[Guideline to prevent claims due to medical malpractice, on how to act when they do occur and how to defend oneself through the courts]. / Guía para prevenir las reclamaciones por presunta mala praxis médica, de cómo actuar cuando se producen y cómo defenderse judicialmente.

Claims due to presumed medical malpractice are increasing in all developed countries and many of them have no basis. To prevent legal complaints, the physicians should know the reasons why complaints are made by their patients and adopt the adequate preventive measures. In the case of a complaint, it is essential to follow the guidelines that allow for adequate legal defense and the action of the physician before the judge that inspires confidence and credibility. The risk of the claims can be reduced with adequate information to the patient, the following of the clinical guidelines, control of the risk factors and adoption of verification lists in each invasive procedure. In case of complication or serious adverse effect, explanations should be given to the patient and family and it should be reported to the facility where one works and to the insurance company. If the physician received a claim, he/she should report it to the insurance compare so that it can name a lawyer responsible for the legal defense who will advise the physician regarding the appearance in court before the judge.

[Frey syndrome in childhood]. / Síndrome de Frey en la infancia.

Frey's syndrome is distinguished by the appearing of erythema, sensation of hotness, sometimes pain, and transpiration discharge in the preauricular and temporal area when ingestion stars. We present an eleven month old child with this pathology and we review the etiology and clinic manifestations of this syndrome.

Two sibs with cleft palate, ankyloblepharon, alveolar synechiae, and ectodermal defects: a new recessive syndrome?

Hay and Wells in 1976 reported seven patients from four families who had an inherited condition of which the main features were ankyloblepharon, ectodermal defects, and cleft lip and palate. The inheritance pattern was determined to be autosomal dominant. This condition is known as AEC syndrome or Hay-Wells syndrome. We report a family with two sibs showing some of these features and congenital adhesions between the upper and lower jaws (alveolar synechiae). There seems to be a recessive pattern of inheritance as neither of the parents has any features of the syndrome. This could be described as a recessive form of Hay-Wells syndrome with additional features or be named as a new syndrome.

Intentional overdose and death with 3,4-methylenedioxyethamphetamine (MDEA; "Eve"): case report.

We report a case of suicide following ingestion of a large dose of 3,4-methylenedioxyethamphetamine (MDEA, "Eve") in a 27-year-old woman with a history of depression. Several days before her death, she had attempted suicide with benzodiazepines resulting in a 24-hour hospital admission; at that time, no physiologic abnormalities were detected. Findings on autopsy were nonspecific. Toxicologic analysis showed a high concentration of MDEA and the appearance of benzodiazepines in body fluids. Ethanol and other drugs of abuse were not found. We discuss the clinical manifestations, toxicologic syndromes, and mechanisms of death with amphetamine intoxication. MDEA intoxication in young people may result in sudden death.

Self-healing infantile familial cutaneous mucinosis.

Childhood cutaneous mucinoses have been rarely reported and are difficult to classify. We describe two brothers who developed multiple, extensive cutaneous lesions during the first few months of life. Histologically the lesions were composed of mucin deposits in the dermis. In the first patient, the lesions spontaneously disappeared over the years. We believe that familial self-healing cutaneous mucinosis represents a unique entity not previously reported.

Neonatal pemphigus vulgaris with extensive mucocutaneous lesions from a mother with oral pemphigus vulgaris.

The clinical phenotype of pemphigus is well explained by the combination of desmoglein (Dsg) 1 and Dsg3 distribution pattern and antiDsg autoantibody profile (Dsg compensation theory). It has been reported that neonatal skin has a similar Dsg distribution pattern to adult mucosal epithelia. We describe a newborn girl with mucocutaneous pemphigus vulgaris (PV) from a mother with mucosal dominant PV. The mother had had painful oral erosions for at least 7 months. Histopathological examination and direct and indirect immunofluorescence studies confirmed the diagnosis of PV and neonatal PV in the mother and daughter, respectively. The mother had a high titre of anti-Dsg3 IgG and a low titre of antiDsg1 IgG, while the neonate had only a high titre of anti-Dsg3 IgG, but no detectable antiDsg1 IgG. AntiDsg3 IgG, which caused the oral dominant phenotype in the mother, induced extensive oral as well as cutaneous lesions in the neonate. Our case provides clinical evidence for the Dsg compensation theory in neonatal PV.

Reticuloendoteliose com eosinofia: doença de Omenn / Reticuloendotheliosis with eosinophilia: Omenn's disease

É apresentado o primeiro caso espanhol publicado de reticuloendoteliose com eosinofilia (doença de Omenn). Este quadro é considerado, atualmente, como uma imunodeficiência mista, grave e mortal, que dá lugar a uma proliferaçäo histiocitária reativa generalizada. No plano clínico, caracteriza-se por apresentar-se em lactentes, em forma de infecçöes diversas, diarréias, eritrodermia, alopécia, deterioraçäo do estado geral, hepatoesplenomegalia, adenopatias generalizadas e eosinofilia persistente. Em alguns casos, tem-se observado um déficit de 5-nucleotidase linfocitária e um aumento de IgE. O diagnóstico diferencial deve ser feito, fundamentalmente, com as histiocitoses malígnas (histiocitose X e histiocitoses que cursam com autocitofagia) e com outras histiocitoses reativas secundárias a déficits imunitários congênitos