Assessment of the healthcare costs for pemphigus and bullous pemphigoid patients in an academic center in Germany.
Assessment of the healthcare costs for pemphigus and bullous pemphigoid patients in an academic center in Germany. Br J Dermatol. 2019 Nov 20;: Authors: Ständer S, Färber B, Radeke S, Schmidt E, Zillikens D, Ludwig RJ Abstract The autoimmune skin blistering diseases pemphigus (vulgaris and foliaceus) and bullous pemphigoid (BP) pose a high burden on affected patients. With current treatment options, induction of remission is achieved in most patients. However, prolonged immunosuppression is required to maintain remission, and treatment-related morbidity and mortality further adds to the patients' burden1,2 . Hence, development of novel therapeutic strategies that are effective and safe is highly warranted. Recently, insights into pemphigus and BP pathogenesis identified new therapeutic targets and drugs3 . PMID: 31749141 [PubMed - as supplied by publisher]
Pregnancy may induce the onset or exacerbation of autoimmune bullous diseases such as pemphigus or pemphigoid gestationis. A shift toward T helper (Th) 2 immune response and the influence of hormonal changes have been evoked as possible triggering factors. Therapeutic management of this setting of patients may represent a challenge, mainly due to safety concerns of some immunosuppressive drugs during pregnancy and lactation. In this narrative review, we provided a comprehensive overview of the therapeutic management of autoimmune bullous diseases in pregnant and breastfeeding women, focusing on pemphigus and pemphigoid gestationis.
CONCLUSIONS: This retrospective study summarizes the patient characteristics, comorbidities, treatment choices and side effects in our 16 years of clinical practice. PMID: 32892539 [PubMed - as supplied by publisher]
Desquamative gingivitis is a generic condition with gingival scaling that can be a manifestation of autoimmune diseases such as lichen planus, pemphigus, and pemphigoid, etc. Diagnosis is based on biopsy of the lesions, hematoxylin and eosin staining (HE), and direct immunofluorescence, and the treatment of choice is the use of corticosteroids. A 59-year-old white female presented with areas of intense erythema and extensive ulcerations spread throughout the gingivae on both arches and on the hard palate, which stopped her from adequately feeding due to pain and bleeding.
Conclusion: Rituximab is safe and effective in childhood/juvenile patients with AIBDs. Furthermore, RTX can be used as first-line treatment in pediatric patients with pemphigus. PMID: 32589481 [PubMed - as supplied by publisher]
Autoimmune blistering diseases (AIBDs), mainly including epidermolysis bullosa acquisita (EBA), pemphigus Vulgaris (PV) and bullous pemphigoid (BP), are a large class of autoimmune diseases presenting the blistering eruptions on the skin and mucosa membrane. Circulating autoantibodies play a critical role in the pathogenesis, antibody-specific B cells and CD4+T cells involved as well. While vitiligo, a common pigmentation disorder, is mostly considered as CD8+T cell-mediated, with multiple melanocyte-derived autoantibodies also detectable in part of the patients, although its pathogenicity remains undetermined.
Autoimmune blistering diseases (AIBDs) are a group of rare acquired blistering skin diseases, which are divided into five major subtypes based on the clinical appearance and pathology: pemphigus diseases, bullous pemphigoid (BP), epidermolysis bullosa acquisita (EBA), dermatitis herpetiformis (DH) and Linear IgA bullous dermatosis (LigA). Current understanding has been greatly increased by genetic investigations mainly focus on the HLA in various populations. We have conducted the HLA association studies on different subtypes of AIBDs in Chinese population by using Next-generation (NGS) based HLA typing methods.
Paraneoplastic pemphigus (PNP), also termed paraneoplastic autoimmune multi-organ syndrome (PAMS), is a severe autoimmune blistering disease of the skin and mucosae associated with an underlying neoplasia. PNP patients have circulating autoantibodies binding to components of stratified as well as non-stratified epithelia [1,2,3]. Specifically, PNP autoantibodies typically bind to members of the plakin family of proteins, including envoplakin, periplakin, desmoplakin, and, more rarely, the bullous pemphigoid antigen 230 and plectin [1,4 –14].
Rituximab is a monoclonal antibody targeting CD20 on B cells with proven efficacy for pemphigus vulgaris, now an FDA-approved indication. Other autoimmune bullous diseases can be challenging to treat and have significant associated morbidity and mortality, but data supporting the use of rituximab in pemphigoid group diseases remain limited. Although rituximab demonstrates efficacy for clinical improvement and remission in pemphigoid, concern for adverse events may also limit the use of this medication.
Autoimmune bullous diseases (AIBDs) are prototypical organ-specific autoimmune diseases of the skin and mucous membranes.1,2 In 1953, Lever divided AIBDs into the 2 groups, pemphigus and pemphigoid diseases, depending on the location of the lesions. The major autoantigens in pemphigus diseases are keratinocyte cell surface adhesion molecules at desmosomes, and those of pemphigoid diseases are components of the epidermal basement membrane zone (BMZ) (Fig 1). Such autoantigens are depicted in Fig 1.