mycophenolic-acid and Mouth-Diseases

Extracorporeal photopheresis (ECP) is a second-line therapy for steroid-refractory chronic graft-versus-host disease (cGVHD).. We describe the long-term efficacy and tolerability of ECP according to the cutaneous phenotype of cGVHD and report on the reduced need for immunosuppressant drugs in this setting.. Fourteen patients (8 females) with cutaneous and/or mucosal cGVHD, treated with ECP between October 2010 and May 2016 within a single center, were included. Final analyses included patients who had received ECP for at least 12 months. We prospectively evaluated the efficacy of ECP using lesion-specific clinical scores and by recording changed doses of systemic immunosuppressants.. Of the 14 patients, sclerotic skin lesions were present in 10 (71%). The mRODNAN score decreased in all patients from month 9 onwards, with 40 and 77% reductions at 12 and 36 months, respectively. Six patients (43%) presented with cutaneous lichenoid lesions: this score was reduced in all patients by month 3, reaching a 93% reduction by month 12. Five patients (36%) experienced oral mucosal lichenoid lesions: these scores were decreased by 55% at month 12 and by 100% by month 33. The use of systemic immunosuppressants was reduced in all patients; 4 patients could stop all immunosuppressant drugs after 2 years. ECP was stopped in 3 patients after a complete response. No major ECP-associated adverse effects were observed.. ECP was an effective long-term therapy for oral and cutaneous cGVHD: consequently, dose levels of therapeutic immunosuppression could be reduced.

Topics: Adult; Chronic Disease; Cyclosporine; Female; Graft vs Host Disease; Hematopoietic Stem Cell Transplantation; Humans; Immunosuppressive Agents; Lichenoid Eruptions; Male; Middle Aged; Mouth Diseases; Mouth Mucosa; Mycophenolic Acid; Photopheresis; Prednisone; Sclerosis; Skin; Tacrolimus

Pemphigus vulgaris (PV) is an autoimmune blistering skin disorder characterized by the presence of suprabasal acantholysis and autoantibodies against desmoglein 3. There are two different clinical forms: mucocutaneous (MCPV) and mucosal (MPV). However, little is known about PV lesions in oral, ear, nose and throat (OENT) areas produced by the very dynamic of the anatomical structures involved in the functions of the aerodigestive tract.. To investigate the pattern of OENT manifestations in PV, and their relationship with physiological traumatic mechanisms in stratified squamous epithelial structures.. A prospective analysis of 40 patients diagnosed with MCPV (22 patients) or MPV (18 patients) was carried out at the University Clinic of Navarra. OENT manifestations were evaluated in all patients endoscopically. OENT involvement was divided into anatomical areas.. The most frequent symptom was pain, mainly on oral mucosa (87·5%). Buccal mucosa (90%), posterior wall of pharynx (67·5%), upper edge of epiglottis (85%) and nasal vestibule (70%) were the areas most frequently affected in the OENT mucosa. These localizations were related to physiological traumatic mechanisms in polystratified squamous epithelial structures.. OENT endoscopy should be included in the examination of all patients with PV. Knowledge of the most frequent localizations of active lesions on OENT mucosa in PV will help us to interpret more efficiently the findings from OENT endoscopy. Also, information related to traumatic physiological mechanisms on OENT areas must be offered to patients in order to avoid the appearance of new active PV lesions.

Topics: Azathioprine; Drug Therapy, Combination; Ear Diseases; Endoscopy; Glucocorticoids; Humans; Immunosuppressive Agents; Middle Aged; Mouth Diseases; Mycophenolic Acid; Nose Diseases; Pemphigus; Pharyngeal Diseases; Prospective Studies; Severity of Illness Index

Graft-vs.-host-disease, or GVHD, is the main cause of morbidity in patients who have received bone marrow transplants. Chronic GVHD, or cGVHD, occurs 100 days or more after the transplant procedure and may take the form of various oral manifestations.. A 23-year-old woman received an allogeneic bone marrow transplant. Although prophylactic therapy was provided, the patient developed cGVHD. Appropriate therapy was initiated, and it received a good clinical response at all sites affected by cGVHD, except in the oral cavity. The patient received complete symptomatic relief through revised systemic therapy, improved oral hygiene, use of topical medications and a monitored diet.. Effective intervention by dentists is an important part of increasing treatment effectiveness and improving quality of life in patients who received bone marrow transplants.

Topics: Adult; Anemia, Aplastic; Anti-Inflammatory Agents; Betamethasone; Bone Marrow Transplantation; Chronic Disease; Cyclosporine; Female; Glucocorticoids; Graft vs Host Disease; Humans; Immunosuppressive Agents; IMP Dehydrogenase; Methotrexate; Mouth Diseases; Mouthwashes; Mycophenolic Acid; Oral Hygiene; Prednisone; Transplantation Conditioning; Transplantation, Homologous

Pemphigus vulgaris, or PV, is a potentially life-threatening illness that manifests itself initially in the mouth in the majority of patients. Paradoxically, it is less commonly recognized when it involves lesions on the oral mucosa rather than on the skin.. This article describes the clinical presentation of 42 cases of oral PV evaluated and diagnosed by dentists. Emphasis is placed on the common distribution and appearance of oral PV lesions and diagnosis of the disease.. The dentist has a unique opportunity to recognize the oral presentation of PV and contribute to an early diagnosis and, therefore, an improved treatment outcome.

Topics: Adult; Aged; Anti-Inflammatory Agents; Autoantibodies; Azathioprine; Corneal Ulcer; Cyclophosphamide; Female; Humans; Immunosuppressive Agents; Male; Middle Aged; Mouth Diseases; Mouth Mucosa; Mycophenolic Acid; Oral Ulcer; Pemphigus; Prednisolone