Subepidermal type VII collagen speckles as an additional clue for diagnosing epidermolysis bullosa acquisita by salt‐split skin serum analysis

E. Gribaleva ◽  
S.M. Molen ◽  
D. Kramer ◽  
B. Horvath ◽  
A. Allenova ◽  
2013 ◽  
Vol 75 (5) ◽  
pp. 409-414 ◽  
Haruna SAWAKI ◽  
Amiko HAKUTA ◽  
Miwa KANAOKA ◽  
Kazuko NAKAMURA ◽  
Takashi HASHIMOTO ◽  

2013 ◽  
Vol 2013 ◽  
pp. 1-25 ◽  
Ralf J. Ludwig

Epidermolysis bullosa acquisita (EBA) is a chronic mucocutaneous autoimmune skin blistering disease. The pathogenic relevance of autoantibodies targeting type VII collagen (COL7) has been well-documented. Therefore, EBA is a prototypical autoimmune disease with a well-characterized pathogenic relevance of autoantibody binding to the target antigen. EBA is a rare disease with an incidence of 0.2 new cases per million and per year. The current treatment of EBA relies on general immunosuppressive therapy, which does not lead to remission in all cases. Therefore, there is a high, so far unmet medical need for the development of novel therapeutic options. During the last 10 years, several novel in vitro and in vivo models of EBA have been established. These models demonstrated a critical role of the genetic background, T cells, and cytokines for mediating the loss of tolerance towards COL7. Neutrophils, complement activation, Fc gamma receptor engagement, cytokines, several molecules involved in cell signaling, release of reactive oxygen species, and matrix metalloproteinases are crucial for autoantibody-induced tissue injury in EBA. Based on this growing understanding of the diseases’ pathogenesis, several potential novel therapeutic targets have emerged. In this review, the clinical presentation, pathogenesis, diagnosis, and current treatment options for EBA are discussed in detail.

2019 ◽  
Vol 18 (1) ◽  
pp. 56-64
Nikolay N. Murashkin ◽  
Leonid A. Opryatin ◽  
Alexander I. Materikin ◽  
Eduard T. Ambarchyan ◽  
Roman V. Epishev ◽  

Background. Epidermolysis bullosa acquisita (EBA) is chronic disease accompanied with subepidermal blistering on skin and mucous membranes as a result of autoimmune aggression to type VII collagen. EBA diagnostics in children is complicated due to similarity of clinical presentation with other bullous dermatosis in children.Clinical Case Description. The description of three clinical cases of EBA in children is provided. It is shown that for establishing the diagnosis it is necessary to estimate clinical evidence and to define the depth of blisters according to the results of histological examination of skin biopsy sample. Determination of IgG deposition positions relatively to the skin basal membrane due to performed indirect immunofluorescence test helps us to establish final diagnosis and specify patient management. Medical drug Dapsone was used in children with EBA, it has shown to be effective and safe to use as the first-line drug in management of such patients.Conclusion. The algorithm for EBA differential diagnosis with other bullous dermatosis in children is provided. Successful results of medical treatment are described. 

2018 ◽  
Vol 19 (2) ◽  
pp. 123-125
Sk Jakaria Been Sayeed ◽  
Md Mujibur Rahman ◽  
AKM Humayon Kabir ◽  
Md Moniruzzaman ◽  
Uzzal Mallik ◽  

Bullous systemic lupus erythematosus (BSLE) is extremely rare but distinct disease, characterized by vesicobullous skin eruptions in systemic lupus erythematosus (SLE). It can develop either before or after a diagnosis of SLE has been established. BSLE is characterized by a dermatitis herpetiformis-like histology and an autoimmunity to type VII collagen. It must be differentiated from other autoimmune vesicobullous diseases such as epidermolysis bullosa acquisita, dermatitis herpetiformis, linear IgA disease, and bullous pemphigoid. Its important to combine clinical, histological, and immunofluorescence findings to establish a diagnosis of BSLE. We report a case of BSLE to illustrate and emphasize the need for an integrative diagnostic approach.J MEDICINE JUL 2018; 19 (2) : 123-125

Sign in / Sign up

Export Citation Format

Share Document