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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
Cicatricial pemphigoid has recurring blisters on mucous membranes or on skin near orifices (mouth, oropharynx, nasopharynx, esophagus, genitals, and conjunctiva).1 The loss of function due to scarring and adhesions often necessitates surgical interventions. Genital areas, conjunctivae, larynx, pharynx and esophagus involvement causes significant morbidity. Brunsting and Perry described a variant of CP characterized by deep skin bullous lesions involving the head and neck that heal with scars.13
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).
Using methods of time series data mining to recognize the influences of environmental factors on bullous pemphigoid
Published in Journal of the Chinese Institute of Engineers, 2018
Jian-Liang Lai, Yu-Ming Chang, Pin-Liang Chen, Lih-Ching Chou, Ding-Dar Lee, Meng-Han Yang
Bullous pemphigoid (BP) is a common autoimmune skin disease. Its symptoms involve the formation of bullae between the epidermis and dermis, and the tense bullae are located in the lower abdomen, inner and anterior thighs, and flexural areas (Lever 1979). It is widely known that BP occurs most commonly in aging populations (Bernard et al. 1995; Langan et al. 2008). Additionally, it is reported that exposures to ultraviolet (UV) light and radiation therapy are potential triggers (Albergo and Gilgor 1982; Perl et al. 1996; Pfau et al. 1994). However, the exact precipitating factors of BP are not completely identified. Conversely, the immunoglobulin G antibodies that target proteins in the keratinocyte hemidesmosome can result in subepidermal blister formations (Stanley et al. 1981; Thoma-Uszynski et al. 2004). Certain drugs including furosemide, nonsteroidal anti-inflammatory agents, penicillamine, and antibiotics are also potentially associated with onset of BP. Poor general health for BP patients is associated with a deteriorating prognosis as indicated by extant studies that observed increases in 1-year mortality rates following the diagnosis of BP (Colbert et al. 2004; Joly et al. 2005). In summary, pathophysiologic reasons for the phenomena associated with BP require further investigations.