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Italian Journal of Dermatology and... Feb 2024The oral mucosa can be involved in a wide variety of mucocutaneous conditions that may present primarily in the mouth or affect other cutaneous or mucosal sites. Many of... (Review)
Review
The oral mucosa can be involved in a wide variety of mucocutaneous conditions that may present primarily in the mouth or affect other cutaneous or mucosal sites. Many of these conditions are immune mediated and typically present as inflammatory mucosal pathology. Patients experiencing such conditions usually seek medical evaluation and treatment due to the associated pain and discomfort, and occasionally taste disturbance or dysphagia and the overall deterioration in the oral health-related quality of life. These conditions share some common features and there could be some overlap in their clinical presentation, which can lead to delays in diagnosis and proper management of patients. Clinicians dealing with such disorders, including dermatologists, need to be aware of the oral manifestations of mucocutaneous conditions, their clinical features, underlying mechanisms, diagnostic approaches, and treatment options, as well as the recent advances in the research on these conditions. This review provides a comprehensive, evidence-based reference for clinicians, with updated insights into a group of immune mediated conditions known to cause oral mucosal pathology. Part one will cover oral lichen planus, erythema multiforme and systemic lupus erythematosus, while part two will cover recurrent aphthous stomatitis, pemphigus vulgaris and mucous membrane pemphigoid, in addition to the less common disorders linear IgA disease, dermatitis herpetiformis and epidermolysis bullosa.
Topics: Humans; Mouth Mucosa; Mouth Diseases; Quality of Life; Pemphigus; Stomatitis, Aphthous
PubMed: 38345290
DOI: 10.23736/S2784-8671.23.07690-9 -
Frontiers in Immunology 2024[This corrects the article DOI: 10.3389/fimmu.2023.1193032.].
[This corrects the article DOI: 10.3389/fimmu.2023.1193032.].
PubMed: 38322255
DOI: 10.3389/fimmu.2024.1331003 -
Medicine Jan 2024Psoriasis is an immune-related disease caused by genetic factors, abnormalities in the immune system and environmental factors, while pemphigus is an autoimmune disease...
RATIONALE
Psoriasis is an immune-related disease caused by genetic factors, abnormalities in the immune system and environmental factors, while pemphigus is an autoimmune disease caused by the autoimmune system attacking the skin and mucosal tissues. Herein, we aimed to report a rare case of adalimumab induced exacerbation of psoriasis patients with pemphigus. The rare disease causes considerable challenges for clinical diagnosis and treatment.
PATIENT CONCERNS
The patient was a 43-year-old man with intermittent erythema and scaling all over the body for more than 20 years, and blisters and vesicles on the trunk and limbs for 1 month. Half a year ago, the patient had blisters on the limbs, and was diagnosed with deciduous pemphigus in a hospital, and the blisters subsided after being given traditional Chinese medicine orally. Half a month ago, the erythema area was enlarged, and adalimumab 80 mg intramuscular injection was given for 1 time after consultation in the hospital. On the following day, the area of erythema and scales was suddenly enlarged obviously compared with the previous 1, and obvious blisters and vesicles appeared on the limbs, neck, and trunk, which were aggravated progressively and accompanied by obvious itching and pain.
DIAGNOSES
The patient was diagnosed with psoriasis in patients with combined pemphigus.
INTERVENTION
After combined treatment with methylprednisolone and cyclosporine, the skin lesions have basically recovered.
OUTCOMES
The skin lesions have basically healed. Follow up for 6 months without recurrence.
LESSONS
Methylprednisolone combined with cyclosporine may be an option in treating patients with psoriasis patients with pemphigus.
Topics: Male; Humans; Adult; Pemphigus; Adalimumab; Blister; Psoriasis; Methylprednisolone; Erythema; Cyclosporine
PubMed: 38277534
DOI: 10.1097/MD.0000000000036988 -
Sisli Etfal Hastanesi Tip Bulteni 2023Koebner phenomenon of skin diseases due to face masks have been reported since COVID-19 pandemic, especially in psoriasis patients. Although there are reports on Koebner...
Koebner phenomenon of skin diseases due to face masks have been reported since COVID-19 pandemic, especially in psoriasis patients. Although there are reports on Koebner phenomenon in pemphigus patients in the literature, pemphigus lesions triggered by face masks have not been described previously. Herein, we report one case of pemphigus vulgaris and one case of pemphigus vegetans with new and persistent lesions on the nose following prolonged use of face masks. Both cases had persistent pemphigus lesions on their noses where face masks irritated the most. The development of lesions after the use of masks and the persistence of nasal lesions despite the improvement of other skin lesions with the treatment in both cases, suggested that minor traumas due to the use of masks played a role in the formation of lesions.
PubMed: 38268656
DOI: 10.14744/SEMB.2023.38107 -
Frontiers in Medicine 2023The success of immunotherapy has made it a lifesaving treatment, but not without side effects. Currently, the risk factors for developing immune-related adverse events...
BACKGROUND
The success of immunotherapy has made it a lifesaving treatment, but not without side effects. Currently, the risk factors for developing immune-related adverse events (irAEs) in patients who receive immunotherapy are poorly understood, and there is no risk-stratifying mechanism for potentially fatal irAEs. It is postulated that oncology patients with preexisting autoimmune diseases are likely to have flares on immunotherapy. However, some patients develop autoimmune conditions on immunotherapy without a prior history. Literature reports have postulated that human leukocyte antigen (HLA) inherence may play a role in irAEs. However, this potential remains underexplored.
METHODS
The oncology patients who developed autoimmune adverse events on immunotherapy for whom the continuation of treatment was prudent or lifesaving were selected. Of note, all nine patients received checkpoint inhibitors (CIs). Of the nine selected patients, only one had a prior history of an autoimmune condition. None of the nine selected patients had an active autoimmune condition at the time of CI initiation. Their HLA was typed, and the results were cross-referenced with the literature reports in PubMed and Google search with the corresponding autoimmune condition of each patient.
RESULTS
Herein, we report nine patients with irAEs for whom retrospective HLA typing revealed the inherence of multiple related HLA alleles that may correspond to the autoimmune condition that they had developed on immunotherapy. It is to be mentioned that the inherence of enriched disease-related HLA alleles was shared among patients with the same irAEs. These patients developed a range of irAEs including bullous pemphigoid, pemphigus foliaceus/vulgaris, thyroiditis, vitiligo, and hepatitis on immunotherapy. Although some combinations of disease-related HLA were well reported in otherwise idiopathic autoimmune diseases, a frequently repeated HLA allele combination in our patient population was found to be rarely seen in the general population.
CONCLUSION
The authors suggest that an enriched inherence of disease-related HLA alleles may play a role in the genetic propensity for the development of irAEs in oncology patients, who receive immunotherapy, including CIs. Inherence of more than one or a cluster of particular autoimmune disease-related HLA alleles in patients who receive immunotherapy may unmask the corresponding autoimmune disease as the genotype inherence presents with the phenotype of the corresponding condition. It is suggested that enriched linked HLA genotypes, which are otherwise rare in the general population, may present as the corresponding phenotype of the autoimmune condition. Such clinical presentation, enhanced by immunotherapy, such as CIs, can play a role in risk stratifying patients for precision medicine and improve the outcome.
PubMed: 38259857
DOI: 10.3389/fmed.2023.1288844 -
Medicina (Kaunas, Lithuania) Jan 2024: Desquamative gingivitis (DG) is a clinical term indicating "peeling gums" and is associated with different oral manifestations. In this study, we aimed to assess the...
: Desquamative gingivitis (DG) is a clinical term indicating "peeling gums" and is associated with different oral manifestations. In this study, we aimed to assess the association between DG and autoimmune blistering mucocutaneous diseases (ABMD) with oral manifestations. : A retrospective study including 88 patients diagnosed between 1998 and 2019 with ABMD (intraepithelial and subepithelial autoimmune blistering diseases) was performed at the Oral Medicine Department, Faculty of Dentistry, "Carol Davila" University of Medicine and Pharmacy in Bucharest. For each patient, the sociodemographic and anamnestic data, as well as clinical features of oral lesions (location), histological evaluation, and direct immunofluorescence data were collected. : Most of the patients involved in the study were female (78.4%). In total, 34 patients (38.63%) were diagnosed with subepithelial autoimmune diseases (SAD) and 54 (61.36%) had intraepithelial autoimmune diseases (IAD). Differences in the anatomic distribution of oral involvement were found between SAD and IAD. The presence of DG was significantly more common in patients with SAD compared to those with a diagnosis of IAD. : Specific anatomical locations of the oral lesions are significantly associated with different subtypes of ABMD, with gingiva and hard palate mucosa being more involved in SAD and the soft palate and buccal mucosa in IAD. Desquamative gingivitis is a clinical sign that raises diagnostic challenges for several conditions in oral medicine.
Topics: Humans; Female; Male; Gingiva; Retrospective Studies; Mouth Mucosa; Autoimmune Diseases; Chronic Disease; Gingivitis
PubMed: 38256427
DOI: 10.3390/medicina60010167 -
Life (Basel, Switzerland) Dec 2023Pemphigus foliaceus (PF) is an autoimmune skin blistering disease characterized by antidesmoglein-1 IgG production, with an endemic form (EPF) in Brazil. Genetic and...
Genetic Association and Differential RNA Expression of Histone (De)Acetylation-Related Genes in Pemphigus Foliaceus-A Possible Epigenetic Effect in the Autoimmune Response.
Pemphigus foliaceus (PF) is an autoimmune skin blistering disease characterized by antidesmoglein-1 IgG production, with an endemic form (EPF) in Brazil. Genetic and epigenetic factors have been associated with EPF, but its etiology is still not fully understood. To evaluate the genetic association of histone (de)acetylation-related genes with EPF susceptibility, we evaluated 785 polymorphisms from 144 genes, for 227 EPF patients and 194 controls. Carriers of were more susceptible (OR = 1.79, = 0.0038), whereas those with (OR = 0.57, = 0.0011) and homozygotes for (OR = 0.39, = 0.0006) were less susceptible to EPF. These variants were not associated with sporadic PF (SPF) in German samples of 75 SPF patients and 150 controls, possibly reflecting differences in SPF and EPF pathophysiology. We further evaluated the expression of histone (de)acetylation-related genes in CD4 T lymphocytes, using RNAseq. In these cells, we found a higher expression of , and and lower expression of and in patients with active EPF without treatment compared to controls from endemic regions. The encoded proteins cause epigenetic modifications related to immune cell differentiation and cell death, possibly affecting the immune response in patients with PF.
PubMed: 38255677
DOI: 10.3390/life14010060 -
Frontiers in Medicine 2023Cutaneous manifestations of hematologic malignancy represent both a clinical challenge for the treating physician and a pathophysiological model for advancing the... (Review)
Review
Cutaneous manifestations of hematologic malignancy represent both a clinical challenge for the treating physician and a pathophysiological model for advancing the knowledge on individual neoplasms. Indeed, a growing body of evidence supports the concept of recurrent molecular defects associating with specific clinical features, as best exemplified by VEXAS. Herein neutrophilic and eosinophilic dermatoses of potential interest for both hematologists and dermatologists will be reviewed, including subcorneal pustular dermatosis-type IgA pemphigus, neutrophilic eccrine hidradenitis, Sweet's syndrome as well as myelodysplasia cutis and VEXAS, pyoderma gangrenosum, eosinophilic annular erythema, eosinophilic dermatosis of hematological malignancy, Wells syndrome and cutaneous involvement in hypereosinophilic syndromes. Possible management approaches are discussed for each, emphasizing scenarios that require treatment of the underlying condition to achieve remission at the skin level.
PubMed: 38249974
DOI: 10.3389/fmed.2023.1324258 -
Antibodies (Basel, Switzerland) Jan 2024Rituximab is currently approved for patients affected by moderate-to-severe pemphigus vulgaris, a severe autoimmune blistering skin disease that can be life-threatening....
Rituximab is currently approved for patients affected by moderate-to-severe pemphigus vulgaris, a severe autoimmune blistering skin disease that can be life-threatening. The standard rituximab dosing regimens, originally established for B-cell non-Hodgkin's lymphomas, have been recognized to exceed the effective dose required for inducing B-cell depletion, considering that the B-cell burden in pemphigus vulgaris is considerably lower than in lymphoproliferative disorders. We herein report our experience with very ultra-low-dose rituximab in two patients affected by pemphigus vulgaris.
PubMed: 38247568
DOI: 10.3390/antib13010004