases, from cutaneous to multi-organ variants. The objective of this report is to describe the clinical case of a pediatric patient with diagnosis of, emphasizing the clinical algorithm. Male patient,1 year and 5 months old, with a history of refractory persistent otorrhea, consulted because of long term severe dermatitis. Physical examination revealed generalized seborrheic dermatitis lesions on the trunk and scalp, cleft intertrigo, plantar erythematous-crusted papules with petechiae, and pus in the external auditory canal. Laboratory findings showed: Hemoglobin 9.5 mg / dl, Hematocrit: 31.9%, leukocytes: 12,400, lymphocytes 33.3%, platelets: 920,000, HSV 27. Lymphocyte subpopulations: CD3: 34.7%, C4: 22.7%, CD8: 9.7%, CD19: 47.8%. HTLV negative, HIV negative. Scabies Negative. Dermatopathology: Dense infiltrate of lymphomonocytic cells in the papillary dermis with widening of the papilla and large epidermotropism, cells show abundant eosinophilic cytoplasm with "kidney nuclei", CD1a and langerin were positive. We recommend elevating the diagnostic suspicion in the face of a generalized seborrheic dermatitis that is outside the characteristic age range and in cases of refractory dermatitis, where the patient persists compromised.

Dowling-Degos disease: a therapeutic challenge: report of a family with no response to laser treatments

La enfermedad de Dowling-Degos (DDD), conocida también como 'anomalía reticulada y pigmentada de las flexuras' es una rara genodermatosis autosómica dominante. Se caracteriza por la aparición de máculas hiperpigmentadas de configuración reticulada; afectando principalmente los grandes pliegues como las axilas e ingles. Pudiendo, además, comprometer otros pliegues como cervicales, antecubitales, submamarios e interglúteos. Otras características asociadas son las lesiones tipo comedones y los pits palmo-plantares. Presentamos el caso de una familia con enfermedad de Dowling-Degos sin respuesta al tratamiento con laser Nd:YAG y CO2. Se realiza una revisión de la literatura de los tratamientos disponibles.

Dowling-Degos disease (DDD), also known as "reticulate pigmented anomaly of the flexures", is a rare autosomal dominant genodermatosis. DDD is characterized by an acquired reticular skin hyperpigmentation which begins in the axillae and groin. It later involves other body folds, including neck, inner aspects of the arms and thighs, inframammary, and intergluteal folds. Associated features include comedolike lesions on the neck or back, pitted facial or perioral scars, and epidermoid cysts. Herein we present a family (proband, mother, grandmother) with DDD that were treated with Q-switched Nd:YAG laser and CO2 laser without response. Treatment options are discussed and the available literature is reviewed.

[Ecthyma gangrenosum caused by Staphylococcus aureus]. / Ectima gangrenoso secundario a Staphylococcus aureus.

Ecthyma gangrenosum is an uncommon necrotizing vasculitis, in most cases secondary to sepsis by Pseudo-mona aeruginosa in immunocompromised patients. However, there have been several reports of ecthyma gangre-nosum caused by other infectious etiologies. We report an unusual case of ecthyma gangrenosum associated with methicillin-resistant Staphylococcus aureus infection in a patient without the classic immunological risk factors described in the literature.

Ectima gangrenoso secundario a Staphylococcus aureus / Ecthyma gangrenosum caused by Staphylococcus aureus

El ectima gangrenoso es una vasculitis necrosante poco frecuente, en la mayoría de los casos secundaria a sepsis por Pseudomonas aeruginosa en pacientes inmunocomprometidos. Sin embargo, existen reportes de ectima gangrenoso secundarios a otras etiologías infecciosas. Presentamos un caso de ectima gangrenoso asociado a una infección por Staphylococcus aureus resistente a meticilina en una paciente sin los factores de riesgo clásicos de inmunosupresión que se describen en la literatura médica.

Ecthyma gangrenosum is an uncommon necrotizing vasculitis, in most cases secondary to sepsis by Pseudo-mona aeruginosa in immunocompromised patients. However, there have been several reports of ecthyma gangre-nosum caused by other infectious etiologies. We report an unusual case of ecthyma gangrenosum associated with methicillin-resistant Staphylococcus aureus infection in a patient without the classic immunological risk factors described in the literature.