Síndrome de BOD / BOD syndrome

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Síndrome de McCune-Albright / McCune-Albright’s syndrome

El síndrome de McCune-Albright se caracteriza por la tríada de manchas café con leche, displasia fibrosa ósea y endocrinopatías hiperfuncionantes.Presentamos una niña de 9 años con manchas café con leche irregulares en mejilla izquierda, cuello y región frontal, así como displasia fibrosa de loshuesos subyacentes del cráneo. No se encontraron endocrinopatías ni pubertad precoz (AU)

The McCune-Albright syndrome is characterized by a triad of café-au-lait macules, bone fibrous dysplasia and hyperfunctioning endocrinopathies.A nine-year-old girl with irregular café-au-lait macules on the left cheek, neck and frontal areas, as well as fibrous dysplasia of underlying bones of theskull is reported. No endocrinopathies nor precocious puberty were found (AU)

Langerhans' cells and lymphocytic infiltrate in AIDS-associated Kaposi's sarcoma. An immunohistochemical study.

The epidermal Langerhans' cells are dendritic cells of the skin capable of triggering cutaneous immune responses. They possess the membrane antigens required to this effect: class II histocompatibility antigen, CD1a and CD4; the latter acts as receptor for the human immunodeficiency virus. The skin is the organ primarily affected by Kaposi's sarcoma (KS). In epidemic KS, the local immunologic conditions of the skin are little known. We therefore studied 12 patients with AIDS-associated KS, evaluating the density and phenotypic expression in KS-affected and unaffected skin of the following antigens: CD1a, HLA-DR, CD4 in dendritic epidermal cells and dermis, and CD3, CD4 and CD8 in cells of the inflammatory infiltrate, using monoclonal antibodies applied to frozen sections with the avidin-biotin-peroxidase technique. Langerhans' cells in the AIDS-KS skin lesions were found to be decreased in number. This decrease was even more pronounced in the case of cells expressing HLA-DR antigen. A number of them were also revealed with CD4. The tumour lymphocytic infiltrate was almost exclusively composed of CD3+ CD8+ phenotype lymphocytes. The dermis also revealed CD4+ dendritic cells. The basal keratinocytes focally expressed HLA-DR. These phenotypical alterations of the Langerhans' cells and the local immunological imbalance observed may contribute to the growth and continuity of the KS lesions.

[Kaposi's sarcoma associated with human immunodeficiency virus infection: a clinical, histological, immunohistochemical, serological and development review]. / Sarcoma de Kaposi asociado a infección por el virus de la inmunodeficiencia humana: revisión clínica, histológica, inmunohistoquímica, serológica y evolutiva.

BACKGROUND: A clinical, histologic, immunohistochemical, serological and evolutive review was carried out in 12 cases of Kaposi's sarcoma, representing 5.5% (12/217) of the HIV positive patients consecutively seen during an 18 month period. METHODS: The patients were clinically examined and staging was assessed. Skin biopsy was performed in all cases, and of healthy perilesional skin in eight, with HE and Perls stains. Immunohistochemical study of the skin lesions was carried out with monoclonal anti-collagen, anti-laminin, anti-HLA-DR, OKM5 and UEA-I antibodies. Statistical analysis was carried out with chi-square and Student's tests. RESULTS: There were 11 homosexual males and a prostitute parenteral drugs addicted female. The clinical presentation spectrum ranged from a single longitudinal lesion to multiple disseminated lesions in the whole skin and mucosae. Visceral or lymph node involvement was shown in three cases. Histological study also showed a spectrum of lesions from initial endothelial proliferation in perilesional healthy skin areas to mixed fusocellular and endothelial proliferation in nodular lesions. The immunohistochemical study with the appropriate monoclonal antibodies demonstrated the endothelial origin of cellular components. Some laboratory parameters (beta-2-microglobulin, total lymphocytes/mm3, total CD4 cells/mm3) were useful for prognostic evaluation at the time of diagnosis. CONCLUSIONS: KS associated with HIV infection is a vascular hyperplasia of endothelial origin virtually exclusive of homosexual males. Although the diagnose of KS does not determine by itself the vital prognosis of these patients, some laboratory parameters at the time of diagnosis are useful for the prognosis of HIV infection.

[Sweet's syndrome]. / Síndrome de Sweet.

A 42 year old female, diagnosed as having Sweet's syndrome, is presented as a new case of this rare disease (less than 100 cases reported before 1985) and the literature of the syndrome is reviewed. The clinical and histological features and its association with other diseases, especially the pathogenesis and treatment, are commented on.

[Cutaneo-visceral leishmaniasis: a new opportunistic infection in patients infected by HIV]. / Leishmaniasis cutaneovisceral: una nueva infección oportunista en pacientes infectados por VIH.

A HIV infected patient was admitted to hospital with fever diarrhoea and a cutaneous nodule on his left groin. Histopathological exam was diagnostic of cutaneous leishmaniasis. Subsequent exams disclosed Visceral Leishmaniasis. Three cycles of treatment (antimonials, pentamidine and metronidazole) were required for the clearance of lesions. The relation between immunosuppression and leishmania infection is commented and its role as an opportunistic pathogen is suggested. In these patients the infection takes a more aggressive course and has a worse response to the classic treatment with antimonials. These facts have made necessary the introduction of other alternative drugs.

[Yellow nail syndrome associated with sinusitis, rheumatoid arthritis, and chronic bronchitis]. / Síndrome de las uñas amarillas asociado a sinusitis, artritis reumatoide y bronquitis crónica.

A case of a 70 year old patient with the "yellow nail syndrome", that was associated to sinusitis, rheumatoid arthritis and chronic bronchitis. We comment the several studies realized (radiologic, mycologic, bacteriologic and laboratory studies) and the low effectivity of the treatment assayed.

[Thomson-type congenital poikiloderma with major bone dysplasias]. / Poïkilodermie congénitale type Thomson avec dysplasies osseuses majeures.

The authors report one case of Thomson type congenital poikiloderma with major bone dysplasias. Acroplasia of all extremities with agenesis of tibia and radius, and a peculiar aspect of the face are in contrast with the scarcity of cutaneous symptoms, the absence of consanguinuity, cataract, photosensitivity. The position of Thomson type congenital poikiloderma among the congenital poikilodermas is reviewed.