Netherton syndrome in a 1-year-old Filipino female

@#Netherton syndrome (NS) is an autosomal recessive genodermatosis characterized by cutaneous and systemic complications (recurrent infections, dehydration, and sepsis). This highlights the urgency of making an accurate diagnosis, especially in infants and children. However, it is important to note that the recognition of NS is usually delayed due to its rarity and similarity to cutaneous disorders with atopiform, erythrodermic, and ichthyosiform presentations. We report a case of a 1-year-old female who was previously diagnosed with a case of infantile psoriasis and was subsequently treated with topical emollients. However, after months of surveillance, the patient developed feeding problems, failure to thrive, recurrent diarrhea, upper respiratory tract, and gastrointestinal infection, leading to repeated hospitalizations. The patient then underwent further clinical examinations and laboratory analysis, which revealed abnormal hair shaft findings, elevated immunoglobulin (Ig) E levels, and normal chromosomal analysis. Multispecialty referrals with other services were done to address the current problems and ensure holistic care for the patient. On her last admission, the patient was given three doses of intravenous Ig therapy with noted improvement in lesion presentation without any systemic symptoms.

Treatment response as a diagnostic feature in zinc deficiency-associated dermatitis in a three-month-old Filipino male: A case report

Introduction@#Zinc deficiency is of high magnitude in developing countries such as the Philippines. Zinc deficiency dermatitis is recognized through characteristic cutaneous presentation supported by diagnostic workups which may not be feasible or practical in low-resource settings.@*Case report@#A three-month-old Filipino male was brought in for erosions of three (3) weeks duration that were unrespon- sive to topical and systemic antimicrobial treatment. On examination, he had multiple erythematous erosions with yellowish to brownish, crusted borders with predilection on the face, inguinal and gluteal areas, flexures of the extremities, and digits. Workup revealed normal zinc levels, decreased alkaline phosphatase, and bacterial growth in cultures. Histopathology revealed intraepidermal vesiculobullous dermatitis. Given the clinicopathologic presentation, a diagnosis of zinc deficiency-associated dermatitis was made. Along with antimicrobials and topical care, oral zinc sulfate with elemental zinc at 3 mg/kg/day was started, with remarkable improvement within three (3) days and near-resolution after eight (8) days of zinc therapy. Zinc supplementation was administered for three (3) months with gradual tapering. The skin remained clear despite the withdrawal of zinc supplemen- tation. Response to treatment supported the impression of zinc deficiency, while sustained skin clearance upon withdrawal verified an acquired etiology.@*Conclusion@#Zinc deficiency-associated dermatitis is more common in areas where costly diagnostic modalities are not readily available. In clinically suspected zinc deficiency, response to treatment can serve as a retrospective diagnostic feature, and sus- tained clearance upon withdrawal may aid in identifying etiology. Trial of therapy may then be considered in optimizing the cost-ef- fective management of zinc deficiency-associated dermatitis.

Childhood bullous pemphigoid: A case report

Introduction@#Bullous pemphigoid (BP) is an acquired autoimmune subepidermal bullous disease characterized by linear depo- sition of IgG and C3 along the basement membrane. It rarely occurs in childhood, especially in adolescence, with only 14 cases identified in literature. Treatment of choice is systemic corticosteroids but other treatment options such as anti-inflammatory antibacterials and methotrexate are available.@*Case report@#A 16-year-old Filipino girl presented with a three-month history of generalized vesicles and bullae. Nikolsky and Asboe-Hansen signs were negative. Histopathology and direct immunofluorescence were consistent with BP. ELISA to BP180 au- toantibody levels was elevated at 135 IU (normal <9 IU). Complete blood count showed leukocytosis with increase in neutrophils. Chest x-ray revealed pulmonary tuberculosis. The patient was given quadruple anti-Koch’s medication (pyrazinamide, rifampi- cin, ethambutol, isoniazid), prednisone, oral erythromycin and topical clobetasol propionate. Complete remission was attained at 10 months and is sustained at the time of writing.@*Conclusion@#To establish a definitive diagnosis and appropriate management, BP requires clinical, histopathologic, and immuno- logical correlation. Childhood BP has good prognosis and rapid treatment response, with rare relapses.

Pediatric dermatoses pattern at a Brazilian reference center

Abstract Objective: The aim of this study was to identify the pattern of pediatric dermatoses of patients evaluated at a dermatologic clinic of a reference center in Brazil and to compare these results to similar surveys conducted in other countries. Methods: A retrospective study was performed of patients up to 18 years old, evaluated at a dermatologic clinic between January 1, 2017 and December 31, 2017. Variables collected for analysis included age, gender, dermatological diagnosis, multidisciplinary follow-up, hospitalization, and complementary exams. Results: A total of 2330 patients were included for analysis, with a mean age of 9.7 years. 295 patients were diagnosed with more than one skin disease, leading to a total of 2668 diagnoses. Skin diseases were organized into categories and inflammatory dermatoses corresponded to the largest group (31.2%), mostly due to atopic dermatitis (18.3%). The other main categories were: genodermatoses (14.2%), infectious diseases (12.6%), adnexal disorders (12.5%), cysts and neoplasms (10.7%), and vascular disorders (7.0%). Fifty-six patients needed to be admitted to the dermatology ward; 25 of them (44.6%) for management of worsening of the skin disease, mainly atopic dermatitis, psoriasis, and drug reactions. There were 885 biopsies performed in 38.0% of the subjects and 751 patients (32.2%) required multidisciplinary care; most of them had some genodermatoses. Conclusions: Dermatologic disorders are very common in the pediatric age group and differ from those in adults, suffering influence from cultural, ethnic, socioeconomic, and environmental factors. Knowing the magnitude and distribution of these dermatoses is important to better plan healthcare policies.

Bullous pemphigoid: can it occur in pediatric age? / Penfigoide ampolloso: ¿puede ocurrir en la edad pediátrica?

El penfigoide ampollar en niños es extremadamen-te infrecuente. La mayoría de los casos ocurre en adultos mayores, resultando fundamental conside-rar que en medicina todo es posible y esta enferme-dad igualmente podría presentarse en niños, por lo que se debe prestar especial atención a sus manifes-taciones clínicas, realizar los exámenes apropiados para descartar diagnósticos diferenciales y de esta forma, iniciar un tratamiento eficaz en el momen-to preciso. A continuación, presentamos un caso clínico de penfigoide ampollar que ocurrió en un paciente pediátrico atendido en nuestro hospital universitario.

Bullous pemphigoid in children is extremely in-frequent. Most of them predominate in elderly persons, resulting fundamental to consider that in medicine everything could be possible, and this disease could occur in children, so we must pay special attention to its clinical manifestations, to take the appropriate exams to rule out differen-tial diagnoses and in this way perform an effective treatment at the precise moment. Following, we present a clinical case of bullous pemphigoid that occurred in a pediatric patient attended at our university hospital.

Epidemiología de dermatosis pediátricas: experiencia de 30 años en Antofagasta, Chile / Epidemiology of pediatric dermatoses: experience of 30 years in Antofagasta, Chile

Introducción: La consulta por dermatología pediátrica es prevalente en dermatología general, por lo que estudiamos su importancia relativa y los motivos de consulta, en un periodo de 30 años en Antofagasta, Chile. Material y método: Se analizó el principal motivo de consulta en los menores de 15 años en una consulta dermatológica privada en 3 décadas, de 1984 a 2013. Se consideró diagnóstico, género, edad, previsión y se estudiaron las diferencias epidemiológicas entre las décadas en estudio. Resultados: El 26,6% de la consulta fue por dermatología pediátrica (15.742 pacientes). Los 10 principales motivos de consulta fueron prurigo insectario, dermatitis atópica, verrugas, escabiosis, impétigo, acné, estigmas atópicos, hemangioma, tiña y nevus melanocítico. Según décadas en estudio, la dermatitis atópica pasó del tercer al primer lugar en el periodo. Asimismo, disminuyeron escabiosis e impétigo y aumentaron acné y nevus melanocítico. Discusión: La dermatitis atópica ha llegado a ser el principal motivo de consulta dermatológica pediátrica en gran parte del mundo, especialmente en países desarrollados. Igualmente, la disminución de enfermedades bacterianas y parasitarias también es propia de países en desarrollo. Conclusiones: El volumen de consulta dermatológica pediátrica es importante, por lo que debe considerarse cuidadosamente en el currículo formativo del dermatólogo. Asimismo, los cambios epidemiológicos demostrados sugieren que nuestro país ha mutado a una epidemiología de país desarrollado.

Introduction: Consultation for pediatric dermatology is prevalent in general dermatology, that's why we studied the relative importance and motives of children's consultation in a period of 30 years in Antofagasta, Chile. Material and method: We analyzed the main motive of consultation in children under 15 years of age at a private dermatological clinic in 3 decades, from 1984 to 2013. Diagnosis, gender, age and social security system were considered and we studied the epidemiological differences between the decades in review. Results: 26,6% of the consultation was for pediatric dermatology (15.742 patients). The 10 main reasons for consultation were papular urticaria, atopic dermatitis, wart, scabies, impetigo, acne, atopic stigma, hemangioma, tinea and melanocytic nevus. According to decades under study, atopic dermatitis went from third to first place in the period. Likewise, scabies and impetigo diminished and acne and melanocytic nevus increased. Discussion: atopic dermatitis has become the main reason for pediatric dermatological consultation largely in the world, especially in developed countries. Similarly, the decline in bacterial and parasitic diseases is characteristic of developing countries. Conclusion: The volume of pediatric dermatological consultation is important, so it should be carefully considered in the training curriculum of the dermatologist. Likewise, the epidemiological changes demonstrated suggest that our country has mutated to a developed country epidemiology. Capsule Summary: Pediatric dermatology consultation is prevalent at a global level and atopic dermatitis is its main reason. In our investigation, both premises are fulfilled, important information that is partly extrapolable to guide public policies in dermatological formation and resource distribution.

Clinical Manifestations of Longitudinal Melanonychia in Childhood / 대한피부과학회지

BACKGROUND: Longitudinal melanonychia is characterized by pigmented bands on the nail plate. It is not unusual to encounter pediatric melanonychia patients in the dermatologic clinic. OBJECTIVE: To assess the clinical manifestations of melanonychia in childhood in Korea, we reviewed the pediatric patient registry of a tertiary referral hospital in Korea. METHODS: Patients under fifteen years of age with longitudinal melanonychia referred between January, 2001, and March, 2015, were enrolled in the study. All patients had clinical as well as photo records. Clinical characteristics including age, sex, location, disease duration, width and color of pigmentation, morphological change over time, underlying disease, and dermoscopic images or biopsy findings were evaluated where available. RESULTS: A total of 92 patients had 158 pigmented nails, with more than one melanonychia identified in 14 patients. The patient sample included 43 male and 49 female patients with an average age of 6.1 years at diagnosis. A follow-up examination was performed for 132 nails in 80 patients with a mean period of 27.8 months and morphological changes were identified in 61 nails. Changes included remission or aggravation of pigmentation. In 48% and 17% of patients, nail plate dystrophy and Hutchinson's sign were observed, respectively. Dermoscopic image analysis was available for 79 nails. A nail biopsy was performed for 8 nails and none showed evidence of malignant melanoma. CONCLUSION: Pediatric longitudinal melanonychia generally presents a benign nature despite overt morphological changes, diffuse pigmentation, or Hutchinson's sign. Characteristically, melanonychia in childhood is frequently accompanied by nail dystrophy. Based on clinical reference of our study, aggressive nail biopsy may not be routinely necessary in pediatric melanonychia.

Alteraciones cutáneas del neonato en dos grupos de población de México / Skin lesions two cohorts of newborns in Mexico City

Introducción. Objetivo: conocer cuáles y qué tan frecuentes son las enfermedades o trastornos cutáneos en 2 grupos representativos del neonato mexicano. Material y métodos. Estudio prospectivo de un millar de neonatos pertenecientes a dos grupos: 500 atendidos en una institución pública (grupo A), y otros 500 en 2 hospitales privados (grupo B) de la Ciudad de México; en un formato diseñado específicamente para este fin, se identificaron y se diagnosticaron todas y cada una de las alteraciones muco-cutáneas presentes en cada sujeto de acuerdo con criterios clínicos, y se registraron para ser analizados por sus frecuencias y poderse comparar estos 2 grupos entre sí y contrastarse con otras series en otros países. Se utilizaron porcentajes y chi-cuadrada como pruebas estadísticas. Resultados. El 100% de los recién nacidos presentó alguna alteración o enfermedad en la piel, como promedio 3 a 6, observándose de 2 tipos: transitorias y permanentes; con claras diferencias entre ambos grupos, y al compararse con otros estudios de otros ámbitos. Las alteraciones transitorias fueron más frecuentes que las permanentes; la descamación fisiológica fue la más común en ambos grupos, otras, como la mancha mongólica, variaron entre ambos grupos, siendo mucho más común en el grupo A. El pólipo fibroepitelial fue la malformación más frecuente en el neonato mexicano. Conclusiones. Las alteraciones de la piel del neonato son, en su mayoría, de tipo transitorio y usualmente no afectan la vida, la función, ni el desarrollo del niño. Las afecciones en el neonato se presentan con una frecuencia absoluta y en nuestro medio varían de acuerdo con las diversas características y tipos de piel del sujeto.

Introduction. Objective: to evaluate the frequency and type of dermatologic lesions in 2 cohorts of newborns in Mexico City. Material and methods. We prospectively studied 1 000 newborns, according to the institution in which they were delivered: 500 in a public institution (group A) and 500 at 2 private hospitals (group B) in Mexico City. A specific data sheet was prepared for this purpose; all identified and diagnosed muco-cutaneous disorders in every child, according to widely accepted clinical criteria were recorded to be analyzed on the basis of their frequency and compared to each other, and also with other series from other countries. Statistical analysis included percentages and chi-square. Results. In this study, 100% of newborns presented an average 3 to 6 skin lesion. The skin lesions fell into 2 categories: transient and permanent. It is also demonstrated that there are very clear differences between our 2 groups; Mongolian patch is the most common transient disease and fibroepithelial polyp is the most frequent permanent disorder. Conclusions. The information obtained in the present paper is new and relevant since there is no previous data about this subject in Mexico. Cutaneous lesions are present in 100% of newborns; they are mostly transient and usually not life-threatening. Newborn skin disorders in our population are present in every child, from 3 to 6 as an average, and vary widely according to ethnic features revealed by skin types.