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Mucous membrane pemphigoid is a medically and immunopathologically heterogenous disease with a prevalence of around 2% residents/year in central Europe. MMP has been referred to as a pemphigoid disease with predominant mucosal lesions. According to the MMP patients, BP180, laminin 332, BP230, and type VII collagen are all targets. Although the main autoantibody is still IgG, additional, and less often, unique IgA autoantibodies can be detected in the majority of patients. Although the primary autoantibody is still IgG. Since a quarter of patients with anti-laminin 332 MMP, a malignancy, mainly solid tumors, is related, assaying for anti-laminin 332 reactivity is pivotal, in about a quarter of patients with anti-laminin 332 MMP, a malignancy, mainly solid cancers, is linked in about a quarter of patients. A new mouse model of anti-laminin 332 MMP replicating key clinical and immunopathological findings of the human disease may be useful in closing the knowledge gap. Diagnosis is established by the clinical picture with the majority mucosal lesions and the identification of tissue-bound anti-basement membrane zone antibodies by direct immunofluorescence microscopy.
Source link: https://europepmc.org/article/MED/34995762
Purpose The aim of this research was to publish the clinical course and findings of patients with refractory ocular mucous membrane pemphigoid treated by repository corticotropin injection. Patients with biopsy-proven ocular MMP treated with RCI from three tertiary medical centers were evaluated. At presentation, the majority of the patients had Foster stage 3 disease, and all patients had active MMPs. Following the use of RCI, 9 patients had medical failure of at least two other agents during the follow-up, and 9 patients had treatment failure of at least two other agents. In any of the 15 patients at the last follow-up, the Foster stage did not change in any of the 15 patients. At the last follow-up, nine patients continued RCI therapy, and in all of them, the disease production of MMP was well controlled. Conclusions RCI can be used as an alternative or an adjunctive therapy in patients with severe and refractory ocular MMP.
Source link: https://europepmc.org/article/MED/34050065
History of mucous membrane pemphigoid disorders can cause a variety of medical and surgical delays. Objection To identify the characteristics of a large number of patients with MMP. Methods A retrospective analysis of medical and surgical characteristics as well as treatment results in 145 patients with MMP include a retrospective analysis of clinical and diagnostic characteristics as well as treatment responses. Result Monosite involvement was seen in 44. 4 percent and multisite participation in 56. 6 percent of the patients, with 58. 6%. In 41. 7% of patients with no apparent mucosal site involvement at the time, Ocular disease developed during the disease course in 41. 7% of patients with no initial mucosal site involvement. Patients with autoantibodies against laminin-332 were significantly higher in patients with autoantibodies against laminin-332 than in patients with MMP without laminin-332 autoantibodies. Patients with MMP have a heterogeneous clinical appearance, and new signs may develop during the disease course. Patients with MMP and, in particular, those with autoantibodies against laminin-332 should be screened for cancer screening.
Source link: https://europepmc.org/article/MED/34896128
Methods: We selected 133 anti-LM332-type MMP patients, all of whom were identified by our unique inclusion criteria: immunoglobulin G deposition to basement membrane zone by direct immunofluorescence or IgG reactivity on the dermal side of split skin by ssIIF, with positivity for at least one of the three subunits of LM332 by immunoblotting of purified human LM332 by immunoblotting of purified human LM332 by immunoglobulin MMP case report 133 anti-LM332-type MMP patients with re LM332 by re chemad's, re reactive immunoblotting of re reactivity on ret rete deposition of re reprot ret ete si LM33221 LM33297017332-32-in LM332-typesyrè LM332'setete LM332'sy LM332-typesteda The sensitivities of ssIIF and direct immunofluorescence are similar, but indirect immunofluorescence using non-split human skin is much higher than indirect immunofluorescence. Conclusions: The new diagnostic criteria without positive direct immunofluorescence reactivity were useful for diagnosis in this retrospective review. In one-third of the patients, additional autoantibodies to non-LM332 autoantigens may have contributed to the patient's difficulty in anti-LM332-type MMP design, which also included the production of anti-LM2 antibodies.
Source link: https://europepmc.org/article/MED/34899722
Against laminin 332, an autoimmune blistering disease with a predominant mucosal lesions and autoantibodies is characterized by laminin 332's autoimmune blistering disease. This review compared two independently developed diagnostic indirect immunofluorescence tests based on recombinant laminin 332 expressed in HEK239 cells and the migration trails of cultured keratinocytes rich in laminin 332 genes. The sera of 54 anti-laminin 332 MMP, 35 non-anti-laminin 332 MMP, 35 non-anti-laminin 332 MMP, 35 non-anti-laminin 332 MMP, 30 pemphi vulgaris patients, as well as 20 healthy blood donors were analyzed blindly and independently. According to say, the biochip mosaic and the footprint were in the positive, with 54 and 54/54 anti-laminin 332 MMP sera.
Source link: https://europepmc.org/article/MED/34899726
Importance Mucous membrane pemphigoid is a rare, heterogeneous subepithelial bullous disease that causes headaches. MMP patients undergoing continuous MMP from January 2007 to February 2020 in two tertiary referral centers in Germany. The aim, structure, and participants A retrospective cohort study encompassing all prospective patients diagnosed with MMP from January 2007 to February 2020 in two tertiary referral centers in Germany. MMP patients were evaluated and analyzed by 154 patients, of whom 125, 61, 34, and 16 were found with lesions involving the oral, ocular, nasal, and genital mucosae, respectively, and 35 were cutaneously affected. Ocular disease was inversely linked to oral and nasal exposure, and it was accompanied by a 13-fold increased risk of malignant neoplasm. Conclusions and relevance In this multi-year study of patients with MMP, malignant neoplasms, and anti-laminin 332 reactivity, indicating potential benefit of malignant neoplasm screening in these patients, MMP, malignant neoplasms were associated with ocular disease and anti-laminin 332 reactivity, indicating potential benefits of malignant neoplasm screening.
Source link: https://europepmc.org/article/MED/34817539
Conclusion and conclusion Our study focuses on previously reported cases of pemphigoid in childhood that involved solely genital involvement as well as a summary of mucous membrane pemphigoid infections in childhood. There seems to be a distinct type of pemphigoid predominantly impacting girls with heavy vulvar involvement and good prognosis. To diagnosis a mucous membrane pemphigoid, dermatologtologic analysis and a skin biopsy with direct immunofluorescence are the key.
Source link: https://europepmc.org/article/MED/34740758
Mucous membrane pemphigoid is one of the few cases of heterogeneous chronic autoimmune diseases that usually appear as blistering of the mucous membranes. MMP lesions often heal with scarring, which may result in poor organ function and severe morbidity. This comprehensive literature review highlights the current state and recent advancements in the field of MMP control, with a particular emphasis on the oral version of MMP.
Source link: https://europepmc.org/article/MED/34704944
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