Tratamiento combinado de cicatrices de larga evolución: Resultado estético y reparador en una adolescente / Combined treatment of old scars: Aesthetic and reparative results in an adolescent

Las cicatrices extensas son lesiones que condicionan la vida de los pacientes que las padecen, produciendo retracción social o como en este caso condicionando la vestimenta de una joven adolescente de 15 años. Se realiza el tratamiento de cicatrices postquemadura que padece desde los 2 meses de edad, combinando diversos métodos como peeling, mesoterapia, dermoabrasión y ultrasonido, obteniendo como resultado la mejoría clínica, estética y psicológica, brindándole una calidad de vida superior.

Extensive scars modify the patient's life. Significant scarring causes functional and cosmetic deformities, discomfort and psychological stress. We report a teenager, who suffered from burning when she was two months old. She has been under scar treatment combining several methods such as peeling, mesotherapy, manual dermoabrasion and ultrasound. Up to now, we improved the patient's life quality, obtaining a clinical improvement, such as aesthetic and psychological one.

Juvenile hyaline fibromatosis.

Juvenile hyaline fibromatosis (JHF) is a rare autosomal recessive disease with onset in infancy or early childhood. It is characterized by papulonodular skin lesions, soft tissue masses, gingival hypertrophy, and flexion contractures of the large joints. The light and electron microscopic features are very distinctive. Here we report an 8-month-old boy with characteristic stiffness of the knees and elbows and pink confluent papules on the paranasal folds, and periauricular and perianal regions. He also had hard nodules all over the scalp and around the mouth, and severe gingival hypertrophy. Histologic and ultrastructural features were typical of JHF. Clinical features, pathology, and physiology are discussed.

Pólipos umbilicales y otras anomalías del conducto onfalomesentérico / Umbilical polyps and other abnormalities of the omphalomesenteric duct

El conducto onfalomesentérico es una estructura tubular que une el intestino primitivo con el saco vitelino del embrión. Normalmente esta estructura desaparece obliterándose completamente al final de la 5º o 6º semana de vida intrauterina, pero en ocasiones puede persistir ocasionando diferentes anomalías como fístulas, quistes vitelinos o pólipos umbilicales. Los pólipos son lesiones tumorales rojo brillante que se observan en la cicatriz umbilical tras la caída del cordón.Se presentan 10 casos de anomalías del cierre del conducto onfalomesentérico, de los cuales 7 fueron varones y 3 mujeres. 9 pacientes presentaron pólipos umbilicales, 2 de ellos con fístulas enterocutáneas, y una paciente un quiste vitelino. Las edades de consulta fueron desde los 4 días de vida hasta los 6 años de edad. En 8 pacientes se realizaron estudios histopatológicos, que evidenciaron mucosa gastrointestinal en contacto con epidermis, en 2 casos se realizaron estudios por imágenes contrastadas, encontrándose fístulas enterocutáneas, y en uno fue necesaria una laparotomía exploradora para llegar al diagnóstico. Es importante conocer estas anomalías umbilicales ya que por su embriogénesis, pueden asociarse con alguna de las otras alteraciones derivadas de la falla del cierre del conducto onfalomesentérico, las cuales pueden ocasionar consecuencias graves para el paciente, de no realizarse el diagnóstico oportuno y el tratamiento correcto (AU)

Phacomatosis pigmentokeratotica: another epidermal nevus syndrome and a distinctive type of twin spotting.

The name epidermal nevus syndrome could be applied to a group of clinically and histopathologically different entities as has been pointed out by Happle. Phacomatosis pigmentokeratotica is a further type of epidermal nevus syndrome distinguished by the presence of a sebaceous nevus and a contralateral speckled lentiginous nevus of the papular type, associated with skeletal or neurological abnormalities. Three new cases of this recently delineated syndrome are presented. A common origin may account for the temporal and spatial relationship between the epidermal and the speckled lentiginous nevus. The concept of melanocytic-epidermal twin spotting similar to the interpretation of vascular twin spotting could explain the pathogenesis of this entity.

Congenital self-healing histiocytosis (Hashimoto-Pritzker).

BACKGROUND: Congenital self-healing Langerhans cell histiocytosis (CSHLCH) is a rare condition, initially seen at birth or in the neonatal period, with generalized papules, vesicles, or nodules. Affected infants are otherwise well and the skin lesions tend to involute spontaneously within weeks to months. METHODS: Twelve patients with CSHLCH were seen from 1989 to 1998. RESULTS: Eight patients were girls and four were boys and all presented with lesions at birth which disappeared 1-3 months later. The lesions consisted of numerous brownish-red papules, papulovesicles, crusts, and nodules distributed on the face, limbs, palms, and soles. Two patients had oral mucosal lesions, and one had ulcerated lesions that evolved leaving hypochromic macules. Light microscopy showed a histiocytic infiltrate in the papillary dermis with epidermotrophism. Two cases were studied by electron microscopy: the Langerhans cells showed Birbeck granules and laminated corpus in their cytoplasm. Immunomarking with S100 protein was performed in all 12 patients and was positive. CD1 was also tested in four cases and was positive. CONCLUSIONS: Because CSHLCH is a rare condition, we emphasize that, although it is usually a benign, self-limited entity, careful evaluation for systemic disease must be performed and long-term follow-up must be carried out to detect evidence of relapse or progression of the disease; this is essential when treating these patients.

Eosinophilic pustular folliculitis in infancy: report of two new cases.

Eosinophilic pustular folliculitis (EPF) is a cutaneous inflammatory follicular disorder of unknown etiology. The diagnosis is made on the basis of clinical and histopathologic features. We describe two patients who had recurrent episodes of pruritic follicular papular and pustular lesions on the face, extremities, and trunk. The eruptions lasted for 1 month with intermittent remissions. Laboratory tests disclosed no infectious or parasitic etiology in patient 2. In patient 1 we isolated methicillin-resistant Staphylococcus aureus in a blood culture. He had sepsis with lung and liver involvement. EPF is a self-limited dermatosis. On occasion, skin lesions may become superinfected, resulting in localized pyoderma or rarely systemic infection (sepsis). Histologically both of our patients showed a moderate mixed inflammatory infiltrate with numerous eosinophils centered around hair follicles. Their lesions responded well to topical corticosteroids.

Lymphedema as a postulated cause of cutis verticis gyrata in Turner syndrome.

Unusual skin lesions were present at birth in four infants with Turner syndrome. The skin changes in these patients appear to have resulted either from in utero entrapment or pinching of edematous skin or from redundant skin remaining after in utero resolution of lymphedema. Distention by lymphedema is thought to cause several of the phenotypic characteristics seen in patients with Turner syndrome, including nuchal webbing and nail changes. In three of these patients the clinical appearance of the skin changes was similar to cutis verticis gyrata, marked by fixed thickened plaques in folds.

Eritema pernio en dos gemelas de ocho meses de vida / Chillblains in two tween sisters eigth months old

Se comunican 2 pacientes gemelas, de ocho meses de vida, que presentan lesiones maculares eritematovioláceas localizadas en el dorso y regiones laterales de los pies, con edema de los dedos. Se realizan los estudios histopatológicos que son compatibles con eritema pernio, entidad que se diagnostica con poca frecuencia en lactantes

Eritema pernio en dos gemelas de ocho meses de vida / Chillblains in two tween sisters eigth months old

Se comunican 2 pacientes gemelas, de ocho meses de vida, que presentan lesiones maculares eritematovioláceas localizadas en el dorso y regiones laterales de los pies, con edema de los dedos. Se realizan los estudios histopatológicos que son compatibles con eritema pernio, entidad que se diagnostica con poca frecuencia en lactantes (AU)