The transforming growth factor-beta 1 (TGFβ1) promotes fibrosis,
differentiating epithelial cells and quiescent fibroblasts into myofibroblasts
and increasing expression of extracellular matrix. Recent investigations have
shown that PPAR (peroxisome proliferator-activated receptor*) is a negative
regulator of fibrotic events induced by TGFβ1. Dehydroepiandrosterone
(DHEA) is an immunomodulatory hormone essential for PPAR functions, and is
reduced in some processes characterized by fibrosis. Although scarring alopecia
characteristically develops in the female biological period in which occurs
decreased production of DHEA, there are no data in the literature relating its
reduction to fibrogenic process of this condition. This article aims to review
the fibrogenic activity of TGFβ1, its control by PPAR and its relation
with DHEA in the frontal fibrosing alopecia.
The authors present part I of a review of multicentric reticulohistiocytosis, a rare systemic proliferative disease of histiocytes of unknown cause. It is clinically characterized by cutaneous and mucosal nodules and by osteoarticular lesions. The disease occurs in outbreaks that progress in severity, with spontaneous regression, but usually leaving incapacitating arthritis and disfiguring facial lesions. The authors discuss the historical, epidemiologic, and clinical aspects of this disease.
In this second part of the review of multicentric reticulohistiocytosis, the authors discuss its association with other diseases, in particular, cancer, and laboratory and therapeutic aspects of this incapacitating and disfiguring disease. Histopathologic aspects are characteristic: dense mononuclear infiltrate with typical multinucleated cells that contain periodic acid-Schiff-positive and diastasis-resistant material, conferring a "ground glass" aspect when stained with hematoxylineosin.
Advances in knowledge of neurocellulars relations have provided new directions in
the understanding and treatment of numerous conditions, including atopic
dermatitis. It is known that emotional, physical, chemical or biological stimuli
can generate more accentuated responses in atopic patients than in non-atopic
individuals; however, the complex network of control covered by these
influences, especially by neuropeptides and neurotrophins, and their genetic
relations, still keep secrets to be revealed. Itching and airway
hyperresponsiveness, the main aspects of atopy, are associated with disruption
of the neurosensory network activity. Increased epidermal innervation and
production of neurotrophins, neuropeptides, cytokines and proteases, in addition
to their relations with the sensory receptors in an epidermis with poor lipid
mantle, are the aspects currently covered for understanding atopic
dermatitis.
-Chédiak-Higashi syndrome is a rare hematological disease characterized by increased fusion of cytoplasmatic granules. Neurological symptoms occur in approximately half of the patients. We describe the clinical, eletrophysiological, hematological and radiological findings in a girl who had Chédiak-Higashi syndrome and seizures.
A síndrome de Sweet pode estar associada a malignidades hematológicas, principalmente, à leucemia mieloide aguda, porém existem poucos relatos demonstrando a associação com a policitemia vera. Relata-se o caso de doente do sexo masculino, de 65 anos, portador de policitemia vera,que evoluiu com aparecimento de síndrome de Sweet na sua forma paraneoplásica.
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