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El síndrome de Wells o celulitis eosinofílica es una enfermedad inflamatoria de origen desconocido, de aparición infrecuente en la edad pediátrica. Suele manifestarse clínicamente como placas eritematoedematosas, nódulos, pápulas, ampollas, entre otros. Se presenta una paciente en edad pediátrica con nódulos subcutáneos asintomáticos generalizados asociados a eosinofilia grave. El estudio histopatológico de las lesiones fue compatible con celulitis de Wells. Se realizó una evaluación interdisciplinaria en busca de la causa y trastornos eosinofílicos asociados, sin resultados positivos. Se indicó tratamiento sistémico con corticoides y presentó buena respuesta, pero, ante la recidiva de las lesiones tras su suspensión, se indicó dapsona como tratamiento de segunda línea, con mejoría posterior de las lesiones y de la eosinofilia. El objetivo del reporte es presentar una paciente con una manifestación atípica de síndrome de Wells y su desafío terapéutico.

Wells' syndrome, or eosinophilic cellulitis, is an inflammatory disease of unknown origin, uncommon in the pediatric age. It usually appears clinically as erythematous and edematous plaques, nodules, papules, blisters, among other symptoms. Here we describe the case of a female pediatric patient with generalized, asymptomatic subcutaneous nodules associated with severe eosinophilia. The histopathological examination of the lesions was compatible with Wells' syndrome. An interdisciplinary evaluation was performed to establish the cause and look for associated eosinophilic disorders; the results were negative. Systemic corticosteroids were indicated and the patient had a good response; however, in view of the recurrence of the lesions after treatment discontinuation, dapsone was indicated as a second-line treatment, with subsequent improvement of the lesions and eosinophilia. The aim of this report was to describe the case of a female patient with an atypical manifestation of Wells' syndrome and the resulting therapeutic challenge.

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La púrpura fulminante adquirida postinfecciosa es una entidad aguda y grave, poco frecuente, caracterizada por necrosis cutánea asociada a coagulopatía intravascular diseminada (CID), en ausencia de infección activa o alteraciones previas de la coagulación. Afecta fundamentalmente a la población pediátrica y, en el 90 % de los casos, está precedida por un proceso infeccioso. El mecanismo fisiopatológico es un déficit transitorio de proteína S mediado por autoanticuerpos que favorece un estado de hipercoagulabilidad. Se presenta el caso de un varón de 8 años previamente sano, con lesiones cutáneas purpúricas características de púrpura fulminante asociada a CID en ausencia de sepsis. Se constató deficiencia plasmática transitoria de proteína S. Requirió tratamiento sustitutivo con plasma fresco congelado y anticoagulación; la evolución fue favorable. La actividad de la proteína S permaneció disminuida durante 2 meses.

Acquired postinfectious purpura fulminans is a rare, acute, and severe disease characterized by skin necrosis associated with disseminated intravascular coagulation (DIC) in the absence of active infection or previous coagulation disorders. It mainly affects the pediatric population and, in 90% of cases, it is preceded by an infectious process. The pathophysiological mechanism is a transient autoantibodymediated protein S deficiency that favors a hypercoagulable state. Here we describe the case of a previously healthy 8-year-old boy with purpuric skin lesions typical of purpura fulminans associated with DIC in the absence of sepsis. A transient plasma protein S deficiency was confirmed. He required replacement therapy with fresh frozen plasma and anticoagulation; he had a favorable course. Protein S activity remained decreased for 2 months.

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Wells' syndrome, or eosinophilic cellulitis, is an inflammatory disease of unknown origin, uncommon in the pediatric age. It usually appears clinically as erythematous and edematous plaques, nodules, papules, blisters, among other symptoms. Here we describe the case of a female pediatric patient with generalized, asymptomatic subcutaneous nodules associated with severe eosinophilia. The histopathological examination of the lesions was compatible with Wells' syndrome. An interdisciplinary evaluation was performed to establish the cause and look for associated eosinophilic disorders; the results were negative. Systemic corticosteroids were indicated and the patient had a good response; however, in view of the recurrence of the lesions after treatment discontinuation, dapsone was indicated as a second-line treatment, with subsequent improvement of the lesions and eosinophilia. The aim of this report was to describe the case of a female patient with an atypical manifestation of Wells' syndrome and the resulting therapeutic challenge.

El síndrome de Wells o celulitis eosinofílica es una enfermedad inflamatoria de origen desconocido, de aparición infrecuente en la edad pediátrica. Suele manifestarse clínicamente como placas eritematoedematosas, nódulos, pápulas, ampollas, entre otros. Se presenta una paciente en edad pediátrica con nódulos subcutáneos asintomáticos generalizados asociados a eosinofilia grave. El estudio histopatológico de las lesiones fue compatible con celulitis de Wells. Se realizó una evaluación interdisciplinaria en busca de la causa y trastornos eosinofílicos asociados, sin resultados positivos. Se indicó tratamiento sistémico con corticoides y presentó buena respuesta, pero, ante la recidiva de las lesiones tras su suspensión, se indicó dapsona como tratamiento de segunda línea, con mejoría posterior de las lesiones y de la eosinofilia. El objetivo del reporte es presentar una paciente con una manifestación atípica de síndrome de Wells y su desafío terapéutico.

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RESUMO

Acquired postinfectious purpura fulminans is a rare, acute, and severe disease characterized by skin necrosis associated with disseminated intravascular coagulation (DIC) in the absence of active infection or previous coagulation disorders. It mainly affects the pediatric population and, in 90% of cases, it is preceded by an infectious process. The pathophysiological mechanism is a transient autoantibody-mediated protein S deficiency that favors a hypercoagulable state. Here we describe the case of a previously healthy 8-year-old boy with purpuric skin lesions typical of purpura fulminans associated with DIC in the absence of sepsis. A transient plasma protein S deficiency was confirmed. He required replacement therapy with fresh frozen plasma and anticoagulation; he had a favorable course. Protein S activity remained decreased for 2 months.

La púrpura fulminante adquirida postinfecciosa es una entidad aguda y grave, poco frecuente, caracterizada por necrosis cutánea asociada a coagulopatía intravascular diseminada (CID), en ausencia de infección activa o alteraciones previas de la coagulación. Afecta fundamentalmente a la población pediátrica y, en el 90 % de los casos, está precedida por un proceso infeccioso. El mecanismo fisiopatológico es un déficit transitorio de proteína S mediado por autoanticuerpos que favorece un estado de hipercoagulabilidad. Se presenta el caso de un varón de 8 años previamente sano, con lesiones cutáneas purpúricas características de púrpura fulminante asociada a CID en ausencia de sepsis. Se constató deficiencia plasmática transitoria de proteína S. Requirió tratamiento sustitutivo con plasma fresco congelado y anticoagulación; la evolución fue favorable. La actividad de la proteína S permaneció disminuida durante 2 meses.

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Generalized lymphatic anomaly (GLA) is an infrequent multiorgan disease characterized by the presence of abnormal proliferation of lymphatic vessels. The diagnosis requires histological confirmation, and the treatment is controversial. We are presenting a case of a 28-year-old male patient who was diagnosed with an extragonadal mediastinal nonseminomatous germ cell tumor. He underwent chemotherapy, and during this treatment, radiologic findings evidenced lytic lesions. Multiple biopsies were performed, which revealed the presence of abnormal lymphatic vessels, characteristic of GLA. There are different etiologies of osteolytic lesions, and on some occasions, they mimic a tumoral entity. The clinical suspicion of GLA is the first step in approaching the diagnosis, particularly in young adult patients.

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La hipertensión arterial (HTA) grave en pediatría responde fundamentalmente a causas secundarias. Presentamos una paciente adolescente de 14 años con HTA grave, alcalosis metabólica e hipopotasemia, secundaria a un tumor de células yuxtaglomerulares productor de renina, diagnosticado luego de dos años de evolución de HTA.

Severe arterial hypertension (HTN) in pediatrics is mainly due to secondary causes. Here we describe the case of a 14-year-old female adolescent with severe HTN, metabolic alkalosis, and hypokalemia, secondary to a renin-secreting juxtaglomerular cell tumor diagnosed after 2 years of HTN progression.

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Severe arterial hypertension (HTN) in pediatrics is mainly due to secondary causes. Here we describe the case of a 14-year-old female adolescent with severe HTN, metabolic alkalosis, and hypokalemia, secondary to a renin-secreting juxtaglomerular cell tumor diagnosed after 2 years of HTN progression.

La hipertensión arterial (HTA) grave en pediatría responde fundamentalmente a causas secundarias. Presentamos una paciente adolescente de 14 años con HTA grave, alcalosis metabólica e hipopotasemia, secundaria a un tumor de células yuxtaglomerulares productor de renina, diagnosticado luego de dos años de evolución de HTA.

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Abstract Basaloid follicular hamartoma is a benign, superficial malformation of hair follicles that can be mistaken both clinical and histopathologically for basal cell carcinoma. Basaloid follicular hamartoma has been linked to a mutation in the PTCH-1 gene, which is part of the same pathway involved in Gorlin-Goltz syndrome. Here we present a 9-year-old patient with an asymptomatic congenital lesion on the forehead, which increased in size over the years. Histopathology showed a basaloid follicular hamartoma associated with follicular mucinosis and inflammation. Gorlin-Goltz syndrome was ruled out by clinical examination.

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Basaloid follicular hamartoma is a benign, superficial malformation of hair follicles that can be mistaken both clinical and histopathologically for basal cell carcinoma. Basaloid follicular hamartoma has been linked to a mutation in the PTCH-1 gene, which is part of the same pathway involved in Gorlin-Goltz syndrome. Here we present a 9-year-old patient with an asymptomatic congenital lesion on the forehead, which increased in size over the years. Histopathology showed a basaloid follicular hamartoma associated with follicular mucinosis and inflammation. Gorlin-Goltz syndrome was ruled out by clinical examination.

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RESUMEN Las porfirias son trastornos metabólicos hereditarios causados por deficiencias enzimáticas de la biosíntesis del grupo HEM. Con presentación en distintos grupos de edades, más común en infancia y tercera a cuarta década de la vida, se caracterizan por elevación de porfirinas, y manifestaciones variadas cutáneas y neuro viscerales. Describimos una serie de 3 casos de pacientes femeninas en tercera década de la vida con dolor abdominal severo e inespecífico y una amplia gama de manifestaciones clínicas con sus complicaciones a corto y largo plazo en quienes se diagnosticó porfiria aguda intermitente (PAI). Se hará revisión en la literatura para aportar al reconocimiento temprano de estas condiciones e instaurar de forma temprana el manejo específico e impactar en desenlaces irreversibles.

ABSTRACT Porphyrias are inherited metabolic disorders caused by enzymatic deficiencies of HEM group biosynthesis. Most common in childhood at the third and fourth decade of life. They are characterized by increased levels of porphyrins, and various cutaneous, neurological, and visceral manifestations. We describe a series of 3 cases of female patients in the third decade of life with abdominal pain and a wide range of clinical manifestations and short and long-term complications. Our review contributes to the early recognition of these diseases to establish early specific managements to impact on irreversible outcomes.

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Introducción: El liquen plano (LP) es una enfermedad inflamatoria crónica, de etiología desconocida, que puede afectar la piel, los anexos y las mucosas. Objetivo: Describir las características epidemiológicas y clínicas y el tratamiento realizado en los pacientes con diagnóstico clínico e histopatológico de LP, valorados en el Servicio de Dermatología del Hospital de Pediatría "Prof. Dr. Juan P. Garrahan" en los últimos 33 años. Diseño:Estudio descriptivo, retrospectivo y transversal. Materiales y métodos: Se revisaron las historias clínicas de los pacientes con diagnóstico histopatológico de LP atendidos en el Hospital desde agosto de 1987 hasta febrero de 2020 y se analizaron las variables epidemiológicas, clínicas y de tratamiento. Resultados: Se incluyeron datos de 26 pacientes, 18 de los cuales eran varones (69,3%). La media de edad en el momento del diagnóstico fue de 9,6 años. Presentaron la variedad de LP clásico 24 pacienes (93%). El compromiso ungueal y de la mucosa bucal se observó en 2 pacientes (7,7%). Ninguno tuvo asociación a fármacos ni a infecciones. En cuanto al tratamiento recibido, 4 pacientes (15,4%) tuvieron pérdida del seguimiento; 13 (50%) realizaron tratamiento tópico; 6 (23%), tratamiento sistémico y 3 (11,5%), tratamiento combinado. El promedio de seguimiento fue de 6 meses y se obtuvo una respuesta favorable en el 68% de los pacientes. Conclusiones: El LP es una patología de baja frecuencia en la infancia. No se encontró asociación a medicamentos ni a enfermedades infecciosas como se describe en la población adulta. Su curso es crónico y, en los pacientes que tuvieron un adecuado seguimiento, se obtuvo una buena respuesta al tratamiento indicado.

Introduction: Lichen planus (LP) is a chronic, inflammatory disease of unknown etiology, which can affect skin, adnexal tissues and mucosa. Objective: to describe the clinical, epidemiological characteristics and the treatment received by patients with clinical and histopathological diagnosis of LP, seen at the Dermatology Service of Pediatric Hospital "Prof. Dr. Juan P. Garrahan" in the last 33 years. Design: Descriptive, retrospective and cross-sectional study. Materials y methods: We reviewed the clinical history of patients with histopathological diagnosis of LP that were treated at our hospital from August 1987 to February 2020 and analysed the epidemiological, clinical and treatment variables. Results: Data of 26 patients were included in this study 18 of which are males (69.3%). The mean age of diagnosis was 9.6 years. 24 patients (93%) presented the classical type of LP. Ungual and mucosal compromise were observed in 2 patients (7.7%) and none had associations with infections or drugs. Regarding treatment, 4 patients (15.4%) did not continue follow-ups, 13 patients (50%) had topical treatment, 6 (23%) had systemic treatment and 3 patients (11.5%) received combined treatment. The average follow-up time was 6 months and 68% of patients had favorable outcome. Conclusions: LP is an uncommon pathology in children. During this study we did not find any etiologic relations with use of drugs or infectious diseases as is described in de adult population. Its course is chronic and in the patients that had a proper follow-up a good response to the indicated treatment was obtained.

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PURPOSE: To evaluate whether a mathematical tool that predicts severe retinopathy of prematurity (ROP) using clinical parameters at 6 weeks of life (ROPScore calculator smartphone application; PABEX Corporation) can be useful to predict severe ROP in a population of premature infants in Argentina. METHODS: In this retrospective study, data from the clinical records of all premature infants examined between 2012 and 2018 in the ophthalmology department of a public third-level hospital in Córdoba, Argentina, were obtained. ROPScore screening was applied using a Microsoft Excel spreadsheet (Microsoft Corporation). The sensitivity, specificity, and positive (PPV) and negative (NPV) predictive values of the algorithm were analyzed. RESULTS: Between 2012 and 2018, a total of 2,894 pre-term infants were examined and 411 met the inclusion criteria, of whom 34% (n = 139) presented some form of ROP and 6% (n = 25) developed severe forms that required treatment. The sensitivity of the algorithm for any ROP and severe ROP was 100%. The PPV and NPV were 35.64% and 100%, respectively, for any ROP and 9.88% and 100% for severe ROP. CONCLUSIONS: One-time only calculation of the ROPScore algorithm could identify severe cases after validation, reducing the number of screened infants by 38% in infants with a birth weight of 1,500 g or less or a gestational age of 32 weeks or younger. [J Pediatr Ophthalmol Strabismus. 2021;58(1):55-61.].

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Porphyrias are inherited metabolic disorders caused by enzymatic deficiencies of HEM group biosynthesis. Most common in childhood at the third and fourth decade of life. They are characterized by increased levels of porphyrins, and various cutaneous, neurological, and visceral manifestations. We describe a series of 3 cases of female patients in the third decade of life with abdominal pain and a wide range of clinical manifestations and short and long-term complications. Our review contributes to the early recognition of these diseases to establish early specific managements to impact on irreversible outcomes.

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El pilomatrixoma es un tumor benigno cutáneo, de frecuente aparición en los niños, que se origina en las células de la matriz del folículo piloso. Se presenta como un nódulo o tumor, aproximadamente, de 0,5 a 3 cm de tamaño, de consistencia cálcica, de bordes facetados, con piel suprayacente eritematoazulada. En ocasiones, la piel puede atrofiarse, dar la apariencia de una ampolla e, incluso, extruir material cálcico. El diagnóstico se sospecha por la presentación clínica característica y los estudios de imágenes complementarios. El diagnóstico definitivo es por los hallazgos histológicos. La extirpación quirúrgica es el tratamiento de elección. Se presentan los datos epidemiológicos, clínicos, estudios complementarios y técnicas quirúrgicas empleadas de 149 pilomatrixomas resecados en 137 pacientes tratados en los Servicios de Dermatología de los hospitales de pediatría Dr. Pedro de Elizalde y Prof. Dr. Juan P. Garrahan.

A pilomatrixoma is a benign skin tumor common in children, which develops from the matrix cells of hair follicles. It presents as a nodule or tumor of approximately 0.5-3 cm in size, with calcium-like consistency, faceted edges, and blue erythematous overlying skin. Sometimes, the skin may atrophy and look like a blister or even extrude calcium. Diagnosis is suspected based on the typical clinical presentation and supplementary imaging tests. The definite diagnosis is made according to histological findings. Surgical removal is the treatment of choice. Here we describe the epidemiological and clinical data, supplementary tests, and surgical techniques in relation to 149 pilomatrixomas resected in 137 patients treated at the Departments of Dermatology of two children's hospitals: Dr. Pedro de Elizalde and Prof. Dr. Juan P. Garrahan.

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A pilomatrixoma is a benign skin tumor common in children, which develops from the matrix cells of hair follicles. It presents as a nodule or tumor of approximately 0.5-3 cm in size, with calcium-like consistency, faceted edges, and blue erythematous overlying skin. Sometimes, the skin may atrophy and look like a blister or even extrude calcium. Diagnosis is suspected based on the typical clinical presentation and supplementary imaging tests. The definite diagnosis is made according to histological findings. Surgical removal is the treatment of choice. Here we describe the epidemiological and clinical data, supplementary tests, and surgical techniques in relation to 149 pilomatrixomas resected in 137 patients treated at the Departments of Dermatology of two children's hospitals: Dr. Pedro de Elizalde and Prof. Dr. Juan P. Garrahan.

El pilomatrixoma es un tumor benigno cutáneo, de frecuente aparición en los niños, que se origina en las células de la matriz del folículo piloso. Se presenta como un nódulo o tumor, aproximadamente, de 0,5 a 3 cm de tamaño, de consistencia cálcica, de bordes facetados, con piel suprayacente eritematoazulada. En ocasiones, la piel puede atrofiarse, dar la apariencia de una ampolla e, incluso, extruir material cálcico. El diagnóstico se sospecha por la presentación clínica característica y los estudios de imágenes complementarios. El diagnóstico definitivo es por los hallazgos histológicos. La extirpación quirúrgica es el tratamiento de elección. Se presentan los datos epidemiológicos, clínicos, estudios complementarios y técnicas quirúrgicas empleadas de 149 pilomatrixomas resecados en 137 pacientes tratados en los Servicios de Dermatología de los hospitales de pediatría Dr. Pedro de Elizalde y Prof. Dr. Juan P. Garrahan.

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La rosácea es una dermatosis crónica que se manifiesta clínicamente con eritema, telangiectasias, pápulas y pústulas en la parte central de la cara. Afecta, sobre todo, a los adultos y, en raras ocasiones, a los niños. La rosácea puede también presentar afección ocular y preceder, aparecer en forma simultánea o con posterioridad a las lesiones cutáneas; es mayor el riesgo de complicaciones oculares en los niños. La baja prevalencia de esta patología en la infancia origina demoras en el diagnóstico. Se presenta a un paciente de 1 año de edad con rosácea ocular y cutánea que desarrolló opacidades corneales con compromiso de la agudeza visual. Se destaca la importancia del diagnóstico y del tratamiento precoz para evitar el desarrollo de secuelas.

Rosacea is a chronic skin disease characterized by erythema, telangiectasia, papules and pustules in the central facial region. It most often affects adults and is rare in children. Rosacea can also present ocular involvement. Symptoms can precede cutaneous findings, appear simultaneously or after them, with a higher risk of ocular complications in children. Because of low prevalence of rosacea in childhood, the diagnosis is frequently delayed. We report a 1-year-old boy with ocular and cutaneous rosacea who developed corneal opacities and visual impairment. Early diagnosis and treatment is considerable to avoid sequels.

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Rosacea is a chronic skin disease characterized by erythema, telangiectasia, papules and pustules in the central facial region. It most often affects adults and is rare in children. Rosacea can also present ocular involvement. Symptoms can precede cutaneous findings, appear simultaneously or after them, with a higher risk of ocular complications in children. Because of low prevalence of rosacea in childhood, the diagnosis is frequently delayed. We report a 1-year-old boy with ocular and cutaneous rosacea who developed corneal opacities and visual impairment. Early diagnosis and treatment is considerable to avoid sequels.

La rosácea es una dermatosis crónica que se manifiesta clínicamente con eritema, telangiectasias, pápulas y pústulas en la parte central de la cara. Afecta, sobre todo, a los adultos y, en raras ocasiones, a los niños. La rosácea puede también presentar afección ocular y preceder, aparecer en forma simultánea o con posterioridad a las lesiones cutáneas; es mayor el riesgo de complicaciones oculares en los niños. La baja prevalencia de esta patología en la infancia origina demoras en el diagnóstico. Se presenta a un paciente de 1 año de edad con rosácea ocular y cutánea que desarrolló opacidades corneales con compromiso de la agudeza visual. Se destaca la importancia del diagnóstico y del tratamiento precoz para evitar el desarrollo de secuelas.

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