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Pemphigus vulgaris (PV) is a group of autoimmune bullous diseases characterized by life-threatening intradermal blisters. Hashimoto thyroiditis (HT) is a kind of autoimmune disease with abnormal increase of thyroid peroxidase autoantibody (TPOAb), which is the thyroid specific antibody, leading to hypothyroidism. In recent years, the probability of HT in patients with PV is increasing, and the co-disease may be related to the effect of TPOAb autoantibody on oral keratinocytes. This article reviews the epidemiological relationship between PV and HT and the mechanism of TPOAb in their co-disease, in order to provide ideas for the diagnosis and treatment of both.

Objective: To explore the diagnosis and treatment of acquired hemophilia A (AHA) based on the analysis of clinical data., Methods: A retrospective analysis was conducted on the clinical manifestations, laboratory characteristics, treatment, and outcomes of 25 patients diagnosed with AHA who were admitted to the Second Hospital of Hebei Medical University., Results: Among all patients, 11 cases had secondary factors, including 5 cases of autoimmune diseases, 3 cases of pregnancy-related disease, 1 case of pemphigoid, 1 case of Graves' disease, and 1 case of monoclonal gammaglobulinemia of unknown significance (MGUS). The bleeding sites include the skin, mucous membrane, muscle, joint cavity and brain tissue. Twenty-three patients were treated with prednisone combined with cyclophosphamide (CP regimen), one patient with rituximab combined with cyclophosphamide because of femoral head necrosis, and one case with rituximab monotherapy because of gastrointestinal bleeding after prednisone treatment. Among them, 23 cases achieved complete remission (CR), 2 cases were partial remission (PR), and 8 cases relapsed after CR. All of 10 patients including 2 cases with PR and 8 relapsed cases after CR were treated with rituximab. At last, 8 patients achieved CR, and 2 patients (both were patients with recurrence after first CR) achieved PR. These two patients achieving PR were treated with low-dose rituximab combined with bortezomib (RB regimen). One patient reached CR after 4 cycles and the other reached CR after 6 cycles of RB regimen. After CR, 4 of the 10 patients treated with rituximab received maintenance therapy with rituximab monotherapy for 1.5 to 2 years, in which, none of them relapsed. Among the 6 patients who did not receive maintenance therapy, 4 patients relapsed after CR, and the median time to relapse was 15 months. Eight patients treated with CP regimen developed common infections, and two patients treated with rituximab developed severe pneumonia. All 25 patients survived until the end of follow-up., Conclusion: Skin ecchymosis, mucous hemorrhage and muscle hematoma are the most common hemorrhagic manifestations in AHA, and joint hemorrhage and cerebral hemorrhage can also occur. CP regimen is the preferred option of first-line therapy for AHA. Rituximab can be used for patients with steroid contraindication or who failed to respond to the above therapy or relapsed after effective treatment, and maintenance therapy is recommended to reduce the risk of recurrence. Meanwhile, close attention should be paid to the occurrence of infection events during rituximab treatment. Rituximab in combination with bortezomib can also be used in patients with refractory or relapsing AHA.

Drug-induced pemphigus (DIP) is a special type of pemphigus, and its pathogenesis, characteristics of treatment, and prognosis are closely related to the inducing drugs. This article reports the diagnosis and treatment of DIP (pemphigus vulgaris) caused by the administration of rifampin to a patient with tuberculosis. Combined with the literature, we discussed the types, pathogenesis, differential diagnosis, and treatment principles of DIP. We propose that in the oral clinical practice for patients with pemphigus vulgaris, the importance of investigating suspected drugs that induce DIP should be emphasized.

Since the discovery of Epstein-Barr (EB) virus from the cultured cells in Burkitt lymphoma tissue in African children in 1964, we have gradually taken in its oncogenicity and the link between this virus and multiple mucocutaneous diseases. This review exhibits the process of EB virus infection and summarizes the clinical manifestations and varied prognosis of EB virus-associated skin diseases. The cases of common skin diseases including psoriasis, dermatomyositis, pemphigus, pemphigoid, parapsoriasis, atopic dermatitis, urticaria, systemic lupus erythematosus, scleroderma, panniculitis,erythema multiforme and vasculitis coinciding with EB virus infection were collected for the analysis of their EB virus-associated complications, treatments and outcomes.

Objectives: The causal relationship between eczema and autoimmune diseases has not been previously reported. This study aims to evaluate the causal relationship between eczema and autoimmune diseases., Methods: The two-sample Mendelian randomization (MR) method was used to assess the causal effect of eczema on autoimmune diseases. Summary data from the Genome-Wide Association Study Catalog (GWAS) were obtained from the Integrative Epidemiology Unit (IEU) database. For eczema and autoimmune diseases, genetic instrument variants (GIVs) were identified according to the significant difference ( P <5×10 -8 ). Causal effect estimates were generated using the inverse-variance weighted (IVW) method. MR Egger, maximum likelihood, MR-PRESSO, and MR-RAPS methods were used for alternative analyses. Sensitivity tests, including heterogeneity, horizontal pleiotropy, and leave-one-out analyses, were performed. Finally, reverse causality was assessed., Results: Genetic susceptibility to eczema was associated with an increased risk of Crohn's disease ( OR =1.444, 95% CI 1.199 to 1.738, P <0.001) and ulcerative colitis ( OR =1.002, 95% CI 1.001 to 1.003, P =0.002). However, no causal relationship was found for the other 6 autoimmune diseases, including systemic lupus erythematosus (SLE) ( OR =0.932, P =0.401), bullous pemphigoid (BP) ( OR =1.191, P =0.642), vitiligo ( OR =1.000, P =0.327), multiple sclerosis (MS) ( OR =1.000, P =0.965), ankylosing spondylitis (AS) ( OR =1.001, P =0.121), rheumatoid arthritis (RA) ( OR =1.000, P =0.460). Additionally, no reverse causal relationship was found between autoimmune diseases and eczema., Conclusions: Eczema is associated with an increased risk of Crohn's disease and ulcerative colitis. No causal relationship is found between eczema and SLE, MS, AS, RA, BP, or vitiligo.

Objective: To assess the value of immunohistochemical analysis for expressions of C3d, C4d, IgG, IgG4, and CD123 in the diagnosis of autoimmune skin diseases.
 Methods: We investigated the expressions of C3d, C4d, IgG, IgG4, and CD123 in paraffin-embedded, formalin-fixed tissues from 27 lupus erythematosus cases, including 8 discoid lupus erythematosus (DLE) cases, 4 subacute cutaneous lupus erythematosus (SCLE) cases, and 15 systemic lupus erythematosus (SLE) cases. Tissues from 15 dermatomyositis (DM) cases, 15 bullous pemphigoid (BP) cases, and 15 pemphigus cases were examined by immunohistochemical analysis. The differences in expression rates of C3d, C4d, IgG, IgG4, and CD123 between immunohistochemical staining and direct immunofluorescence were compared in the diagnosis of these diseases.
 Results: In the lupus erythematosus group, the positive rates of C3d and C4d deposited along the dermoepidermal junction were 85.2% and 51.9%, respectively. In the dermatomyositis group, the positive rates of C3d and C4d deposited along the dermoepidermal junction were 40% and 0, respectively. The expressions of C3d and C4d in lupus erythematosus tissues were significantly higher than those in DM tissues (P<0.05). The expression of CD123 protein in skin lesions of the lupus group was significantly higher than that in the DM group (P<0.05). In the BP group, the positive rates of C3d and C4d deposited along the dermoepidermal junction were 100% and 86.7%, respectively. In the pemphigus group, the positive rates of C3d and C4d deposited in the intercellular space of keratinocytes were 100% and 60%, respectively. The expressions of IgG and IgG4 in pemphigus tissues were higher than those in BP tissues (P<0.05). And the ratios of IgG4 to IgG in the pemphigus group was significantly higher than that in the BP group (P<0.05).
 Conclusion: The assays of C3d and C4d define an important diagnostic adjunct in evaluation of lupus erythematosus, BP and pemphigus. In some cases, it may even replace the direct immunofluorescence as a diagnostic adjunct. The expression of CD123 possesses certain clinical significance for the differential diagnosis of lupus erythematosus, and IgG4 and IgG expressions have adjunctive diagnostic significance for pemphigus.

Objective: To determine clinical curative effects of ozone therapy for pemphigus vulgaris.
 Methods: Ozone hydrotherapy was used as an aid treatment for 32 patients with pemphigus vulgaris. The hydropathic compression of potassium permanganate solution for 34 patients with pemphigus vulgaris served as a control. The main treatment for both groups were glucocorticoids and immune inhibitors. The lesions of patients, bacterial infection, usage of antibiotics, patient's satisfaction, and clinical curative effect were evaluated in the 2 groups.
 Results: There was no significant difference in the curative effect and the average length of staying at hospital between the 2 groups (P>0.05). But rate for the usage of antibiotics was significantly reduced in the group of ozone hydrotherapy (P=0.039). The patients were more satisfied in using ozone hydrotherapy than the potassium permanganate solution after 7-day therapy (P>0.05).
 Conclusion: Ozone hydrotherapy is a safe and effective aid method for pemphigus vulgaris. It can reduce the usage of antibiotics.

Paraneoplastic pemphigus is a rare autoimmune bullous dermatosis, which is caused by potential neoplasm, especially the Castleman's disease. Castleman's disease associated with paraneoplastic pemphigus is misdiagnosed frequently and easily in clinical practices. Furthermore, it is reported that the mortality rate for this disease is very high. Bronchiolitis obliterans is the most common complication and the most important cause of death. There was a female patient presenting recalcitrant mucocutaneous erosions, ulcers and scattered erythemas in the body. The patient was diagnosed and treated for pemphigus vulgaris with little success in Xiangya Hospital, Central South University in January 2015. Further investigations confirmed the diagnosis of paraneoplastic pemphigus with retroperitoneal tumor. Subsequently, the patient was treated with tumor resection in combination with intravenous immunoglobulin and corticosteroids. The pathology revealed that it was the Castleman's disease. Her mucocutaneous performance recovered obviously and the bronchiolitis obliteran did not appear in the follow-up. Castleman's disease associated with paraneoplastic pemphigus should be considered when mucosal and skin lesions showing no improvement under corticosteroids. Early and complete removal of the tumor together with immunotherapy could be beneficial to the patient's prognosis.

The patient is a 73-year-old female who developed bilateral corneal ulcers one month after cataract surgery in her left eye. The diagnosis is bilateral conjunctival pemphigoid. She underwent a left-eye amniotic membrane transplant and a right-eye lamellar corneal transplant, and was treated with oral immunosuppressants. The patient's condition is stable.

Objective: To study the etiological, clinical, radiological, diagnostic, therapeutic, and prognostic manifestations of exogenous lipoid pneumonia (ELP), and therefore to improve the diagnosis and treatment of this disease. Methods: The clinical data of 12 cases of ELP confirmed by pathology were retrospectively analyzed. Results: The patients consisted of 9 males and 3 females, with an average age of 73.8 years (range, 44 to 100 years). The underlying diseases were variable, including diseases affecting the general condition (multiple organ failure, chronic heart and renal insufficiency, pemphigoid, etc) and conditions with increased risk of aspiration (sequelae of cerebrovascular disease, Alzheimer's disease, advanced stage of nasopharyngeal carcinoma, paralytic ileus, etc). The 12 cases were all caused by Inhalation of mineral oil. Common symptoms included cough, sputum production and dyspnea. ELP had no special physical signs. Inflammation indexes, such as white blood cell, neutrophil percentage, ESR, C reactive protein, procalcitonin, D-Dimer, and blood lipid levels were usually normal. Radiological features of ELP mainly included consolidation, mass or nodules, with a little ground-glass opacity. Some patients had ventilation and/or diffusion dysfunction. The diagnostic methods included CT-guided percutaneous lung biopsy, thoracoscopy, thoracotomy or autopsy. Histopathological findings showed accumulation of large foamy macrophages in the alveolar spaces, with a few lipid deposition and polykaryocytes. The main treatment of ELP was cessation of lipid material contact. One case died of ELP, 6 died of other coexisting diseases, and the rest 6 improved with treatment and were discharged. The survival patients were all stable during a follow-up of 2-4.5 years. Conclusions: ELP was rare and its clinical manifestation was atypical. Its radiological manifestations were indistinguishable from pneumonia, lung cancer, interstitial lung diseases, etc. Pathological examination was the gold standard for diagnosis, and the preferred means of sampling was bronchoscopy. In cases whose diagnosis could not be confirmed by BALF, CT-guided percutaneous lung biopsy might be considered. The most important treatment is cessation of lipid material contact. The prognosis is good.

Objective: To investigate the clinicopathological features of acantholytic mammary Paget's disease (AMPD). Methods: From January, 2010 to October, 2016, a total of 28 patients were diagnosed as AMPD in the Department of Dermatology, Peking Union Medical College Hospital. The clinical and histopathological data of these patients were analyzed retrospectively. Results: The patients were all female. The mean age of onset was (51±15)years (range, 24 to 78 years). The median course of disease was 10.5 months (range, 3 months to 2 years). All the cases were unilaterally involved. The ratio of left breast involvement to the right breast involvement was 1.55∶1. In histopathological examination, all the cases showed acantholytic pattern. In addition to that, the prototypical pattern accounted for 50.0% (14/28) of all the AMPD cases. The frequencies of other different accompanied histopathological patterns were: 3 (10.7%) upper nest, 2 (7.1%) budding, 1 (3.6%) tall nest, and 1 (3.6%) sheet-like. Hailey-Hailey-disease-like acantholysis was observed in 18 patients (64.3%), whereas pemphigus-vulgaris-like acantholysis was noted in 7 patients (25.0%) and Darier's-disease-like acantholysis in 3 patients (10.7%). About 75.0% (21/28) of the AMPD cases were found to be accompanied with underlying breast cancers. There was no recurrence in 18 of 20 patients who completed treatment and were followed up for over 1 year. Conclusions: AMPD might involve the left breast more frequently. It is mostly associated with the prototypical pattern of mammary Paget's disease. Hailey-Hailey-disease-like acantholysis may be the most frequent subtype of AMPD. Most AMPD cases are associated with underlying breast cancers, but with a low recurrence rate after treatment.