[Clinical consequences of the altered function of the epidermal desmosomes in man].
The epidermic desmosomes are formed in their molecular structure by transmembrane components from the cytoplasmic plaque. These are linked up to the tonofilaments of keratin forming a continuous proteinic web which is crucial to keep the tissue integrity. The functional and structural deterioration of the desmosomes produces in human clinic a series of very typical skin diseases, the most frecuent of which is the penghigus. In them, some molecular parts of the desmosomes become strange and antigenic and trigger to an autoimmune reaction which produces a blistering disease of serious prognosis. Depending on the affected molecules the clinical manifestation are different and as Antholt says there is to make a distinction of the following varieties: phenfigus vulgaris and vegetant, phenfigus foliaceus and its varietie, phenfigus of herpes shape. For is study, it is necessary to bear in mind the clinic, the histopathology and inmunopathology (Inmunofluorescence). Some other stranger diseases are of a hereditary genetic origin because of mutations in the domains of the desmosomal molecules which produce clinical manifestations of keratodermia, hypotricosis, woolly hair, skin frailty and cardiomyopapathy. Finally, there are bacteriological staphylococcal toxins, "exfoliatin A-toxin" which produce a separation of the extracellular domain of the desmoglein 1 causing the "staphylococical scalded skin syndrome".
Robledo Aguilar
Alfredo
A
D003896:Desmosomes / Q000276:immunology; D006801:Humans; D010392:Pemphigus / Q000209:etiology / Q000473:pathology; D012872:Skin Diseases, Vesiculobullous / Q000209:etiology / Q000473:pathology