Esta doença aparece nas substancias que contem as células da pele (talvez a extra-celular matrix), parece ser produzida por anticorpos e tudo isso leva a crer que é uma disfunção sistemica. Parece que é causada tambem por radiação solar, o que nos lembra “luz”. Muito importante ler este paper para ir entendo como o corpo humano funciona como sistema, como os sistemas biológicos desenvolveram suas propriedades ( por exemplo, sistema imunológico) a partir de LUCA para se adaptar ao ambiente terrestre.
Importante paper em português contendo todas as mais detalhadas informações, fotos, etc., está na tese em pdf:
Titulação de Anticorpos Séricos Anti-Epiteliais e Células Dendríticas no Pênfigo Foliáceo Endêmico
Desmosomes are cell-cell junctions between epithelial, myocardial and certain other cell types. Desmoglein 1 is a calcium-binding transmembrane glycoprotein component of desmosomes in vertebrate epithelial cells. Currently, four desmoglein subfamily members have been identified and all are members of the cadherin cell adhesion molecule superfamily. These desmoglein gene family members are located in a cluster on chromosome 18. The protein encoded by this gene has been identified as the autoantigen of the autoimmune skin blistering disease pemphigus foliaceus
Langerhans cells are dendritic cells (antigen-presenting immune cells) of the skin and mucosa, and contain large granules called Birbeck granules. They are present in all layers of the epidermis, but are most prominent in the stratum spinosum. They also occur in the papillary dermis, particularly around blood vessels, as well as in the mucosa of the mouth, foreskin, andvagina. They can be found in other tissues, such as lymph nodes, particularly in association with the condition Langerhans cell histiocytosis (LCH).
Dendritic cells (DCs) are immune cells forming part of the mammalian immune system. Their main function is to process antigen material and present it on the surface to other cells of the immune system. That is, dendritic cells function as antigen-presenting cells. They act as messengers between the innate and adaptive immunity.
Dendritic cells are present in tissues in contact with the external environment, such as the skin (where there is a specialized dendritic cell type called Langerhans cells) and the inner lining of the nose, lungs,stomach and intestines. They can also be found in an immature state in the blood. Once activated, they migrate to the lymph nodes where they interact with T cells and B cells to initiate and shape the adaptive immune response. At certain development stages they grow branched projections, the dendrites that give the cell its name (δένδρον or déndron being Greek for “tree”). While similar in appearance, these are distinct structures from the dendrites of neurons. Immature dendritic cells are also called veiled cells, as they possess large cytoplasmic ‘veils’ rather than dendrites
Innate immune system
(primeira manifestação do sistema imunológico nos sistemas biológicos, parece que vem da entidade de sistema de LUCA que o aplica para se manter como sistema fechado. Para se adaptar às condições terrestres os Sb desenvolveram o segundo tipo de sistema imunológico, chamado: adaptive immune system
The innate immune system, also known as non-specific immune system and first line of defense, comprises the cells and mechanisms that defend the host from infection by other organisms in a non-specific manner. This means that the cells of the innate system recognize and respond to pathogens in a generic way, but unlike the adaptive immune system, it does not confer long-lasting or protective immunity to the host. Innate immune systems provide immediate defense against infection, and are found in all classes of plant and animal life.
The innate immune system is thought to constitute an evolutionarily older defense strategy, and is the dominant immune system found in plants, fungi, insects, and in primitive multicellular organisms.
Adaptive immune system
The adaptive immune system, also known as the acquired immune system or, more rarely, as the specific immune system, is composed of highly specialized, systemic cells and processes that eliminate or prevent pathogen growth.
This mechanism can be contrasted with innate immunity in that pathogen-specific receptors of innate immunity are encoded in the germline, whereas pathogen-specific receptors of acquired immunity are acquired through a somatic expression process during the lifetime of the organism. Both immune systems are “adaptive” in the physiological and evolutionary sense of allowing the organism to adapt to changing external circumstances. Both can be maladaptive in sense that over-activity can lead to pathological inflammation and/or autoimmunity. The pathogen-receptors of innate and acquired immune mechanisms are both specific, but the specificities of the former have evolved over evolutionary time for highly conserved molecular features of the microbial world, while the specificities of the latter mature in each organism. For this reason, the term acquired is generally preferred over adaptive or specific.