Biomedical subjects
Publications and source records attributed to A Torrelo.
Costello syndrome is an uncommon multisystemic condition with highly characteristic cutaneous manifestations. We describe here the case of a patient with Costello syndrome, and review previous reports of this entity.
Cutaneous, noninfectious, granulomatous lesions have been reported occasionally in different types of immunodeficiencies, including common variable immunodeficiency (CVD). We present a child with CVID and cutaneous granulomas with a strikingly prominent caseating necrosis. We think that such granulomatous lesions constitute a distinctive manifestation of immunodeficiency, and may reflect a altered immune response. Corticosteroids have been of benefit to our patient, as well as in similar cases.
We report a case of hobnail hemangioma presenting as a congenital 2-mm brownish papule on the face of an 11-year-old girl. Hobnail hemangiomas have recently been characterized as a group, and they are related to the so-called targetoid hemosiderotic hemangiomas. Immunohistochemical data (positivity for factor VIII-related antigen, CD-34 and CD-31) support a vascular origin.
A young boy developed childhood keratosis lichenoides chronica. The eruption cleared spontaneously after 13 years. This is a rare condition, regarded by some as a variant of lichen planus. The great preponderance of cases occur in adults, whereas the disease in childhood is uncommon.
Mastocytosis is a disorder of mast cell proliferation that may appear during infancy, childhood, or adulthood. We studied 67 consecutive patients (33 males, 34 females) with urticaria pigmentosa and assessed them fully to determine the presence of systemic involvement. Ages at onset of lesions ranged from birth to 11 years, with most developing in the first year of life. Pruritus was the primary symptom. Hematologic and serum chemistry profile, radiologic skeletal surveys, and bone marrow aspirations were performed. Slight anemia was present in three patients. Radiologic bone lesions were observed in eight. Bone marrow aspirates showed slight changes in six patients, with only an increased number of mast cells in an additional patient. The disease tended to resolve spontaneously. This prospective study emphasizes the benign nature of pediatric urticaria pigmentosa.
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We describe a patient with severe eyelid and lip angioedema lesions in whom biopsy specimens from angioedematous labial mucosa disclosed features of lymphomatoid granulomatosis. To our knowledge, angioedema lesions with characteristic histological findings of lymphomatoid granulomatosis have not been previously described as a presenting sign of this disease.
Thirteen patients with a severe adult form of atopic dermatitis (AD) received 3.0 x 10(6) IU of recombinant interferon-alpha 2a (rIFN-alpha 2a) 3 times a week. A satisfactory response was obtained in 5 of them. Serum IgE levels in all 13 patients remained unchanged throughout the study. Flu-like symptoms were common, but clinical or laboratory adverse effects were otherwise slight. The moderately beneficial therapeutic effects observed in this study support a possible role for IFN-alpha in controlling immunologic deficiencies in atopic dermatitis.
We report 6 patients with the diagnosis of leukocyclastic vasculitis, with a neoplasia as the only clinical manifestation associated. Evolution of vasculitis was chronic or recidivant; rapid erythrocyte sedimentation rate, positivity for rheumatoid factor, and hypocomplementemia were frequent in our cases. The clinical course of vasculitis and tumour was discordant. The authors believe that there are not sufficient data to demonstrate an association between leukocytoclastic vasculitis and tumours.
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Periarticular fibrous nodules have been described in patients with late-phase acrodermatitis chronica atrophicans (ACA) in the setting of Lyme borreliosis. We report the clinical and histopathological study of two patients with periarticular fibrous nodules and with Lyme borreliosis and discuss the relationship between these nodules and ACA.
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