[The treatment of cyclosporine A-resistant pyoderma gangrenosum with recombinant interferon alfa-2].
Explore the source record for details and available documents.
Biomedical subjects
Publications and source records attributed to T Rodríguez Cañas.
Explore the source record for details and available documents.
Congenital leukaemia is a condition occurring very rarely. In a recent review in 1993, 175 cases are reported, 25-30% of them being well documented as leukaemia cutis. We reported a new case of congenital leukaemia diagnosed as an acute non lymphoblastic leukaemia M4 (FAB) and diagnosed at birth. It involves a newborn female at 42 weeks of gestational age. The most relevant clinical features were hepatomegaly and cutaneous petechial lesions along with a generalized distribution of nodules. From the blood peripheral count, leukocytosis is observed (177 x 10(9)/L) with 48% blasts of myeloid immunophenotype. The coagulation studies were consistent with a disseminated intravascular coagulation syndrome. A biopsy carried out on a cutaneous nodule, revealed diffuse dermoepidermic infiltration by immature cells of myeloid lineage, with cellularity and count similar to that of bone marrow and peripheral blood. The karyotype in the peripheral blood was normal. Infectious and immune causes were excluded as well as constitutional illnesses associated with unstable haematopoiesis. The family rejected treatment with chemotherapy and the baby died on day 53 of life due to progressive leukocytosis and concurrent infection. Our case, like 80% of the cases reported, is of myeloid origin and confirms the fatal evolution of untreated congenital leukaemia.
Pathergic reaction (PR) has been recently included in a system of diagnostic criteria for Behçet's disease (BD), justified for its high frequency in several countries like Turkey (58%). As in occidental countries it is much less common, we advise to evaluate its diagnostic usefulness in our country. An anatomopathological study of the PR in 23 patients with BD, and 14 healthy volunteers, was conducted. Punch samples were stained with hematoxylin-eosine and for immunofluorescence. Macroscopically, there was no PR in any person (neither patients nor controls). About half of the patients showed leukocytoclastic vasculitis and/or cellular infiltration (mainly neutrophilic). These findings are no specifics (they were seen in same controls) and have a low diagnostic sensitivity. Authors consider unsuitable the inclusion of PR, as an independent BD diagnostic criterion, in our country.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Conradi-Hünermann syndrome is a type of chondrodysplasia punctata characterized by skeletal, cutaneous, and ocular anomalies. Genetic heterogeneity and incomplete penetrance may explain the wide clinical spectrum. We report a 7-day-old girl, product of a preterm pregnancy and delivery, with ichthyosiform erythroderma on the right half of the body at birth, as well as patchy cutaneous involvement of the contralateral side. On physical examination, we observed an ipsilateral shortening of the leg and a lenticular opacity of the right eye. Histopathologic study showed parakeratotic hyperkeratosis with prominent follicular involvement and atrophy of the granular and spinous layers. The skin eruption disappeared during the first 2 months. At 2 months of age, radiologic examination revealed stippled calcifications in the ribs, vertebral, and paravertebral areas. The most important differential diagnosis was CHILD syndrome. We emphasize the importance of the histopathologic study in the differential diagnosis of both syndromes.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.