ABSTRACT
Graft-versus-host disease (GvHD) is common after haematopoietic cell transplantation (HCT). Mucocutaneous manifestations are variable and may simulate autoimmune bullous dermatoses. However, the association of GvHD with autoimmune disorders, including bullous dermatoses, is also well recognized. We describe a patient with GvHD in whom severe and relapsing epidermolysis bullosa acquisita (EBA) was diagnosed 3 years after transplant and propose a causal association with GvHD. A 66-year-old woman developed GvHD following allogeneic HCT for acute myeloid leukaemia in 2016. This affected her gastrointestinal tract and skin but improved with oral corticosteroids and ciclosporin. In 2019 she presented with a widespread rash consisting of large, tense, haemorrhagic blisters. Histological features were in keeping with EBA. Direct immunofluorescence was also consistent with EBA, demonstrating linear positivity for IgG and C3 confined to the blister base, as was detection of collagen VII antibodies on indirect immunofluorescence. She was admitted and treated with high-dose oral steroids, ciclosporin and intravenous immunoglobulin (IVIg) with eventual resolution of blistering. Although further IVIg administration was planned as an outpatient, this coincided with the start of the COVID-19 pandemic and she elected not to attend and also stopped all medication. Despite this, her EBA remained quiescent until September 2021 when she was readmitted with a severe deterioration in blistering and significant dysphagia due to an oesophageal stricture, with a weight of 31.7 kg. Once again, she responded rapidly to oral prednisolone and IVIg. Dapsone was considered but precluded by G6PD deficiency and there were clinical and adherence concerns about using mycophenolate mofetil. Upon discharge she was again nonadherent to medication and failed to attend for planned IVIg. She flared and was admitted for a third time in December 2021, requiring gastrostomy for nutritional support;her weight at this time was 26.4 kg. Her EBA is currently well controlled on prednisolone and IVIg. EBA is a rare, acquired blistering disorder secondary to autoantibodies targeting type VII collagen. Previous studies have found circulating basement membrane zone (BMZ) antibodies in 24% of chronic GvHD patients, possibly generated in response to chronic BMZ damage (Hofmann SC, Kopp G, Gall C et al. Basement membrane antibodies in sera of haematopoietic cell recipients are associated with graft-versushost disease. J Eur Acad Dermatol Venereol 2010;24: 587-94). Corresponding clinical manifestations are rare, with bullous pemphigoid the most frequently reported. EBA is much less common with four previously reported cases [Brassat S, Fleury J, Camus M, et al. (Epidermolysa bullosa acquisita and graftversus- host disease). Ann Dermatol Venereol 2014;141: 369-73 (in French)]. As a fifth case of EBA, our patient provides further evidence of a likely pathophysiological relationship between GvHD and autoimmune subepidermal bullous dermatoses, and highlights the significant challenges of managing these vulnerable patient groups during the COVID-19 pandemic.
ABSTRACT
The blistering disease Epidermolysis bullosa acquisita is a genetic/autoimmune disorder deriving from alterations of the human protein Collagen alpha-1(VII) chain (CO7A1). Exposure to severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) promotes a wide variety of autoimmune diseases and might be a risk factor for Epidermolysis bullosa acquisita;in order to further our understanding of the link between this blistering disease and SARS-CoV-2, this study analyzes the peptide-sharing between CO7A1 and SARS-CoV-2 proteome. Results indicate a high level of molecular mimicry between CO7A1 and SARS-CoV-2 and hCoV-229E, and hCoV-NL63, thus suggesting a potential role of COVID-19 as a risk factor for Epidermolysis bullosa acquisita. Copyright © by BIOLIFE, s.a.s. This publication and/or article is for individual use only and may not be further reproduced without written permission from the copyright holder.
ABSTRACT
The proceedings contain 42 papers. The topics discussed include: a pediatric case series of COVID-19-associated chilblain-like acral lesions;genital dermatology: a window to the gut. Three cases of genital cutaneous Crohn disease;Influence of biologics on COVID-19-positive patients: a case series;cutaneous thrombosis associated with skin necrosis following Oxford/AstraZeneca COVID-19 vaccination: a case report;reactive infectious mucocutaneous eruption (RIME) secondary to COVID-19 infection;adverse events following immunization (AEFI) with COVID-19 vaccines: case series and literature review;challenges in the management of toxic epidermal necrolysis and COVID-19: a case report;and a rare case of epidermolysis bullosa acquisita in one of identical twins.
ABSTRACT
The proceedings contain 17 papers. The topics discussed include: a helix-swapped C3d dimer mediated by immune evasion protein Sbi hints at a novel s. aureus complement modulation strategy;the role of factor H in macrophages;an antibody targeting complement factor H causes anti-tumor immunity through B-cell activation;C5aR2 deficiency ameliorates inflammation in antibody transfer experimental epidermolysis Bullosa Acquisita and suggests regulating action on the decisive C5a receptor 1;clinical and biomarker characteristics of patients with C3G enrolled in two phase 2 studies investigating the factor D inhibitor Danicopan;perceiver-based machine learning diagnosis of TMA in renal biopsies;and recurrence of atypical hemolytic uremic syndrome After COVID-19 vaccination.