Exantema periflexural asimétrico de la infancia: reporte de 2 casos / Asymmetric periflexural exanthem of childhood: 2 case reports

El exantema periflexural asimétrico de la infancia es una entidad subdiagnosticada, de etiología incierta, que afecta, predominantemente, a niñas alrededor de los dos años de edad. Se caracteriza por ser un exantema que solo afecta a un hemicuerpo y que se resuelve sin tratamiento específico.Se reportan dos casos clínicos acerca de esta entidad. El primero, referente a una niña de 14 meses con exantema papuloeritematoso de inicio en la axila siete días antes y extensión a la extremidad superior y el hemitronco ipsilateral sin otras alteraciones. El segundo, referente a una niña de 24 meses de edad con exantema papuloeritematoso pruriginoso de inicio en la extremidad inferior izquierda y la posterior extensión por el hemicuerpo izquierdo.Dadas las características del exantema y la edad de las pacientes, se diagnosticó exantema periflexural asimétrico y recibió tratamiento sintomático, con resolución del cuadro de forma espontánea

The asymmetric periflexural exanthema of childhood is an underdiagnosed entity of unknown etiology that affects, most commonly, girls around two years of age. Clinically, it affects only one side of the body and it eventually disappears without the need of any specific treatment.We report two cases of this entity. The first case is a 14-month-old female patient with an erythematosus papular eruption that initiates near the axilla and progresses, in a period of seven days, towards the ipsilateral upper limb and hemithorax, with no other alterations. The second case is a 24-month-old female with a pruriginous erythematosus papular exanthema that begins on the left lower extremity and extends throughout the left hemibody.Due to the characteristics of the exanthema and the age range of the patients, they were diagnosed with asymmetric periflexural exanthema. They both received symptomatic treatment with spontaneous resolution of the condition

Exantema laterotorácico unilateral con evolución atípica / Unilateral laryngeal eruption with atypical evolution

Una niña lactante presentó un exantema maculo-papular pruriginoso unilateral con evolución uni y contralateral. Aparentemente, el exantema fue desencadenado por una infección viral del tracto respiratorio.Los términos con que se ha descrito esta dermatosis son:exantema latero-torácico unilateral, exantema periflexural asimétricoy más recientemente exantema superpuesto lateralizado de la infancia. Este exantema es deevolución benigna yautolimitada,se presentacomúnmente como un exantema escarlatiniforme o morbiliforme que generalmente se inicia en la axila y después se disemina al tronco y miembro superior homolateral. El tratamiento es sintomático.

A female infant presented with pruritic unilateral macular rash with bilateral evolution. Apparently, the rash was triggered by a viral respiratory tract infection. Unilateral laterothoracicexanthem, asymmetric periflexural exanthema and more recently superimposed lateralized exanthem of childhoodare terms used to define this rash. It has self-limiting evolution. Commonly presented as a scarlatiniform or morbilliformrash that usually begins in the axilla and then spreads to the trunk and homolateralupper limbs. The treatment is symptomatic.

Quality of life in glaucoma patients and normal subjects related to the severity of damage in each eye. / Relación entre la calidad de vida y la severidad del daño funcional en pacientes con glaucoma.

OBJECTIVE: To assess the quality of life in glaucoma patients and normal subjects, and to assess its relationship with the severity of damage in each eye. METHODS: A cross-sectional study was conducted with prospective selection of cases. The study included 464 subjects and were distributed into 4categories. Subjects included in group 1 had both eyes normal, that is with a normal intraocular pressure (IOP), optic disk and visual fields (VF), or mild glaucoma, defined as untreated IOP>21mmHg and abnormal VF with mean defect (MD) over -6dB. Group 2 consisted of patients with both eyes with mild or moderate glaucoma, defined as untreated IOP>21mmHg and abnormal VF with MD between -6 and -12dB. Group 3 included patients with moderate to severe glaucoma, that is, untreated IOP>21mmHg and abnormal VF with MD of less than -12dB in both eyes. Group 4 consisted of patients with asymmetric glaucoma damage, that is, they had one eye with severe glaucoma and the other eye normal or with mild glaucoma. All subjects completed 3 different questionnaires. Global quality of life was evaluated with EuroQol-5D (EQ-5D). Vision related quality of life was assessed with Visual Function Questionnaire (VFQ-25). Quality of life related to ocular surface disease was measured with Ocular Surface Disease Index (OSDI). RESULTS: VFQ-25 showed that group 3 had significantly lower scores than group 1 in mental health (P=.006), dependence (P=.006), colour vision (P=.002), and peripheral vision (P=.002). EQ-5D showed no significant differences between any group, but a trend was found to greater difficulty in group 3 than in groups 1 and 2, and in all dimensions. OSDI showed a higher score, or which was the same as a major disability, in groups 2 and 3 than group 1 (P=.021 and P=.014, respectively). VFQ-25 only found significant differences between group 1 and group 4. Dimensions with significant differences were found between group 1 and 3 (both eyes with advanced or moderate glaucoma). These were not found between group 1 and group 4 (the group in which one eye has only mild glaucoma or no glaucoma). This finding confirms that the eye with less glaucoma damage determines the quality of life. CONCLUSIONS: Our results demonstrate that quality of life is impaired in patients with glaucoma, and this alteration is greater the more advanced is glaucoma damage in the best or both eyes.

Exantema periflexural asimétrico de la infancia, dos formas de presentación / Asymmetric periflexural exanthem of childhood, two forms of presentation

El exantema periflexural asimétrico de la infancia es una entidad benigna, autolimitada, subdiagnosticada, de etiología desconocida, que compromete mayormente niñas de edad preescolar. Tiene predominancia estacional (invierno y primavera). Su diagnóstico es clínico, y se presenta como un exantema escarlatiniforme, morbiliforme o eccematoso, que generalmente comienza en la axila y se extiende centrífugamente hacia el tronco y el miembro superior homolateral. Puede acompañarse de adenopatías regionales y fiebre. No requiere tratamiento específico, salvo el uso de cremas humectantes y antihistamínicos, en caso de presentar prurito. Se describen 2 pacientes con diferentes formas de presentación de esta entidad, una típica y otra infrecuente.

Asymmetric periflexural exanthema of childhood is a benign, self-limited, underdiagnosed disease of unknown etiology that mainly affects girls at preschool age. It has seasonal predominance (winter and spring). The diagnosis is clinical and is presented as scarlatinform, morbiliform or eczematous exanthem that generally begins in the axilla and centrifugally extends to the trunk and the homolateral upper limb. It may be accompanied with regional adenopathies and fever. It does not require specific treatment except for the use of moisturizing and antihistaminic creams in case of pruritus. Two patients with different forms of presentation, one typical and another unfrequent, were described.

Artritis reactiva por virus de Epstein Barr

Paciente masculino de 24 años de edad, con antecedente de buena salud, que inicio un cuadro de fiebre, faringitis, adenopatías cervicales y hepatoesplenomegalia; 3 días después, se acompaña de, artritis aditiva en muñeca derecha y rodilla izquierda, y tenosinovitis de los dedos de los pies que tomaron aspecto de dedos en salchicha, sin obtener respuesta favorable con 150 mg diarios indometacina más antibióticos; luego de los estudios realizados durante dos ingresos, se concluyó como una artritis reactiva por virus de Epstein Barr, lo cual se confirmo por estudios virológicos en el Instituto Pedro Kouri, con respuesta favorable a los esteroides y la azulfidina

Masculine patient of 24 years of age, with antecedent of good health, health history homeof fever, pharyngitis, cervical lymphadenopathy and hepatosplenomegaly; 3 days afterwards, accompanies of, additive arthritis in right doll and left knee, and tenosinovitis of the fingers of the feet that took appearance of fingers in sausage, without obtaining favorable answer with indomethacin at doses of 150 mg daily more antibiotics; afterwards of the studies realized during two income, concluded like a reactive arthritis by virus of Epstein Barr, which confirm by virological studies in the Pedro Kouri Institute, with favorable answer to the steroids and the azulfidine.

Edema hemorrágico agudo del lactante: reporte de un caso de presentación atípica / Acute hemorrhagic edema of infancy: case report

Acute infant hemorragic edema is a benign condition that affects children between 4 month and 2 years of age. Quick onset inflammatory edema and generalized ring shaped symmetric purpura are characteristic. Histology is of leucocitoclastic vasculitis. Clinical case: 9 month old child with a respiratory tract affection 2 weeks previous, consulting for left leg edema and fever, afterwards cutaneous and enteral mucosa purpura. Discussion: Acute hemorrhagic edema is a benign and self-limited illness. Lhe main differential diagnosis is Schonlein Henoch Purpura, and a careful evaluation and clinical follow-up is neccesary.

Introducción: El Edema Hemorrágico Agudo del Lactante (EHAL) es una condición benigna que afecta a niños entre 4 meses y 2 años, de inicio brusco, con edema inflamatorio y lesiones cutáneas del tipo purpúrico, anulares generalizadas y simétricas. A la histología los hallazgos son compatibles con una vasculitis leucocitoclástica. Caso clínico: Lactante de 9 meses de edad, que cursó con cuadro respiratorio 2 semanas previas y que consulta por aumento de volumen de la extremidad inferior izquierda y fiebre, agregándose en forma progresiva lesiones cutáneas purpúricas, compromiso de mucosas y gastrointestinal. Discusión: El EHAL es una condición benigna y autolimitada, cuyo principal diagnóstico diferencial es Púrpura de Schonlein-Henoch, la cual requiere una cuidadosa evaluación y segumiento clínico.

Síndrome de Proteus: presentación de dos casos / Proteus syndrome: presentation of two cases

El síndrome de Proteus es un complejo y raro desorden caracterizado por malformaciones y sobre crecimiento asimétrico de diferentes partes del cuerpo por la presencia de múltiples tejidos a modo de mosaico: nevus de tejido conectivo, nevus epidérmicos, hamartomas, lipomas, malformaciones vasculares, linfáticas y desregulación del tejido adiposo, de gran variabilidad clínica. Ponemos a consideración dos casos de síndrome de Proteus que acudieron a la consulta de Genética: la primera paciente derivada de Neurocirugía por dismorfia cráneo facial y el segundo paciente fue referido de un centro asistencial periférico por el médico pediatra, con la sospecha diagnóstica de este síndrome. En los últimos años, se ha hecho énfasis en la delimitación rigurosa de los criterios diagnósticos de esta patología, debido a los errores frecuentes de interpretación de los mismos (AU)

Proteus syndrome (PS) is a complex disorder comprising malformations and overgrowth of multiple tissues. The disorder is highly variable and appears to affect patients in a mosaic pattern: epidermal nevi, connective tissue nevi, hamartomas, lipomas, vascular and lymphatic malformations and dysregulated adipose tissue.We present two cases of PS, one of them, was refered from pediatric neurosurgeon due to cranial and face dysmorphia, the second one was refered with PS diagnosis from his pediatrician.During the last years,medical community has remarked that is necessary to make a very careful interpretation of the diagnostic criteria of this pathology, because them were misunderstood and is possible the overdiagnosis of this pathology (AU)

Exantema laterotorácico unilateral de la infancia, una enfermedad poco diagnosticada: An infrequently diagnosed disease entity / Unilateral laterothoracic exanthema of childhood

El exantema laterotorácico unilateral, también conocido como exantema asimétrico periflexural de la infancia, es una erupción eritemato-papulosa, poco o nada pruriginosa, de causa desconocida, aun cuando la mayoría de las evidencias apuntan hacia etiología viral. Se presenta en un lado del tórax, habitualmente cercano a la axila, progresa en forma centrífuga en ese lado del cuerpo y contralateralmente, para desaparecer sin secuelas en un período de 4 a 6 semanas. Se han reportado aproximadamente 300 casos en la literatura; sin embargo, la mayoría de los autores coinciden en que la falta de diagnóstico se relaciona con el desconocimiento de la enfermedad.

The unilateral laterothoracic exanthema, also known as asymmetric peri flexural exanthema of childhood, is an erythematosus papular eruption, slightly or non pruriginous, of unknown etiology, even when most of the evidence support a probable viral origin. Clinically, it initiates on one side of the thorax, generally near the axilla, progressing centrifugally on the ipsilateral side of body and contra lateral side and eventually disappears without sequelae in a period of 4 to 6 weeks. There have been reports of approximately 300 cases in the literature. However, the majority of the authors agree that the lack of diagnosis is result of the lack of knowledge about this entity.