[Lymphoblastif lymphoma (contribution to its prognosis)]. / Linfoma linfoblástico (aportes para el pronóstico)

The authors report a case of lymphoblastic lymphoma in a woman of 41 years, with characteristic skin lesions, long evolution and relative benignity (good general condition, lymph nodes, liver and spleen not palpable, and the bone marrow study was normal). We wish to underline the existence of a Monoclonal Disglobulinemia (Paraproteins in the zone of Inmunoglobulin M); this observation was made in some cases of leukemia. This laboratory data would be of a prognostic value, indicating a rapid malign evolution, in contrast with other clinical and laboratory manifestations.

[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.

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.

Haga su diagnóstico / Make your diagnosis

El diagóstico es Enfermedad de Darier.

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.

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.

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.

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.

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.