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Last update 08 May 2025

Epidermolysis Bullosa Acquisita

Last update 08 May 2025

Basic Info

Synonyms

Acquired Epidermolysis Bullosa, Acquired Form of Epidermolysis Bullosa, Acquired epidermolysis bullosa

+ [17]

Introduction

Form of epidermolysis bullosa characterized by trauma-induced, subepidermal blistering with no family history of the disease. Direct immunofluorescence shows IMMUNOGLOBULIN G deposited at the dermo-epidermal junction.

Drugs associated with Epidermolysis Bullosa Acquisita

LAS-191954

Target

PI3Kδ

Mechanism

PI3Kδ inhibitors

Active Org.-

Originator Org.

Almirall SA

Active Indication-

Inactive Indication

Epidermolysis Bullosa Acquisita

Drug Highest PhasePending

First Approval Ctry. / Loc.-

First Approval Date20 Jan 1800

Clinical Trials associated with Epidermolysis Bullosa Acquisita

NCT06834035

/ Not yet recruitingPhase 1/2IIT

Targeting Collagen VII Antibodies with IV IgG in Dystrophic Epidermolysis Bullosa

The study objective is to see if IV IgG treatment in Recessive Dystrophic Epidermolysis Bullosa (RDEB) skin in conjunction with VYJUVEK treatment improves wound healing and affects the levels of C7 and HSV-1 antibody levels in serum.
Fewer wounds, more rapidly healing wounds, and decreased C7 and HSV-1 antibodies could improve quality of life.

Start Date06 Mar 2025

Sponsor / Collaborator

Stanford University

NCT01952275

/ Unknown statusNot ApplicableIIT

Assessment of the Enrichment of Rare Coding Genetic Variants in Patients Affected by Neutrophil-Mediated Inflammatory Dermatoses

This study investigates the genetic architecture of Neutrophil-Mediated Inflammatory Skin Diseases. After collecting informed consent, all patients' clinical phenotype is graded at inclusion with a detailed case report form and a discovery cohort formed based on the certainty of diagnosis. The DNA of patients in the discovery cohort is analyzed by whole exome sequencing which identifies all protein-coding genetic variants. Subsequently, statistical burden tests are going to identify enrichment of rare coding genetic variants in patients affected by Neutrophil-Mediated Inflammatory Skin Diseases.
The ultimate goal is to reveal the responsible gene(s) that may then be targets for clinical intervention.

Start Date01 Jan 2014

Sponsor / Collaborator

University of Zurich

JPRN-UMIN000004428

/ CompletedPhase 2IIT

Exploratory research on effectiveness and safety of Rituximab treatment for steroid intractable pemphygus, bullous pemphigoid and epidermolysis bullosa acquisita. - Rtx-BD Trial

Start Date01 Nov 2010

Sponsor / Collaborator-

100 Clinical Results associated with Epidermolysis Bullosa Acquisita

100 Translational Medicine associated with Epidermolysis Bullosa Acquisita

0 Patents (Medical) associated with Epidermolysis Bullosa Acquisita

1,021

Literatures (Medical) associated with Epidermolysis Bullosa Acquisita

01 Apr 2025·The Journal of Dermatology

Treatment of refractory, inflammatory epidermolysis bullosa acquisita with a combination of dupilumab and systemic glucocorticoid

Letter

Author: Ren, Jie ; Chen, Li ; Dan, Yanjun ; Liu, Ye ; Xing, Xiaoxue

01 Apr 2025·The Journal of Dermatology

Correlation of BP180, BP230, and type VII collagen antibody titers in serum, blister fluid, erosion, and saliva in pemphigoid diseases

Article

Author: Kosaka, Mieko ; Ishii, Norito ; Tsutsumi, Masahiro ; Koga, Hiroshi ; Teye, Kwesi ; Nakama, Takekuni

AbstractAutoantibodies, including anti‐BP180 and anti‐BP230 antibodies in bullous pemphigoid (BP) and mucous membrane pemphigoid (MMP), and anti‐type VII collagen (COL7) antibodies in epidermolysis bullosa acquisita (EBA), are well characterized. Enzyme‐linked immunosorbent assays (ELISAs) for the detection of these antibodies in the serum are valuable for diagnosis. Previous studies have indicated that anti‐BP180 and anti‐BP230 antibodies in blister fluid and saliva can be detected using ELISA. The aim of this study was to detect anti‐BP180, anti‐BP230, and anti‐COL7 antibodies in several types of samples, including blister fluid, an erosive surface swab, and saliva, by ELISAs and to compare the titers of these antibodies between different types of samples. Thirty‐three patients with BP/MMP, two patients with EBA, and 13 patients with non‐pemphigoid diseases were included in this study. Samples were collected from each patient and analyzed using commercial ELISA kits. In the cases of BP/MMP, the mean ± standard deviation indices of BP180 ELISA were 411.9 ± 538.3, 253.5 ± 333.3, 2.360 ± 1.714, and 1.599 ± 1.262, and those of BP230 ELISA were 27.73 ± 37.65, 20.52 ± 30.04, 0.486 ± 0.7915, and 0.346 ± 0.4373, from sera, blister fluids, erosive surface swabs, and saliva, respectively. The indices of BP180 and BP230 ELISAs using serum were significantly correlated with those of BP180 and BP230 ELISAs using blister fluid (r = 0.9342 and r = 0.9882), an erosive surface swab (r = 0.7586 and r = 0.9332), and saliva (r = 0.5731 and r = 0.6147), respectively. This study revealed that autoantibodies were detectable in blister fluids, erosive surfaces, and saliva, and their correlation with serum titers in pemphigoid diseases was highest in blister fluid. In summary, blister fluid can be a minimally invasive sample alternative to serum for the detection of pemphigoid‐related antibodies.

01 Apr 2025·Journal of Drugs in Dermatology

Rituximab in the Treatment of Epidermolysis Bullosa Acquisita: A Systematic Review of the Literature

Review

Author: Asrani, Falguni ; Marson, Justin W. ; Xenopoulou, Dimitra

Epidermolysis bullosa acquisita (EBA) is a rare autoimmune disorder characterized by blistering of the skin and mucous membranes. Current pathophysiology implicates autoantibodies targeting type VII collagen, which serves as a crucial component of anchoring fibrils that attach the epidermis to the dermis. Management of EBA remains challenging and relies on a combination of anti-inflammatory, immunosuppressive, and immunomodulating agents. Despite treatment, EBA is often chronic and relapsing, thus significantly impacting patients' quality of life.13 In this report, we aimed to systematically review the literature to provide an overview of the effectiveness of pharmacotherapy in treating cases of EBA with rituximab (RTX), specifically. We explore the efficacy of RTX as immunomodulator monotherapy and also its use in combination with other agents. A total of 51 patients were included over 20 studies and all studies were either case reports/series or retrospective chart reviews. The most utilized adjuvant therapies were glucocorticoids (n = 29) in the forms of methylprednisolone (MP), prednisolone (PL), and prednisone (P). RTX use in combination with PL had the best overall result; this regimen resulted in 15.7% (n = 8) of patients having clinical remission (CR) and 9.8% (n = 5) having well-controlled (WC) disease. Notably, the review elucidated that 4 patients had treatment with RTX alone and 100% of them achieved either CR or partial remission/control (PR/C). Our review highlights the relative efficacy of RTX in combination therapy and as monotherapy for the treatment of this rare condition, but more powerful research in the form of randomized clinical trials is imperative in order to obtain a more comprehensive understanding of its utility in the treatment of EBA. J Drugs Dermatol. 2025;24(4):387-393. doi:10.36849/JDD.8483.

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