暂无摘要。

COVID-19 has been implicated in various cutaneous autoimmune diseases. Pemphigus is a group of autoimmune blistering diseases that target the desmosomal complexes. Pemphigus triggered by COVID-19 has been seldom reported in the literature and remains both a diagnostic and therapeutic challenge. We report a case of COVID-19-induced pemphigus that responded well to prednisone and mycophenolate mofetil after 9 months from initial presentation. On histologic examination, both intercellular and basement membrane staining were noted. Indirect immunofluorescence staining was positive against the intercellular cement of the stratified epithelium from monkey esophagus. We hypothesize that COVID-19 stimulated the release of multiple pemphigus antigens, which resulted in the unusual histologic pattern reported in the present case. Although malignancy should be suspected when features of paraneoplastic pemphigus, such as basement membrane staining on direct immunofluorescence, are noted, it may also be a histologic pattern of pemphigus secondary to COVID-19 that clinicians may consider.

Pemphigus vulgaris (PV) is a chronic autoimmune bullous disease that is characterized by mucocutaneous blister formation resulting in painful erosions. The autoantibody immunoglobulin (Ig) G directed toward glycoproteins desmoglein (Dsg) 3 and desmoglein 1 is the main underlying mechanism behind PV leading to intraepithelial clefting and bulla formation. Patients usually present with oral ulcers causing severe pain and dysphagia that can be misdiagnosed as erythema multiforme (EM) or viral infections. The diagnostic process requires the correlation between clinical, histopathological, and immunopathological findings. Systemic and/or local corticosteroids are considered the cornerstone therapy of PV cases. This article describes a case of a 42-year-old male patient who presented in the Department of Oral Medicine and Radiology with chronic oral ulcers that were diagnosed with PV and treated using systemic corticosteroids.

Cutaneous vesiculobullous eruptions associated with multiple myeloma (MM) are rare. Although the development of blisters is mostly driven by amyloid deposits of paraproteins in the skin, autoimmunity may play a role. In this study, we report an unusual case of an MM patient with blisters who presented with both flaccid and tense vesicles and bullae. Direct immunofluorescence revealed IgA autoantibody deposits in the basement membrane zone (BMZ) and intercellular space of the epidermis, which displayed an atypical autoantibody deposition pattern. The patient showed rapid disease progression and died during follow-up. We performed a literature review of autoimmune bullous diseases (AIBDs) associated with MM or its precursors and found 17 previously reported cases. Together with the present case, cutaneous involvement of the skin folds was frequently reported, and mucous membranes were barely affected. IgA pemphigus, with consistent IgA monoclonality, was observed in half of the cases. Five patients displayed atypical autoantibody deposition patterns in the skin; the prognosis of these patients appeared to be poorer than that of other patients. We aim to increase our understanding of AIBDs associated with MM or its precursors.

Pemphigus and lupus erythematosus are both B-cell-mediated autoimmune diseases, dependent on autoreactive CD4+ T lymphocytes to modulate autoimmune B-cell response. Many forms of pemphigus have been reported to occur in association with systemic lupus erythematosus (SLE) as well as other autoimmune diseases. However, it remains unclear whether this association occurs because of a shared immunopathogenesis or the coexistence may be coincidental. We hereby present a case report of discoid lupus erythematosus and paraneoplastic pemphigus associated with marginal zone lymphoma in a 54-year-old Thai man who had persistent oral erosions for 1 year together with generalized polymorphic cutaneous eruptions for 2 months. Simultaneous occurrence of paraneoplastic pemphigus and discoid lupus erythematosus without SLE has never been reported in the same individual. Hydroxychloroquine, immunosuppressive agents including prednisolone and azathioprine together with chemotherapy were given to treat these conditions.

暂无摘要。

Autoimmune bullous diseases (AIBDs), presenting cutaneous and/or mucosal bullous lesions, are classified into pemphigus and pemphigoid diseases. A longtime observation for complicated AIBD cases is rarely reported. In this study, serum samples of one AIBD patient were collected at seven different time points during the disease course including a relapse, which were examined by our conventional and newly developed methods for the detection of autoantibodies. Interestingly, we found changes of both the presence and the titers of various autoantibodies in accordance with the changes of clinical features during the whole disease course, which indicated that the patient started as bullous pemphigoid and relapsed as concurrence of bullous pemphigoid and mucosal-dominant-type pemphigus vulgaris.

暂无摘要。

Antigenic competition in the skin is a phenomenon in which the current dermatitis is distributed away from the area of previously existing dermatitis. Bullous pemphigoid may present such phenomenon, even if the responsible antigen was the same.

UNASSIGNED: Little data are available concerning the outcome of rituximab (RTX) therapy in pediatric patients with autoimmune bullous diseases (AIBDs).
UNASSIGNED: We sought to evaluate safety and efficacy of RTX administration in pediatric patients with AIBDs and to assess first-line RTX therapy in pemphigus patients.
UNASSIGNED: AIBD patients consisting of 12 pemphigus patients and a patient with bullous pemphigoid who received RTX before the age of 18 were enrolled. Detailed information regarding patients\' outcome after the first RTX cycle was assessed.
UNASSIGNED: The mean age of the patients at RTX infusion was 15 ± 2 years. Six patients in the pemphigus group received RTX as first-line therapy. In pemphigus patients: complete remission (on minimal therapy) was achieved by seven patients, partial remission (on minimal therapy) and complete remission (off therapy) were achieved by three patients and one, respectively. Relapse occurred in nine patients, which were mostly mild. Likewise, the BP patient received RTX with a good clinical response. The observed adverse events were mostly mild infusion reactions and a case of sepsis.
UNASSIGNED: Rituximab is safe and effective in childhood/juvenile patients with AIBDs. Furthermore, RTX can be used as first-line treatment in pediatric patients with pemphigus.