Idiopathic aseptic facial granuloma: A retrospective study of 43 cases.

BACKGROUND: Idiopathic aseptic facial granuloma (IAFG) is an underrecognized pediatric skin disease, currently considered within the spectrum of rosacea. It usually manifests as a solitary, reddish, asymptomatic nodule on the cheek that resolves spontaneously. METHODS: Retrospective and descriptive observational study of 43 pediatric patients with a clinical diagnosis of IAFG, followed between 2004 and 2022, at two general hospitals in Argentina. RESULTS: IAFG predominated in girls (65%) and the average age of onset was about 6 years. A single asymptomatic nodule was seen in 79% of patients. The most common localization was the cheek (58%) followed by lower eyelids (41%). Family history of rosacea was present in 16% of patients. A concomitant diagnosis of rosacea and periorificial dermatitis was made in 14% and 9% of our population, respectively. Past or present history of chalazia was detected in 42% of the children. IAFG diagnosis was mainly clinical (88% of cases). Oral antibiotics were the most common indicated treatment (84%). Complete healing was achieved by the majority, but 18% of those with eyelid compromise healed with scars. CONCLUSIONS: IAFG is a benign pediatric condition that physicians should recognize in order to manage correctly. We herein refer to a particular morphologic aspect of IAFG lesions affecting the lower eyelids, where nodules adopt a linear distribution and have a higher probability of involute leaving a scar. Also, we consider that the concomitant findings of rosacea, periorificial dermatitis and chalazia in our patients, reinforce the consideration of IAFG within the spectrum of rosacea.

Happle-Tinschert syndrome variable phenotype as part of the mosaic hedgehog spectrum: Report of three cases.

Happle-Tinschert syndrome is a rare genodermatosis caused by a postzygotic mutation in SMO gene. The most recognized clinical findings include segmentally arranged basaloid follicular hamartomas, nevoid hypertrichosis, linear atrophoderma, and hypopigmentation or hyperpigmentation following Blaschko lines associated with osseous, dental, and cerebral alterations. We report three additional cases, two of which lacked the pathognomonic basaloid follicular hamartomas, with genetic confirmation and detailed clinical characterization and describe new cutaneous features of this infrequent syndrome.

Erythrosis Pigmentosa Peribuccalis in an Adolescent: Dermoscopic Description and Management.

Erythrosis pigmentosa peribuccalis is an infrequent condition, with fewer than 35 cases reported in the literature, that affects mainly women. It presents as small papules that form a hyperpigmented plaque around the mouth and nose. Little is known about the etiology of this condition, its dermoscopic characteristics have not been described, and no effective treatment has been reported. We report a 15-year-old girl with this rare dermatosis who was successfully managed with daily calcipotriol, weekly topical ivermectin, and strict photoprotection. We also describe for the first time the dermoscopic findings of this entity.

Nevus comedonicus: a case series.

Twelve previously unreported cases of nevus comedonicus are presented. Characteristic closely grouped dilated follicular openings with horny plugs that mimic comedones led to the diagnosis. One patient had nevus comedonicus syndrome and there were cases with atypical locations and unusual complications of this condition. We also highlight clinical associations and therapeutic options.

[Recommendations for psoriasis treatment in Pediatrics]. / Recomendaciones para el tratamiento de la psoriasis en Pediatría.

Introduction: in one third of patients with psoriasis, symptoms start during childhood and adolescence, with a strong emotional and psychosocial impact. Objective: to develop a guideline for the systemic treatment of psoriasis in pediatric patients by means of recommendations based on the best available evidence. Materials and methods: Sources: articles indexed in PubMed, Epistemonikos, Google Scholar, Cochrane Library and Scielo, published between January 2010 and May 2022, in English, Spanish and Portuguese. Study selection: evidence-based clinical practice guidelines, systematic reviews, meta-analyses, randomized controlled studies, observational studies (case-control, cohort studies, real-life registries) and evaluations of biosimilar drugs in patients up to and including 17 years of age were considered. The keywords "psoriasis" and "treatment" were used in all three languages. Data extraction: the literature was evaluated using Grading of Recommendations Assessment, Development and Evaluation (GRADE) recommendations. Data synthesis: evidence tables were developed and analyzed by the expert committee. The questions for the development of recommendations were based on the PICO system (population, intervention, comparison, outcome). Results: A total of 8 recommendations and 7 points of good practice were developed. The direction and strength of the recommendations were expressed according to the GRADE system. Conclusions: the final decision on a specific therapy should be based on the best opinion of the treating physician, the individual characteristics, and the values and preferences of the patients and their caregivers.

Introducción: un tercio de los pacientes con psoriasis comienzan con sus síntomas en la niñez y la adolescencia, con fuerte impacto emocional y psicosocial. Objetivo: elaborar una guía de tratamiento sistémico de la psoriasis en pacientes pediátricos mediante recomendaciones fundamentadas en la mejor evidencia disponible. Materiales y métodos: Fuentes: artículos indexados en PubMed, Epistemonikos, Google Académico, Cochrane Library y Scielo, publicados entre enero de 2010 y mayo de 2022, en inglés, castellano y portugués. Selección de estudios: se consideraron guías de práctica clínica basadas en la evidencia, revisiones sistemáticas, metanálisis, estudios controlados y aleatorizados, estudios observacionales (casos y controles, estudios de cohortes, registros de la vida real) y evaluaciones de medicamentos biosimilares en pacientes de hasta 17 años de edad inclusive. Se utilizaron las palabras clave "psoriasis" y "tratamiento" en los tres idiomas. Extracción de datos: la bibliografía fue evaluada mediante las recomendaciones del sistema Grading of Recommendations Assessment, Development and Evaluation (GRADE). Síntesis de datos: elaboración de tablas de evidencia que fueron analizadas por el comité de expertos. Las preguntas para el desarrollo de recomendaciones se fundamentaron en el sistema PICO (población, intervención, comparación, outcome [desenlace]). Resultados: se elaboraron un total de 8 recomendaciones y 7 puntos de buena práctica. La dirección y fuerza de las recomendaciones se expresaron de acuerdo con lo sugerido por el sistema GRADE. Conclusiones: la decisión final de una terapia específica se fundamentará en la mejor opinión del médico tratante, las características individuales, y los valores y preferencias de los pacientes y sus cuidadores.

Nevus trichilemmocysticus: report of a new case of a recently recognized entity.

A new type of organoid epidermal nevus, characterized by the presence of multiple trichilemmal cysts disposed in a linear arrangement, was recently recognized and named nevus trichilemmocysticus. We report a 10-year-old girl with a congenital linear dermatosis affecting the head, neck, trunk, limbs, palms, and soles. Initially, the lesions that followed the lines of Blaschko consisted of yellowish plaques covered by verrucous papules and filiform hyperkeratoses. Additionally, since the age of 6 years, numerous cyst-like structures developed on the linear lesions involving the limbs. On histopathological examination, all of the excised cystic lesions were found to be trichilemmal cysts. Hence, we herein describe a new case of nevus trichilemmocysticus.

Neonatal kerion Celsi: report of three cases.

Tinea capitis is a fungal infection caused by dermatophytes, frequent in children but uncommon in the neonatal period. Kerion Celsi is the inflammatory manifestation of tinea capitis secondary to host immunologic responses and its occurrence in newborns is extremely infrequent. We describe three neonates with the diagnosis of kerion Celsi. The isolated dermatophytes were Trichophyton mentagrophytes var. mentagrophytes in two patients and Trichophyton rubrum in the third. Both patients with T. mentagrophytes referred an indirect contact with rabbits and were successfully treated with systemic antifungal (griseofulvin and fluconazole). The patient with T. rubrum had a father with a tinea manuum and both received just topical antimycotic treatment.

Infantile myofibromatosis: report of nine patients.

Infantile myofibromatosis is a rare fibrous tumor of infancy that can be solitary or multiple. Although most of the cases are limited to the skin, in some instances systemic involvement can be present. Solitary tumors limited to the skin usually present a good prognosis with spontaneous regression. We performed a retrospective observational review of the clinical and pathologic characteristics of nine patients diagnosed as having infantile myofibromatosis, followed during a 10-year period in a Pediatric Dermatology Department.

Subcutaneous midline suprasternal mass in a ten-month-old girl.

A ten-month-old girl presented to our clinic with a progressively enlarging congenital mass at the midline suprasternal location. Histopathologic examination revealed a benign cyst with stratified squamous keratinized epithelium with numerous eccrine glands in the sinus wall. The diagnosis was compatible with dermoid cyst. Dermoid cysts of the trunk are rare lesions, but a midline location is characteristic for these congenital masses. A dermoid cyst should be high on the list of differential diagnoses given a unilocular midline cyst in a neonate.

Eccrine angiomatous hamartoma: report of five congenital cases.

Eccrine angiomatous hamartoma is a rare entity characterized histologically by the combination of proliferative eccrine and vascular elements. It generally arises before puberty, as solitary or multiple lesions, with a heterogeneous clinical appearance, affecting predominantly the distal extremities, with or without associated pain or hyperhidrosis. It may require surgical treatment due to cosmetic concern, progressive enlargement or the presence of pain or excessive hyperhidrosis. We report five congenital cases of eccrine angiomatous hamartoma, emphasizing a clinically uncommon tumor-like appearance, with numerous telangiectasias on their surfaces resembling vascular lesions, in two of them.

Recomendaciones para el tratamiento de la psoriasis en pediatría / [Recommendations for psoriasis treatment in Pediatrics]

Un tercio de los pacientes con psoriasis comienzan con sus síntomas en la niñez y la adolescencia, con fuerte impacto emocional y psicosocial. Elaborar una guía de tratamiento sistémico de la psoriasis en pacientes pediátricos mediante recomendaciones fundamentadas en la mejor evidencia disponible. Fuentes: artículos indexados en PubMed, Epistemonikos, Google Académico, Cochrane Library y Scielo, publicados entre enero de 2010 y mayo de 2022, en inglés, castellano y portugués. Selección de estudios: se consideraron guías de práctica clínica basadas en la evidencia, revisiones sistemáticas, metanálisis, estudios controlados y aleatorizados, estudios observacionales (casos y controles, estudios de cohortes, registros de la vida real) y evaluaciones de medicamentos biosimilares en pacientes de hasta 17 años de edad inclusive. Se utilizaron las palabras clave "psoriasis" y "tratamiento" en los tres idiomas. Extracción de datos: la bibliografía fue evaluada mediante las recomendaciones del sistema Grading of Recommendations Assessment, Development and Evaluation (GRADE). Síntesis de datos: elaboración de tablas de evidencia que fueron analizadas por el comité de expertos. Las preguntas para el desarrollo de recomendaciones se fundamentaron en el sistema PICO (población, intervención, comparación, outcome [desenlace]). Se elaboraron un total de 8 recomendaciones y 7 puntos de buena práctica. La dirección y fuerza de las recomendaciones se expresaron de acuerdo con lo sugerido por el sistema GRADE. la decisión final de una terapia específica se fundamentará en la mejor opinión del médico tratante, las características individuales, y los valores y preferencias de los pacientes y sus cuidadores.

Childhood flexural comedones: a new entity.

BACKGROUND: Comedones are usually found in acne and involve the seborrheic areas of the skin. Disseminated comedones can be found in other skin disorders. Flexural comedones are characterized by double orifices connected by a thin layer of epidermis that reveals the comedo content below it. To the best of our knowledge, flexural comedones have not been previously described as an entity. Our objective was to characterize this disorder. OBSERVATIONS: A cross-sectional descriptive study was performed from April 2004 to July 2006. We included 40 pediatric and adolescent patients with flexural comedones; 21 were female (52%), and 19 were male (48%) (mean age, 6.2 years). In 29 cases the lesions were single (72%) and in 32 cases (80%) unilateral. The lesions were located in the axilla in 88% of the patients. We performed biopsies of skin samples in 6 cases. CONCLUSIONS: To our knowledge, flexural comedones have not been previously described as an entity, and we felt that they deserved attention owing to the relative frequency of cases in our clinical practice. Because of its clinical appearance, flexural localization, and age distribution, we named this disorder childhood flexural comedones. Further investigation and follow-up of a larger number of patients is needed.

Kaposi's varicelliform eruption: A case series.

Kaposi's varicelliform eruption is a rare and potentially fatal viral infection caused mainly by reactivation of herpes simplex virus. It concomitantly occurs with pre-existing skin conditions, mostly atopic dermatitis, so it is predominately found in children. We present a case series that includes four adults, familial cases, and previously healthy patients. We also highlight clinical features, associations and therapeutic options.

Capillary malformation-arteriovenous malformation: a clinical review of 45 patients.

BACKGROUND: Capillary malformation-arteriovenous malformation (CM-AVM) is a recently described autosomal dominant disorder that results from mutations in RASA1. It has been initially described as multiple CMs affecting several members of the same family, associated with fast-flow malformations in at least one family member. OBJECTIVE: To report and analyze clinical data on 45 patients with CM-AVM assessed at the Department of Pediatric Dermatology, Ramos Mejía Hospital (Buenos Aires, Argentina). METHOD: Retrospective clinical review of all the patients clinically diagnosed as having CM-AVM over a period of eight years. RESULTS: Forty-five patients were recorded (24 females and 21 males). The age ranged from one month to 44 years. In 36 patients, the stains were congenital; progressive acquired lesions were observed in 39. Family history was positive in 32 subjects. Well defined, round to oval, pink-purple or reddish-brown macules were found in all the patients; pinpoint red lesions with a pale halo were found in nine cases. The macules were warmer than normal skin in 15 cases and surrounded by a white halo in 26 cases. Three subjects presented associated overgrowth, lymphatic malformation was present in one case, retinal vascular lesion in one patient, and isolated port wine stain in two cases. Three patients also had infantile hemangioma. We had no cases of fast-flow vascular malformation or combined vascular syndromes. CONCLUSIONS: CM-AVM is a heterogeneous disorder with phenotypic variability, from fast-flow malformation, limb enlargement, or Parkes Weber syndrome to multiple CMs without internal involvement.

[Rapidly involuting congenital hemangiomas: twenty five case series]. / Hemangiomas congénitos rápidamente involutivos: serie de 25 casos.

Rapidly involuting congenital hemangiomas are very rare vascular tumours, that are characterized for being completely developed at birth and for involuting in a short period of time after birth. We describe a case series of 25 patients with rapidly involuting congenital hemangiomas. Twelve patients were male and 13 female; they were all born at term. Lesions were small in 17 cases and big in 8. No patient needed active intervention and all lesions showed a rapid initial involution.

[Onychomadesis associated with mouth, hand and foot disease]. / Onicomadesis asociada a la enfermedad de boca, mano y pie.

Onychomadesis is the spontaneous, complete shedding of the nail from its proximal side, without pain or inflammation, following nail matrix arrest. This disorder is uncommon in children and it can occur in fingernails, toenails or both. It may be secondary to systemic disorders, Kawasaki disease, bullous dermatoses, drugs, paronychia, stress and radiotherapy. Since 2000, Hand, Foot, and Mouth Disease (HFMD) has been described as a cause of onychomadesis, and has been associated with outbreaks of this condition in different regions of the world. HFMD is an infection characterized by vesicular and erosive stomatitis in combination with a vesicular eruption in palms and soles. It occurs in small children during summer and autumn months, and it is caused by coxsackie virus. We present a study that reflects the current situation of onychomadesis in Argentinian children and shows a strong association between this disorder and HFMD, suggesting that onychomadesis is a new manifestation of a previously known disease.

[Contact Paederus dermatitis in a 12 years old boy]. / Dermatitis de contacto por Paederus en un niño de 12 años.

Paederus dermatitis is a peculiar contact dermatitis caused by pederine contained in the hemolimph of a small insect of the genus Paederus. It is characterized by vesico-pustulous eruption arising over an erythematous background, of sudden onset, over exposed areas of the body, and the history of outdoor activities. Diagnosis is based on the typical clinical features and the epidemiological context. The aim of this publication is to report a case and evaluate the epidemiological and clinical characteristics of Paederus dermatitis.

[Fabry disease]. / Doença de Fabry.

Fabry disease is an uncommon, X-linked lysosomal storage disorder, caused by partial or complete deficiency of the enzyme a-galactosidase A. The defect leads to accumulation of uncleaved globotriaosylceramide on the vascular endothelium and visceral tissues, being the skin, heart, kidneys and central nervous system the most affected organs. We performed review of the literature related to the disease and emphasized that early recognition of angiokeratomas and hypohidrosis are key diagnostic signs of this serious disease. We also addressed the need of multidisciplinary assessment of these patients.

[Subungual exostosis in a 14 years old boy]. / Exostosis subungueal en un adolescente de 14 años.

Subungual exostosis is a benign bony outgrowth surrounded by a fibrocartilaginous capsule that mainly affects hallux terminal phalanx with subungual or periungular localization, and that clinically determines variable degree of nail plate or periungular soft tissues alterations, respectively, It is uncommon in childhood and more frequent in females. Clinical suspicion is confirmed by the radiographic finding of a distinct trabecular bone growth arising from the dorsal aspect of the phalanx of the affected finger, on the lateral incidence. Curettage or surgical excision is the treatment of choice. We report a case of subungual exostosis located at the hallux in a 14-year-old boy suffering from subungual exostosis of the hallux that presented the characteristic triad of this entity: subungular tumor with nail plate deformity, digital pain and radiologic typical findings.

Hemangiomas congénitos rápidamente involutivos: serie de 25 casos / Rapidly involuting congenital hemangiomas: twenty five case series

Los hemangiomas congénitos rápidamente involutivos son tumores vasculares muy poco frecuentes, que se caracterizan por estar completamente desarrollados al nacer (congénitos) y por tener una involución espontánea en un corto período (rápidamente involutivos). Describimos una serie de 25 casos de hemangiomas congénitos rápidamente involutivos, de los cuales 12 eran del sexo masculino y 13, femenino. Todos los pacientes eran nacidos a término, con peso adecuado para la edad gestacional. En 17, las lesiones fueron de pequeño tamaño y, en 8, de gran tamaño. Ningún paciente requirió tratamiento. En todos ellos, las lesiones mostraron una rápida involución inicial.

Rapidly involuting congenital hemangiomas are very rare vascular tumours, that are characterized for being completely developed at birth and for involuting in a short period of time after birth. We describe a case series of 25 patients with rapidly involuting congenital hemangiomas. Twelve patients were male and 13 female; they were all born at term. Lesions were small in 17 cases and big in 8. No patient needed active intervention and all lesions showed a rapid initial involution.