China
Africa
Explore chapters and articles related to this topic
Blistering skin disorders
Published in Ronald Marks, Richard Motley, Common Skin Diseases, 2019
The condition may respond to the same types of treatment as pemphigoid but other treatments may be required including the administration of pooled human immunoglobulin, or even plasmapheresis. When severe and uncontrolled the condition may be fatal. In some cases epidermolysis bullosa acquisita is associated with an underlying condition such as rheumatoid disease or an abnormality of immunoglobulins.
Ciclosporin
Published in Sarah H. Wakelin, Howard I. Maibach, Clive B. Archer, Handbook of Systemic Drug Treatment in Dermatology, 2015
Murtaza Khan, John Berth-Jones
There are numerous reports of off-label uses of ciclosporin in dermatology, including the following: Behçet’s disease.Chronic urticaria.Connective tissue diseases including scleroderma and dermatomyositis.Immunobullous disorders such as pemphigus and epidermolysis bullosa acquisita.Lichen planus.Nodular prurigo (prurigo nodularis).Palmo–plantar pustulosis.Photodermatoses such as chronic actinic dermatitis and solar urticaria.Pyoderma gangrenosum.Toxic epidermal necrolysis.
Photoaggravated Dermatoses
Published in Henry W. Lim, Herbert Hönigsmann, John L. M. Hawk, Photodermatology, 2007
Victoria P. Werth, Herbert Hönigsmann
Acute CLE can clinically present as the typical malar erythema, but other manifestations include widespread morbilliform or exanthematous eruption in a photodistribution, sparing the knuckles, and bullous or TEN-like acute CLE skin lesions. The lack of targeting of the metacarpal phalangeal (MCP), proximal interphalangeal (PIP), and distal interphalangeal (DIP) joints can help distinguish LE from dermatomyositis (DM). The malar erythema can be either patch or plaque-like, and there is a tendency to spare the nalolabial folds, as opposed to DM, which can sometimes be in the differential diagnosis. Unusual presentations seen in acute CLE skin lesions also include involvement of the lips and periorbital edema. The bullous LE subset seen in acute CLE is a subepidermal blistering process. The criteria normally utilized for making the diagnosis include the diagnosis of SLE based on the American Rheumatologic Association (ARA) criteria, vesicles and bullae arising on, but not limited to, sun-exposed skin, routine histopathological findings consistent with dermatitis herpetiformis, and a direct immunofluorescence revealing IgG and/or IgM and often IgA and C3 at the dermal–epidermal junction (70,71). Sera from such patients often contain autoantibodies that react with the epidermolysis bullosa acquisita antigen, type VII collagen (70). However, some patients do have immunoreactants to the epidermal side of 1 M NaCl split skin as well as other autoantibodies in their sera (71,72).
Altered levels of complement components associated with non-immediate drug hypersensitivity reactions
Published in Journal of Immunotoxicology, 2020
Feng Wang, Liping Huang, Junfeng Yu, Dandan Zang, Liangping Ye, Qixing Zhu
The complement cascade acts as a bridge connecting innate and adaptive immunity, especially during T-cell-mediated skin inflammatory diseases. Recent studies found complement activation is an essential step in the process of sub-epidermal blistering during bullous pemphigus (Kasprick et al. 2018). Other studies on epidermolysis bullosa acquisita (subepidermal blistering disease) reported that C3, C5b, and C5b-9 were deposited in the skin lesions (Mihai et al. 2007). In addition, C5a was shown to contribute significantly to acquired T-cell immunity by facilitating the accumulation of T-cells in mice during contact sensitivity responses (Tsuji et al. 2000). Of note, it seems a common phenomenon that complement components participate in multiple T-cell dysfunctions, confirmed by a large body of literature (Iwata et al. 2013; Wang et al. 2013; Haeberle et al. 2018). Thus, the current study sought to define expression profiles and characterize the extent of various complement components in niHDR that are also T-cell-mediated disorders.
Biological therapy of autoimmune blistering diseases
Published in Expert Opinion on Biological Therapy, 2019
Epidermolysis bullosa acquisita (EBA) is a rare and chronic subepidermal autoimmune mucocutaneous bullous disease caused by autoantibodies directed against type VII collagen [2]. Several lines of evidence indicate that the role of cytokines in EBA is well established. In particular, it has been demonstrated that IL1b drives EBA [86] and blockades of TNF, GM-CSF, CXCR1/2, and IL17A have therapeutic effects in experimental EBA [87,88]. Furthermore, there is evidence that IL6 protects from induction of EBA through increased IL1ra expression whereas MIP1a is increased but does not contribute to EBA pathogenesis [89,90]. Consequently, these cytokines could be useful therapeutic targets for the treatment of this debilitating autoimmune bullous skin condition.
Bullous pemphigoid induced by biologic drugs in psoriasis: a systematic review
Published in Journal of Dermatological Treatment, 2022
Husein Husein-ElAhmed, Martin Steinhoff
In our work, circulating IgG autoantibodies were detected in 33% of the records, whereas they have been detected in up to 75% cases of other drug-induced BP (58). Salt-split skin laboratory tests were conducted in 7 out of the 15 (46.67%) retrieved records (8,12,14,15,17,19,20). This allowed to rule out the diagnosis of epidermolysis bullosa acquisita. The diagnosis of BP was confirmed as the IgG deposits were located in above (BP) instead of not below (epidermolysis bullosa acquisita) of the lamina dense in the BMZ (59).