Guía mexicana para el diagnóstico y el tratamiento del hemangioma infantil.

Infantile hemangioma is a benign vascular tumor, the most common in childhood, whose natural evolution is the disappearance of the lesion in the pediatric age and which has effective and safe treatments that limit its growth and favor its disappearance at younger ages. Infantile hemangioma continues to be a reason for attention to complications, due to erroneous diagnoses, lack of knowledge of the condition, late referral or fear of the effects of the medications used for its treatment. Furthermore, its presence is normalized without taking into account that it can cause uncertainty, anxiety, feelings of guilt and, as a consequence, a significant impact on the quality of life, mainly in the parents or caregivers of the child. The need for a clinical practice guideline in our country arises from the high presentation of late-remitted complications in infantile hemangioma even with the availability of adequate treatments, the continuous evolution of medicine and the appearance of new evidence. Throughout the guide you will find recommendations regarding the diagnosis, treatment and follow-up of patients with infantile hemangioma, taking into account the paraclinical tests that can be performed, topical or systemic management options, as well as adjuvant therapies. For the first time, objective tools for patient follow-up are included in a guide for the management of infantile hemangioma, as well as to help the first contact doctor in timely referral.

El hemangioma infantil es un tumor vascular benigno, el más frecuente de la infancia, cuya evolución natural favorece la desaparición de la lesión en la misma edad pediátrica y que cuenta con tratamientos eficaces y seguros que limitan su crecimiento y favorecen su desaparición a edades más tempranas. Continúa siendo motivo de atención de complicaciones, debido a diagnósticos erróneos, desconocimiento del padecimiento, referencia tardía o temor de los efectos de los fármacos utilizados para su tratamiento. Además, se normaliza su presencia sin tomar en cuenta que puede llegar a causar incertidumbre, ansiedad, sentimientos de culpa y, como consecuencia, importante afectación de la calidad de vida, principalmente en los padres o cuidadores del niño. La necesidad de una guía de práctica clínica en nuestro país surge ante la alta presentación de complicaciones del hemangioma infantil referidas de manera tardía aun con la disponibilidad de tratamientos adecuados, la evolución continua de la medicina y la aparición de nueva evidencia. A lo largo de la guía se encontrarán recomendaciones en relación con el diagnóstico, el tratamiento y el seguimiento de los pacientes con hemangioma infantil, tomando en cuenta los paraclínicos que pueden realizarse, las opciones de manejo tópico o sistémico, y las terapias adyuvantes. Por primera vez se incluyen en una guía para el manejo del hemangioma infantil herramientas objetivas para el seguimiento de los pacientes, así como para ayudar al médico de primer contacto en su referencia oportuna.

Bart syndrome: the congenital localized absence of skin may follow the lines of Blaschko. Report of six cases.

Three cutaneous manifestations are characteristic of Bart syndrome: congenital localized absence of skin (CLAS), mucocutaneous blistering, and nail abnormalities. Six cases of Bart syndrome are herein reported. Localized absence of skin is present at birth, particularly on the anterior aspects of the lower extremities and dorsa of the feet. Physical trauma in utero has been proposed as a mechanism to explain the denuded areas on the limbs. The recurrent, highly similar pattern of the congenital defect in regard to location and clinical appearance in our patients and in most of the reported cases strongly suggests that trauma is too simplistic an explanation. Because of the observed bilateral and symmetric distribution of denuded areas in an S-shaped broad band, their sharply demarcated borders, the involvement of the toe webs, and the frequent similar involvement of the soles, we suggest that congenital localized absence of skin in Bart syndrome may follow the lines of Blaschko.

Elejalde syndrome--a melanolysosomal neurocutaneous syndrome: clinical and morphological findings in 7 patients.

BACKGROUND: Silvery hair and severe dysfunction of the central nervous system (neuroectodermal melanolysosomal disease or Elejalde syndrome) characterize this rare autosomal recessive disease. Main clinical features include silver-leaden hair, bronze skin after sun exposure, and neurologic involvement (seizures, severe hypotonia, and mental retardation). Large granules of melanin unevenly distributed in the hair shaft are observed. Abnormal melanocytes and melanosomes and abnormal inclusion bodies in fibroblasts may be present. Differential diagnosis with Chédiak-Higashi syndrome and Griscelli syndrome must be done. OBSERVATIONS: We studied pediatric patients with silvery hair and profound neurologic dysfunction. Immune impairment was absent. Age of onset of neurologic signs ranged from 1 month to 11 years; the signs included severe muscular hypotonia, ocular alterations, and seizures. Mental retardation since the first months of life was noted in 4 cases. Psychomotor development was normal in 3 cases, but suddenly the patients presented with a regressive neurologic process. Four patients died between 6 months and 3 years after the onset of neurologic dysfunction. One patient showed characteristic ultrastructural findings of Elejalde syndrome. CONCLUSIONS: Elejalde syndrome is different from Chédiak-Higashi and Griscelli syndrome and is characterized by silvery hair and frequent occurrence of fatal neurologic alterations. Psychomotor impairment may have 2 forms of presentation: congenital or infantile. Although Elejalde syndrome and Griscelli syndrome are similar, the possibility that they are 2 different diseases, although probably allelic related, is suggested.

Surgical facial wounds: simple interrupted percutaneous suture versus running intradermal suture.

The purpose of this study is to compare the esthetics of scars resulting from small surgical facial wounds sutured either with simple interrupted percutaneous suture (SIPS) or with running intradermal suture (RIS). 93 small (0.6-1.9 cm) benign, facial new growths, mostly (86%) intradermal nevi were surgically removed. Forty-seven surgical wounds were sutured with SIPS and 46 with RIS. All surgical procedures were performed by the same dermatologic surgeon. For comparison, lesions were grouped in five facial areas. Other variables such as the age of the patient and type and size of lesions were similar in both groups. Evaluation of each scar was made blindly by two independent observers and by the patients. Judged by independent observers 90 days after surgery, excellent (45 %) to good (45%) results were obtained in similar proportion with either SIPS or RIS suturing in 90% of the patients. In the patients' self evaluation, excellent (85%) to good (14%) results were obtained in 99% of the cases. Suture marks and tracks present 30 days after surgery in 28% of scars sutured with SIPS disappeared 90 days after surgery. The esthetic results obtained by suturing small surgical facial wounds with SIPS or RIS were similarly good to excellent when observed 90 days after surgery. There was no advantage in using RIS over SIPS in the type of wounds described.

The use of retinoids in the pediatric patient.

Oral retinoids are molecules derived from vitamin A that represent one of the most important steps forward in dermatologic therapeutics in the present century. The treatment of acne, severe psoriasis, and severe disorders of keratinization, prevalent diseases in children and adolescents, have radically changed since the advent of oral retinoids. Like most highly-effective medications, oral retinoids also have important untoward effects. Specialists, and in particular, dermatologists and pediatricians should be prepared to maneuver the delicate balance between therapeutic efficacy and side effects in order to give the pediatric patient the maximum benefit with the lowest possible risk.

Cleansers, moisturizers, and talcs: patterns of use among children of low socioeconomic level.

The use of cosmetics (cleansers, moisturizers, and talcs) was investigated in 200 infants and children of low socioeconomic level with and without skin disease. For children with atopic dermatitis, mothers preferred to use unscented soap. Lubricating or hydrating creams were widely used in children without skin disease. Talc and mineral oil were significantly more frequently used in infants than in children. Both of these products are potentially harmful and represent an unnecessary expense for families with a survival-level budget. Dermatologists should discourage their use.

Intrauterine epidermal necrosis: report of three cases.

BACKGROUND: Extensive epidermal necrosis in newborn infants is an unusual event of heterogeneous cause. OBJECTIVE: The objective of this article is to describe what seems to be a previously unrecognized lethal disease. METHODS: The clinical and histopathologic features of three premature infants, two of them nonidentical twins, and the autopsy findings of one of them were analyzed. RESULTS: Intrauterine lethal epidermal necrosis with hair follicle calcification, except for the face, hands, feet, elbows, and knees, was present in all three patients. Some histopathologic features were suggestive of epidermal apoptosis. CONCLUSION: We propose that the clinicopathologic alterations in our patients represent a new condition that may be caused by massive epidermal apoptosis.

Malignant melanoma in children. A review.

The increasing incidence during the last decades and the projection for the future places malignant melanoma (MM) among the most important public health problems world-wide. Given the limited success of therapy for MM, for the time being the fighting strategy against MM should focus mainly on prevention and early detection.

Postinflammatory hypopigmentation and hyperpigmentation.

Postinflammatory hypopigmentation and hyperpigmentation are frequently encountered problems and represent the sequelae of various cutaneous disorders as well as therapeutic interventions. However, the underlying mechanisms and the variability individuals show for developing hypopigmentation or hyperpigmentation are not well understood. The authors propose an inherited individual chromatic tendency that is based on "weak" or "strong" melanocytes and their tendency to respond to trauma or inflammation with either hypopigmentation or hyperpigmentation. Clinical examples and management of both hypopigmentation and hyperpigmentation are discussed.

Giant congenital melanocytic nevi, neurocutaneous melanosis and neurological alterations.

BACKGROUND: Magnetic resonance imaging (MRI) findings suggestive of neurocutaneous melanosis (NCM) have been reported in asymptomatic patients with giant congenital melanocytic nevi (GCMN). OBJECTIVE: To investigate the presence of NCM and the clinical neurologic status of patients with GCMN involving the head an neck. METHODS: Thirteen patients with GCMN involving the head and neck were clinically examined by pediatric specialists in dermatology, ophthalmology and neurology. Electroencephalograms, noncontrasted and contrasted computerized tomography (CT) scans and MRI were performed. RESULTS: Eleven of 13 patients with GCMN of the head and neck previously considered asymptomatic were found to present mild but evident neurologic alterations. No signs of NCM were found in the CT scans or in the MRI. CONCLUSIONS: Patients with GCMN of the head and neck may have associated neurologic alterations not related to the presence of neurocutaneous melanosis.

Encephalocraniocutaneous lipomatosis: an uncommon neurocutaneous syndrome.

We describe a 2 1/2-year-old girl with encephalocraniocutaneous lipomatosis. The dysmorphologic manifestations in the skull, brain, skin, and eyes associated with a normal karyotype suggested the diagnosis of this rare neurocutaneous syndrome.

Edematous, scarring vasculitic panniculitis: a new multisystemic disease with malignant potential.

BACKGROUND: Hydroa vacciniforme (HV) is a disease of unknown origin characterized by erythema, vesicles, necrosis, and varicelliform scars in light-exposed skin. Systemic involvement is absent. A few patients have been reported with "severe HV" with systemic involvement, development of non-Hodgkin's lymphoma, and a poor prognosis. OBJECTIVE: Our purpose was to characterize and differentiate our patients' disease from HV. METHODS: We performed a retrospective clinicopathologic study of 14 children previously diagnosed as having "severe HV." RESULTS: The extension and severity of the cutaneous lesions, fever, wasting, failure to thrive, hepatosplenomegaly, vasculitis, panniculitis, and potential development of lymphoma are features that clearly differentiate edematous scarring vasculitic panniculitis from HV. CONCLUSION: Edematous scarring vasculitic panniculitis is a novel multisystemic disease with malignant potential that is not related to classic HV.

[Tuberous sclerosis. Early diagnosis with autonomic nervous system responses in hypopigmented skin]. / Esclerosis tuberosa. Diagnóstico precoz mediante respuestas de sistema nervioso autónomo en piel hipopigmentada.

The earliest clinical sign for the diagnosis of tuberous sclerosis (TS) is the presence of cutaneous hypopigmented macules. However, there are no clinical, histopathological or functional criteria to discriminate between hipopigmented macules of TS and those without associated pathology. In this prospective study, the responses of the autonomous nervous system, erythema and sweating induced through iontophoresis with pilocarpine, were studied in three groups of patients (20 with TS, 10 with hipomelanosis of Ito, and 10 with hipopigmented macules without associated pathology). In hipopigmented macules without associated pathology, the responses were similar to those observed in normal skin. In TS erythema and sweating were significantly diminished (p = < 0.001). In hypomelanosis of Ito the decrease in erythema and sweating were not statistically significant. In TS the degree of decrease of erythema and sweating correlated positively with the severity of the neurological alterations. Light and electronmicroscopic studies of the hypopigmented macules in the three groups showed morphologically normal sweat glands and nerves. The latter suggests a disfunction of the sweat glands in TS as a cause of their abnormal behavior. We conclude that sweat testing in hypopigmented macules is a useful mean for the early diagnosis of ET.

Malignant cutaneous tumors in children. Twenty years of experience at a large pediatric hospital.

The frequency of different malignant cutaneous tumors (MCTs), primary and metastatic, in children is not known. We reviewed all MCTs, primary and metastatic, seen during a 20-year period in a large general pediatric hospital. Fifty-three MCTs, 36 primary and 17 metastatic, were diagnosed in 36,207 pediatric dermatology patients. The incidence was 1.4 per 1000 patients. The relative frequency of occurrence of the different tumors was as follows: rhabdomyosarcoma, 25%; lymphomas, 19%; basal cell carcinoma, 13%; leukemia, 13%; neuroblastoma, 10%; malignant melanoma, 6%; squamous cell carcinoma, 6%; unclassified sarcomas, 4%; epithelioid schwannoma, 2%; ependymoma, 2%. The mean follow-up was 3 years; 48% died, 27% were lost to follow-up, and 25% are under control. We conclude that primary and metastatic MCTs in children are rare. Their types differ from MCTs in an older age population. MCTs in children are associated with a high mortality rate, often related to late recognition.

Trichoepitheliomatous infiltration of the skin simulating leprosy.

A 13-year-old girl had a six-year history of infiltrated erythematous plaques on the face, alopecia of the eyebrows, diffuse alopecia of the scalp, and absence of body hair. Histologically, the lesions on the face and body corresponded to trichoepitheliomas. The lesions on the face clinically simulated lepromatous leprosy. This case probably represents an entity not previously described.

Dermoid cyst connected with the lacrimal canaliculum.

An infant had a 3 x 1.5-cm congenital, slow-growing, lobulated tumor below the right inferior eyelid. The mass transilluminated, and needle aspiration yielded tears. Dacryocystography showed a large cystic area connected with the lower lacrimal canaliculum. Histologically, the tumor proved to be a dermoid cyst. This association has not been reported previously. Surgical excision and suture of the pedicule resulted in permanent cure.