Haga su diagnóstico / Make your diagnosis

El diagóstico es Enfermedad de Darier.

Tissue Doppler deformation imaging identifies myocardial stunning occurring during the Tako-Tsubo syndrome. Case report in a Caucasian patient.

Only a few reports are available on the Tako-Tsubo syndrome in Caucasian patients. The aetiology remains unknown but several pathophysiological mechanisms have been proposed so far. We believe that this condition is not so rare in Caucasian patients because many diagnoses may be missed or misinterpreted. Tissue Doppler evaluation identifies myocardial reversible dysfunction, putatively stunning, at the onset of cardiomyopathy. This can help in diagnosis, prognosis and in choosing the best time for coronary angiography.

Enfermedad de injerto contra huésped crónica / Chronic graaft versus host disease

La enfermedad de injerto contra huésped crónica (EICHc) es la principal causa de mortalidad tardía relacionada con el transplante alogénico de células progenitoras hematopoyéticas (TACPH) y causa importante de morbilidad. Se analizó la forma de presentación, evolución, respuesta al tratamiento y complicaciones de un grupo de niños y adolescentes que presentaron EICHc luego de recibir un TACPH. La EICHc fue clasificada según su relación con la EICH aguda en progresiva, quiescente o de novo. Según su extensión se clasificó en limitada o extensa. Durante el período estudiado recibieron un TACHP 140 pacientes. Treinta y uno (22%) de ellos presentaron EICHc. La enfermedad fue extensa en 18 casos y limitada en 13. Al diagnóstico 6 ptes. presentaron plaquetopenia. Recibieron tratamiento 28 ptes. 24 con prednosina, 3 con prednisona y ciclosporina, y 1 con PUVA. Respondieron al tratamiento inicial 61% de los tratados por enfermedad extensa y el 86% de aquellos con enfermedad limitada. En los ptes. que no respondieron al tratamiento inicial se agregaron otras drogas como azatioprina, talidomida, tacrolimus y micofenolato. Todos los ptes. recibieron profilaxis para infecciones por bacterias capsuladas y pneumocistis carinii. se registraron 10 infecciones graves (ocho neumonías, una sepsis y una infección del SNC). la mortalidad relaconada con el transplante fue del 16% (4 ptes. por infección y 1 pte. por bronquiolitis obliterante). Se observaron como secuelas cataratas (4), necrosis aséptica de cadera (3) y déficit hormonales (2). Los sobrevivientes presentaron una buena capacidad funcional de acuerdo a los scores de Lansky y/o Karnosfky. palabras clave: enfermedad de injerto contra huésped, transplante alogénico de células progenitoras hematopoyéticas.

Acute annular urticaria in infants and children.

Characteristic features of acute annular urticaria in 34 infants and small children were large, erythematous annular and polycyclic lesions with violaceous centers, eyelid, hand, and foot edema, absence of angioedema of the airway, absence of pruritus, spontaneous resolution in 8 to 10 days, and frequent history of furazolidone medication for diarrhea. Nondermatologists often misdiagnose acute annular urticaria as erythema multiforme and unnecessarily overtreat patients. The differential diagnosis with other conditions presenting with annular lesions in children is discussed.

Giant pigmented nevi: clinical, histopathologic, and therapeutic considerations.

Eighty pediatric patients with giant pigmented nevi more than 20 cm in their greatest diameter are reported. The incidence was 1 in 4150 general pediatric outpatients. The mode of inheritance of giant pigmented nevi is probably multifactorial; four second-degree relatives of our patients also had large nevi, and there was a 2:1 female predominance. Satellite nevi were present in 74% and nevi in mucous membranes in 31% of the patients. Eighty-six percent of nevi were pigmented and hairy. Benign nodules were observed in 19% of the patients and plexiform overgrowths in 6%. Nevi extensively involving the extremities resulted in reduced growth of the affected limb. Electroencephalograms showed abnormalities in 20% of the patients with giant pigmented nevi involving the head and upper portion of the trunk. Malignant transformation appeared in four patients and was fatal in three of them. Management consisted of observation only in 49%, surgery in 27.5%, chemical peel in 21%, and dermabrasion in 2.5% of the patients. The mean follow-up was 4.7 years.

Hypomelanosis of Ito: diagnostic criteria and report of 41 cases.

We conducted a 20-year prospective review of 41 pediatric patients with a diagnosis of hypomelanosis of Ito. No evidence pointed to hereditary transmission of the disease. Associated extracutaneous pathology was mainly of the nervous and musculoskeletal systems. Three of 19 patients had chromosomal anomalies. Hypomelanosis of Ito is a clinically well-characterized syndrome in which chromosomal instability may be a component. Criteria for its presumptive and definitive diagnosis are proposed.

[Incidence of malignant, primary and metastatic solid skin tumors at a pediatric dermatology service]. / Incidencias de tumores cutáneos sólidos malignos, primarios y metastásicos en un servicio de dermatología pediátrica.

The incidence of primary and metastatic cutaneous malignant solid tumors was investigated in a pediatric dermatology department. Among 25,000 first time patients seen between 1971 and 1985, 19 had cutaneous malignant solid tumors with an annual incidence of 0.7 for every 1,000 pediatric dermatology patients. Nine cases had primary cutaneous tumors and 10 cases metastatic tumors. The majority of patients were infants (zero to two years). The tumors found were rhabdomyosarcoma, six cases; basal-cell carcinoma, four cases; neuroblastoma, three cases; malignant melanoma, two cases, squamous-cell carcinoma, dermatofibrosarcoma, atypical fibroxanthoma and myxopapillary ependymoma, one case of each. Predisposing factors for the developing of malignancy were present in 42% of patients.

Optic nerve: a concise review of the anatomy, pathophysiology and principal acquired disorders.

The Authors review the acquired diseases of the optic nerve (o.n.) through a selection of a few main nosological categories elaborated according to recent literature and neuro-imaging developments. The proposed selection does not coincide with a classification as it includes different topics, some listed according to their aetiology, others on the clinical presentation. However, from a practical point of view, a simplified nosology should help in dealing with the complex variety of o.n. diseases and in tackling the difficulties of their differential diagnosis.

Phacomatosis pigmentovascularis: a new syndrome? Report of four cases.

We examined four patients who had a combination of extensive nevus flammeus, significant oculocutaneous pigmentation, and severe neurologic alterations. All cases were sporadic. The vascular and neurologic alterations were clinically similar to those observed in the Sturge-Weber syndrome. The capillary vessels are ultrastructurally different in phacomatosis pigmentovascularis from those of a nevus flammeus in the Sturge-Weber syndrome; however, melanocytes of normal aspect were present in the middle and deep dermis. The characteristic oculocutaneous pigmentation probably represented a noncoincidental association. The term phacomatosis pigmentovascularis seems appropriate for this apparently new neurocutaneous syndrome.

Problems of etiology in femoral neuropathies.

29 cases of femoral mononeuropathy are reported. While the clinical features of the femoral neuropathy are easily identified, the etiology is often hard to establish. The cases reported tend to fall into three general categories: 1) cases without major diagnostic difficulties (e.g. diabetic neuropathy); 2) those in which the definite diagnosis results from combined evidence of laboratory and instrumental data (degenerative changes in the lumbar spine, compressions, entrapments, etc.); 3) those in which the negative result of the investigations prevents a positive diagnosis and hence a presumptive etiology (spondylosis, inflammatory process, ischemia of the nerve) may be formulated. Attention is drawn to the favorable course of the condition in the patients of this group.

Quistes eruptivos vellosos. / Vellus eruptive cysts

Se presentan dos casos de "quistes vellosos eruptivos" en un nino de 10 y otro de 6 anos, con un cuadro clinico caracterizado por pequenas papulas ubicadas en cara anterior de torax, algunas con tapon corneo en su centro.El estudio histopatologico mostro pequenos quistes ubicados en dermis media, rodeados por epitelio estratificado, el que en algunos puntos esta en contacto con estructuras de aspecto folicular; dentro del quiste existen restos de queratina laminar y abundantes tallos pilosos

Quistes eruptivos vellosos. / Vellus eruptive cysts

Se presentan dos casos de "quistes vellosos eruptivos" en un nino de 10 y otro de 6 anos, con un cuadro clinico caracterizado por pequenas papulas ubicadas en cara anterior de torax, algunas con tapon corneo en su centro.El estudio histopatologico mostro pequenos quistes ubicados en dermis media, rodeados por epitelio estratificado, el que en algunos puntos esta en contacto con estructuras de aspecto folicular; dentro del quiste existen restos de queratina laminar y abundantes tallos pilosos

Pigmentacion maculosa eruptiva idiopatica. Comunicacion de cuatro casos. / Idiopathic eruptive macular pigmentation.Communication of four cases

Se comunican cuatro casos de pigmentacion maculosa eruptiva idiopatica, nueva entidad que afecta a ninos y adolescentes y se caracteriza por manchas ovales diseminadas de color cafe claro o gris pizarra, asintomaticas, de evolucion cronica, involucion espontanea y etiologia desconocida