A 41-year-old woman with myelodysplastic syndrome underwent unrelated bone marrow transplantation following conditioning with fludarabine, busulfan, total body irradiation, and anti-thymocyte globulin. She received tacrolimus and short-term methotrexate for graft-versus-host disease (GVHD) prophylaxis. After engraftment, she developed acute GVHD involving the skin, gut, and liver. Even after treatment with glucocorticoids, human mesenchymal stem cells, and ruxolitinib, skin GVHD progressed and caused extensive epidermolysis and erosions with persistent bleeding. The patient was started on a daily skin care regimen, which included washing, application of dimethyl isopropylazulene and betamethasone ointment, and topical trafermin and hydrogel wound dressing for bleeding sites. A multi-disciplinary team consisting of hematologists, plastic surgeons, and nurses, and physical therapists, psychiatrists/clinical psychologists, and palliative care providers for physical, mental, and pain supportive care managed the patient's care. After 4 months of treatment under this team, complete epithelial regeneration was achieved. This case demonstrates the efficacy of local skin care and multi-disciplinary collaboration in acute GHVD causing extensive skin damage.
Download full-text PDF |
Source |
---|---|
http://dx.doi.org/10.11406/rinketsu.66.100 | DOI Listing |
Rinsho Ketsueki
March 2025
Department of Hematology, Tokyo Medical and Dental University Hospital.
A 41-year-old woman with myelodysplastic syndrome underwent unrelated bone marrow transplantation following conditioning with fludarabine, busulfan, total body irradiation, and anti-thymocyte globulin. She received tacrolimus and short-term methotrexate for graft-versus-host disease (GVHD) prophylaxis. After engraftment, she developed acute GVHD involving the skin, gut, and liver.
View Article and Find Full Text PDFOral Dis
January 2025
Facultad de Odontología, Universidad de Chile, Santiago, Chile.
Introduction: Junctional epidermolysis bullosa (JEB) is a rare genetic disease manifesting with skin and mucosal blistering. As part of the JEB, patients present with syndromic amelogenesis imperfecta (AI). Reports have described external crown resorption (ECR) in the teeth of patients with JEB, but its prevalence is unknown.
View Article and Find Full Text PDFOrphanet J Rare Dis
December 2024
Department of Medical Genetics, Telemark Hospital Trust, Skien, Norway.
Background: Epidermolysis bullosa (EB) comprises a group of genetically and clinically heterogeneous diseases characterized by skin fragility and blistering. EB is incurable, and treatment consists of preventing blisters in addition to painful and time consuming skin care, often performed by the parents, in addition to monitoring other symptoms in cases of severe EB.
Results: The purpose of this study was to explore parental experiences of caring for a child with EB.
Int Wound J
December 2024
Student Research Committee, Isfahan University of Medical Sciences, Isfahan, Iran.
Toxic epidermal necrolysis (TEN) is a severe skin reaction caused by extensive epidermal and mucosal necrosis. This clinical phenomenon is known as an acute syndrome of apoptotic pan-epidermolysis (ASAP). The ASAP phenomenon is observed in conditions that mimic TEN, highlighting the challenge in distinguishing these conditions.
View Article and Find Full Text PDFMatrix Biol
February 2025
Department of Dermatology, Medical Center - University of Freiburg, Freiburg, Germany. Electronic address:
Integrin α6β4 subunits and type XVII collagen are critical transmembrane proteins involved in cell-matrix adhesion in skin, while laminin 332 serves as their ligand in the basement membrane zone (BMZ). Those proteins contribute to the composition of hemidesmosomes (HDs) and pathogenic variants in their corresponding genes cause junctional epidermolysis bullosa (JEB). Although the genotype-phenotype relationships in JEB have been extensively studied, the pathogenetic changes of extracellular matrix (ECM) and cell-matrix adhesion resulting from gene mutations remain unclear.
View Article and Find Full Text PDFEnter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!