Epidermolysis bullosa acquisita

Miyamoto, Denise; Gordilho, Juliana Olivieri; Santi, Cláudia Giuli; Porro, Adriana María

doi:10.1016/j.abd.2021.09.010

Cited by 15 publications

(14 citation statements)

References 82 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…It predominantly affects the mucous membranes and, up to 30% of patients may also have skin involvement [37,40,41]. The disease most frequently involves the oral mucosa (85% of patients), followed by ocular conjunctiva (65%), nasopharynx, larynx, anogenital region, and esophagus involvement can be seen and results in mucosal blistering, ulceration, and subsequent scarring [41,42]. The disorder if not recognized and treated early and aggressively in some cases, often may cause blindness, esophageal strictures, and even difficulty speaking and breathing [17,22].…”

Section: Mucous Membrane Pemphigoidmentioning

confidence: 99%

“…The disorder if not recognized and treated early and aggressively in some cases, often may cause blindness, esophageal strictures, and even difficulty speaking and breathing [17,22]. MMP mainly occurs in the elderly population, commonly observed between 60 and 80 years of age and seen in women more often than men [37,42].…”

Section: Mucous Membrane Pemphigoidmentioning

confidence: 99%

“…Certain HLA types like HLA DRB1*1503 have been associated with an increased risk of developing MMP [17,22]. Autoantibodies against different antigens of the basement membrane zone, such as BP180, BP230, integrin subunits α6/β4, laminin-332 (also called epiligrin and laminin-5), laminin-6, and type VII collagen has been blamed for dermo-epidermal separation and blister formation in MMP [40,42]. Generally, MMP has been classified into three types based on the antigenic target classic MMP (BP180); ocular MMP (α6β4 integrin); and anti-laminin 332 MMP (laminin 332) [18].…”

Section: Mucous Membrane Pemphigoidmentioning

confidence: 99%

“…As in other chronic diseases, the patient's age, comorbid conditions, drug use, and severity of the disease are effective in the choice of treatment. In addition, a multidisciplinary approach and careful clinical examination are important in treatment because of irreversible tissue damage due to the disease [42].…”

Section: Mucous Membrane Pemphigoidmentioning

confidence: 99%

“…With or without prednisone treatment, depending on the patient's response to treatment; methylprednisolone 500 mg-1 g/day -3 days (pulsed steroid therapy) or cyclophosphamide 1 g + dexamethasone 100 mg/day every 28/28 days or IV Immunoglobulin 2 g/kg/cycle and/or rituximab 375 mg/m2/week can be preferred. Also, azathioprine 1-3 mg/kg/day, mycophenolate mofetil 2-3 g/day, methotrexate 10-17.5 mg/week, and cyclophosphamide 1-2 mg/kg/day are also agents that can be used in the treatment [18,37,38,42].…”

Section: Mucous Membrane Pemphigoidmentioning

confidence: 99%

See 4 more Smart Citations

Skin Blister Formation and Subepidermal Bullous Disorders

Aygar¹,

Gönül²

2023

Wound Healing - Recent Advances and Future Opportunities

View full text Add to dashboard Cite

Blistering diseases comprise a large group of clinically polymorphic and sometimes devastating diseases. Blistering diseases are evaluated according to the level of the blister, the mechanism of blister formation and the type of inflammation. There are many connections in the normal structure of the skin that hold the cells together. These connections both hold the cells in the epidermis together and ensure that these cells attach to the basement membrane. As a result of damage to these connections by genetic, immune, infectious or physical reasons, intercellular connections are broken and blistering developments due to the accumulation of extracellular fluid in the intercellular spaces. Autoimmune bullous diseases are classified according to the decomposition site of the epidermis. While the pemphigus group is used to classify diseases with intraepidermal separation, the pemphigoid group diseases are used to classify diseases with subepidermal separation. In this section, pemphigoid group diseases, such as bullous pemphigoid, mucous membrane pemphigoid, acquired epidermolysis bullosa, linear IgA bullous dermatosis, and anti-p200 pemphigoid, will be explained with a brief introduction to blistering diseases of the skin.

show abstract

Section: Mucous Membrane Pemphigoidmentioning

confidence: 99%

Section: Mucous Membrane Pemphigoidmentioning

confidence: 99%

Section: Mucous Membrane Pemphigoidmentioning

confidence: 99%

Section: Mucous Membrane Pemphigoidmentioning

confidence: 99%

Section: Mucous Membrane Pemphigoidmentioning

confidence: 99%

See 3 more Smart Citations

Skin Blister Formation and Subepidermal Bullous Disorders

Aygar¹,

Gönül²

2023

Wound Healing - Recent Advances and Future Opportunities

View full text Add to dashboard Cite

show abstract

From Multiple Sclerosis to Organ-Specific Autoimmune Disorders: Insights into the Molecular and Clinical Implications of Comorbidity

Zabihi,

Zangooie,

Piroozkhah

et al. 2024

Mol Neurobiol

View full text Add to dashboard Cite

Oleogel-S10 in Dystrophic Epidermolysis Bullosa: A Case Series Evaluating the Impact on Wound Burden Over Two Years

Torres Pradilla,

Álvarez,

Novoa

et al. 2024

Adv Ther

View full text Add to dashboard Cite

Epidermolysis bullosa (EB) is a group of rare, difficult-to-treat, inherited multisystem diseases affecting epithelial integrity. Impaired wound healing is central and can lead to serious clinical complications, deformities, and symptoms with a devastating impact on quality of life (QoL). Dressing changes and wound care are central to the management of EB. Recently Oleogel-S10 (also known as birch bark extract or birch triterpenes) was approved in Europe and the UK for treating EB wounds. This approval was based on data from the EASE phase 3 study, which demonstrated Oleogel-S10 accelerated wound healing, reduced total wound burden, and decreased the frequency of dressing changes in patients with EB. A retrospective analysis of medical records was conducted for up to 24 months in 13 patients with EB treated with Oleogel-S10 through an early access programme in Colombia. Effectiveness was assessed by measuring body surface area percentage (BSAP) and total body wound burden (EBDASI). Tolerability and safety were monitored throughout. This is the first report to evaluate the effectiveness of Oleogel-S10 in clinical practice. The results showed a reduction in percentage of BSA affected, from a mean of 27.3% at baseline to 10.4% at 24-month follow-up, despite treatment interruptions. A reduction in EBDASI skin activity score of − 16.2 (24 months) together with a reduced skin damage index score of − 15.4 (18 months) was also observed. Physicians, patients, and caregivers perceived faster wound closure. Adherence with therapy by patients was good, and patients expressed satisfaction with treatment and reported improvements in self-esteem, productivity, and social interaction. Oleogel-S10 was well tolerated; however, two patients reported worsening wounds related to gauze adherence. Two deaths during treatment interruption were reported and was not considered related to Oleogel-S10. This study supports the effectiveness of Oleogel-S10 in a real-world scenario in a country with scarce resources for the treatment of EB.

show abstract

Epidermolysis bullosa acquisita

Cited by 15 publications

References 82 publications

Skin Blister Formation and Subepidermal Bullous Disorders

Skin Blister Formation and Subepidermal Bullous Disorders

From Multiple Sclerosis to Organ-Specific Autoimmune Disorders: Insights into the Molecular and Clinical Implications of Comorbidity

Oleogel-S10 in Dystrophic Epidermolysis Bullosa: A Case Series Evaluating the Impact on Wound Burden Over Two Years

Contact Info

Product

Resources

About