Explore chapters and articles related to this topic
Hematopoietic Stem Cell Transplantation in Patients with Autoimmune Bullous Skin Disorders
Published in Richard K. Burt, Alberto M. Marmont, Stem Cell Therapy for Autoimmune Disease, 2019
PF is similar to PV10 but does not have mucosal blisters, which helps to distinguish it from PV. Unlike PV, the clinical presentation can resemble a papulosquamous condition, like psoriasis or seborrheic dermatitis rather than a blistering disease. A rare variant of pemphigus folliaceous, called fogo sevageum, is endemic in the Amazon basin and it has been linked to an arthropod vector. Subcorneal vesicles favor the diagnosis of PF, while suprabasilar vesicles favor the diagnosis of PV. Mortality for PF is not as high as for PV, but some PF patients also become refractory to treatment and can evolve into a clinical presentation similar to pemphigus vulgaris. The cause of death is generally infection. The antibody in pemphigus foliaceus is directed against desmoglein 1 (Dsg-1).11 Similar to PV, titers of circulating autoantibodies correlate with extent and activity of the disease.
A comprehensive summary of disease variants implicated in metal allergy
Published in Journal of Toxicology and Environmental Health, Part B, 2022
The term ‘bullous autoimmune dermatoses’ comprises several disease subtypes with shared but distinctive pathophysiological characteristics – the two most common of which are pemphigus and pemphigoid. In both of these diseases, autoantibodies are involved in blistering eruptions of the skin and oral mucosa. Pemphigus-type diseases involve the development of autoantibodies reactive toward desmogleins – proteins involved in cell-cell adhesion – which results in the loss of keratinocyte structural integrity within the epidermis and subsequent lesion formation (Hammers and Stanley 2016). Comparatively, pemphigoid-type conditions emerge in response to autoantibody formation wherein reactivity to hemidesmosomes – proteins that mediate cell adhesion to the basement membrane – results in fixation of complement and subsequent inflammation and lesion emergence (Hofmann, Juratli, and Eming 2018). Both diseases primarily implicate IgG isoforms of effector autoantibodies, however, IgA-mediated variants of both disease types also exist (Kasperkiewicz et al. 2017).