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Objective To investigate the clinical features of unicentric Castleman's disease(UCD)with paraneoplastic pemphigus(PNP)and bronchiolitis obliterans(BO).Method Data of UCD patients with PNP and BO from Peking Union Medical College Hospital were retrospectively analyzed,along with literatures review. Results Totally 23 cases(11 males and 12 females)were enrolled.The median age was 31 years(13-56 years).The most common pathological type was hyaline-vascular variant(91.4%),and most tumors located in abdominopelvic cavity(69.6%).Considerable cases presented bulky masses(26.3%).Most cases were first diagnosed on presentation with the symtoms of PNP(90.0%).BO was characterized by progressive dyspnea after excision of CD lesions.The average follow-up duration was 27.5 months(1-135 months).The median overall survival time was 36.0 months(95% CI=13.9-58.1).Respiratory failure was the dominant cause of death(91.7%).Conclusions PNP should be considered among those patients with specific oral or cutaneous lesions.Earlier diagnosis and treatment of latent UCD are important for reducing complications and deaths.

Background: Cytological detection of acantholytic keratinocytes (acantholytic cells [AC]) helps to identify canine pemphigus foliaceus (cPF) yet AC also occurs in superficial pyoderma (SP), the main differential diagnosis., Hypothesis/objectives: To compare selected cytomorphological features of cPF and SP and to establish cytological diagnostic criteria that could differentiate cPF from SP., Animals: 40 and 51 client-owned dogs with PF and SP, respectively., Materials and Methods: Impression smears from cPF (64), impetigo (40) and exfoliative superficial pyoderma (ESP) (17) samples were stained with Romanowsky stain, randomised, blinded and evaluated by two investigators independently. The entire sample was screened (×500 or ×1000 magnification) for round (AC1), boat (AC2) and raft AC, eosinophils and bacteria. Interobserver agreements were calculated., Results: The average number of the 10 highest ×500 fields for AC1 and AC2 was significantly higher in PF than SP (p < 0.0001; Kruskal-Wallis test). Rafts and eosinophils were more common in PF than SP (p < 0.0001; chi-square test), while bacteria were rare in PF (5%; p < 0.0001; chi-square test). Observations between the experienced and novice investigators were highly correlated. An ROC analysis identified five AC1/×500-magnification field as a suitable cut-off value for predicting PF diagnosis. This cut-off value was tested by two additional investigators, who identified sensitivity of 84%-100%, specificity of 95%-97% and accuracy of 95%-96% for the diagnosis of cPF., Conclusions and Clinical Relevance: Criterion-based impression smear cytological evaluation can provide strong evidence to support the clinical diagnosis. Acantholytic cell morphology varies in cPF and SP, and experience can improve accuracy in cytological differentiation., (© 2024 The Author(s). Veterinary Dermatology published by John Wiley & Sons Ltd on behalf of ESVD and ACVD.)

Successful treatment of pemphigus foliaceus (PF) often requires a multimodal therapeutic approach. The dog described herein underwent four therapeutic plasma exchange treatments for severe, refractory PF, resulting in a 50% reduction of lesional body surface area. This treatment option should be considered for the management of canine PF., (© 2023 The Authors. Veterinary Dermatology published by John Wiley & Sons Ltd on behalf of ESVD and ACVD.)

Background: Oclacitinib (Apoquel; Zoetis) has been reported to be beneficial for treating immune-mediated disorders., Hypothesis/objectives: This retrospective study evaluates in which group of dogs [oclacitinib (OC) or azathioprine (AZ)] remission of pemphigus foliaceus (PF) was more effectively achieved with matched induction dosing of glucocorticoids; it further evaluates which group had a higher glucocorticoid-sparing effect., Animals: Review of 30 medical records of dogs diagnosed with PF presented to a private practice dermatological service., Materials and Methods: Retrospective analysis of dogs diagnosed with PF and treated with OC or AZ in combination with glucocorticoids., Results: There was no significant difference in the ability to induce remission between AZ and OC groups. In the AZ group, 13 of 15 dogs went into some type of remission (partial or complete), compared with 11 of 15 in the OC group. There was no significant difference between the two groups for the glucocorticoid-sparing effect. The AZ group had an average reduction of 77.9% from the induction glucocorticoid dose, and OC group had an average reduction of 64.4%. One of 15 patients in the AZ group and three of 15 patients in the OC group had a 100% reduction of the glucocorticoid dose., Conclusions and Clinical Relevance: These results indicate that OC can be considered as a treatment option for canine PF., (© 2023 ESVD and ACVD.)

Background: While the clinical features were described recently, the histopathological characterisation of trunk-dominant canine pemphigus foliaceus (PF) is lacking, and whether it differs from classic facial or insecticide-triggered PF is unknown., Hypothesis/objectives: This study describes the histopathological findings of trunk-dominant PF, and compares the results to classic facial and insecticide-triggered PF., Animals: Skin biopsies from 103 dogs with clinically characterised trunk-dominant (n = 33), classic facial (n = 26) and insecticide-triggered PF (n = 44) were included., Materials and Methods: Histological sections, randomised and blinded, were scored for over 50 morphological parameters of pustules, epidermis, dermis, adnexa and crusts. Intact pustule area and width were measured by digital microscopy., Results: In trunk-dominant PF, 77 intact pustules were predominantly subcorneal (0.0019-1.940 mm 2 area, 0.0470-4.2532 mm wide), and contained from one to over 100 acantholytic keratinocytes. Pustules had boat acantholytic cells, corneocytes, perinuclear eosinophilic rings, neutrophil rosettes, acantholytic cell necrosis, rafts, cling-ons and/or eosinophils. Peripustular epidermal spongiosis, necrosis and lymphocyte exocytosis occurred, as did follicular pustules. Mixed dermal inflammation often contained eosinophils. Trunk-dominant PF did not differ from the other PF groups except for few parameters, such as having fewer rafts (p = 0.003). Additional autoimmune inflammatory patterns occurred in all PF groups., Conclusions and Clinical Relevance: Trunk-dominant PF and other canine PF variants are histologically similar, which indicates shared pathomechanisms. The identification of common boat acantholytic cells and corneocyte separation has implications for the mechanisms of acantholysis. The diversity of histopathological and polyautoimmunity features support complicated immune mechanisms. Finally, results indicate that diagnostic biopsies cannot differentiate between these PF variants in dogs., (© 2023 The Authors. Veterinary Dermatology published by John Wiley & Sons Ltd on behalf of ESVD and ACVD.)

Background: Canine trunk-dominant pemphigus foliaceus (PF) is mentioned rarely in the literature., Hypothesis/objectives: The goal of this study was to provide clinical description of trunk-dominant PF and to demonstrate the prevalence of serum antikeratinocyte, anti-desmocollin-1 (DSC1) and anti-desmoglein-1 (DSG1) antibodies, and determine their diagnostic value in this particular PF phenotype., Materials and Methods: Clinically relevant information was collected from 31, 25 and 34 dogs with trunk-dominant and facial PF and superficial pyoderma (SP), respectively. Sera from these dogs were tested for antikeratinocyte, anti-DSC1 and anti-DSG1 antibodies using indirect immunofluorescence on canine tissues and DSC1- and DSG1-transfected cells. Sera from healthy dogs and dogs with clinically irrelevant diseases served as controls., Results: Footpad involvement and grouped/polycyclic lesion organisation were identified as features of both PF phenotypes, and not of SP. Antikeratinocyte immunoglobulin (Ig)G was not specific for canine PF. By contrast, antigen-specific IgG was detected only in PF sera; anti-DSC1 IgG in 100% and 58% of dogs with facial and trunk-dominant PF, respectively, and anti-DSG1 IgG in 7% of dogs with trunk-dominant PF only., Conclusions: Trunk-dominant PF shares DSC1 as a major autoantigen with facial PF. The ability to detect anti-DSC1 IgG is lower in trunk-dominant PF, yet despite the lower sensitivity, the positive predictive value and accuracy of this particular anti-DSC1 IgG test are high. A negative test result, however, cannot exclude the diagnosis, and characteristic clinical features such as footpad involvement and/or grouped/polycyclic lesions must be considered when distinguishing trunk-dominant PF from its most relevant differential diagnosis: SP., (© 2022 ESVD and ACVD.)

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 clinical features and treatments of Chinese patients with bullous pemphigoid (BP) induced by immune checkpoint inhibitors (ICI). Methods A retrospective analysis was conducted on 18 Chinese patients with ICI-induced BP treated in the Peking Union Medical College Hospital and 14 Chinese patients with this disease reported in the literature.Furthermore,the research data of non-Chinese patients were used for comparison to outline the clinical features and treatment responses of the Chinese patients. Results A total of 32 patients (21 males and 11 females) were enrolled,and all of them presented BP induced by programmed death-1/programmed death ligand-1 inhibitors.Compared with non-Chinese patients,the Chinese patients with ICI-induced BP showed low average ages [(65.2±9.5) years vs. (69.9±10.3) years, P =0.020],increased proportion of BP induced by tislelizumab (28.1% vs. 0, P <0.001),decreased proportions of BP induced by pembrolizumab (18.8% vs. 39.4%, P =0.029) and nivolumab (3.1% vs. 52.8%, P <0.001),and decreased proportion of primary malignant melanoma (9.4% vs. 43.3%, P <0.001).The incidence of pruritus (96.9% vs. 66.1%, P <0.001) and mucosal involvement (59.4% vs. 15.7%, P <0.001) in the Chinese patients were higher than those in the non-Chinese patients.The proportions of the Chinese patients treated with tripterygium glycosides (9.4% vs. 0, P =0.008),dupilumab (18.8% vs. 0.8%, P <0.001),and topical corticosteroids (87.5% vs. 53.5%, P <0.001) were higher than those of non-Chinese patients. Conclusions The Chinese patients with ICI-induced BP tend to have a younger age and a higher probability of BP induced by tislelizumab than the non-Chinese patients.Unlike that in the non-Chinese patients,the primary tumor in the Chinese patients with ICI-induced BP is predominantly lung cancer.Pruritus is more obvious,and mucosal involvement is more common in the Chinese patients.Systemic corticosteroid therapy is the international standard treatment for ICI-induced BP,while tripterygium glycosides and dupilumab are characteristic therapies in China.

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.

Background: Goats are important worldwide as a source of milk, meat, fibre and hide, and as show animals and pets., Hypothesis/objectives: To document the type, signalment associations and prevalence of skin disease in a referral hospital population., Animals: Case population at a university veterinary teaching hospital., Methods and Materials: Retrospective study by searching computerised medical records of goats seen between 1 January 1988 and 1 January 2021. Key words employed were "alopecia, caseous lymphadenitis, Chorioptes, dermatitis, dermatophyte, dermatophytosis, goat, lice, louse, mange, mite, pemphigus foliaceus, Psoroptes, ringworm, seborrhea, skin" RESULTS: Of 1,488 records reviewed, 358 (24%) goats had skin disease recorded. Seventy-nine (22.1%) of 358 goats presented primarily for skin disease. The Nigerian Dwarf goat breed was at higher risk of developing skin disease (P < 0.0002). As goats aged, the odds for developing skin disease was higher [odds ratio (OR) = 1.07 per year, 95% confidence interval (1.04, 1.12)] as was the predilection for malignant skin tumours (P < 0.001). Sex was not associated with skin disease (P = 0.98). The most common clinical sign was exfoliative dermatitis, noted in 94 (26.4%) of 358 goats. The most common diagnoses were pediculosis, bacterial skin disease and squamous cell carcinoma. Less frequent diagnoses were Chorioptes spp. infestation, contagious ecthyma (orf), coronitis/interdigital dermatitis, demodicosis, dermatophytosis, Psoroptes spp. infestation, dermatophilosis and pemphigus foliaceus., Conclusions and Clinical Importance: Skin diseases are common in goats. Nigerian Dwarf goats and older goats are at greater risk of developing skin disease; Nigerian Dwarf goats had a predilection for malignant skin tumours. Clinicians should include a dermatological examination in goats regardless of the reason for presentation., (© 2021 ESVD and ACVD.)

Pemphigus vulgaris is a rare immune-mediated skin disorder of the dog, usually treated with immunosuppressive medications such as oral glucocorticoids, azathioprine or ciclosporin. Herein we report the successful treatment of pemphigus vulgaris in a dog, using oclacitinib and a topical product containing fucidic acid (0.5 % w/w) and betamethasone valerate., (© 2022 ESVD and ACVD.)

Background: Oral mycophenolate mofetil (MMF) currently is considered a low-risk steroid-sparing therapeutic option for the management of canine pemphigus foliaceus (PF)., Objectives: This retrospective study evaluates the therapeutic outcomes of dogs with PF treated with the combination of oral MMF and glucocorticoid (GC). Clinical outcomes and adverse side effects are reported., Animals: Eleven dogs diagnosed with PF., Materials and Methods: Retrospective review of medical records from dogs presented with PF to the dermatology service of a veterinary teaching hospital between 2015 and 2020., Results: Eleven dogs were identified which had received concurrent GCs and MMF. The MMF dose range was 19.8-45 mg/kg/day. Only two dogs (two of 11) treated with a mean MMF dosage of 39 mg/kg/day along with oral prednisone or dexamethasone achieved complete remission (CR). Partial remission (PR) was achieved in four of 11 dogs who received either prednisone, prednisolone or dexamethasone along with MMF (mean dosage 26 mg/kg/day). Four dogs (four of 11) showed poor response to MMF given at 28.5 mg/kg/day along with prednisone or dexamethasone. In one dog (one of 11) MMF was discontinued due to severe GI upset; transient vomiting and diarrhea was observed in four of 11 dogs. The median duration of MMF therapy in conjunction with GC for all groups was 70.5 days. Tapering of oral GCs while continuing MMF administration at the same dosage and frequency led to recurrence of lesions in all PF patients., Conclusion: Oral MMF combined with GC achieved CR in two of 11 PF dogs included in this study. Further research of MMF efficacy in PF may need to be performed., (© 2021 ESVD and ACVD.)

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.

Autoimmune blistering diseases (AIBDs) are a heterogenous group of skin conditions, broadly classified into two categories depending on the location of blister formation: intraepidermal blistering in the pemphigus group and subepidermal blistering in the pemphigoid group. Although AIBDs occur in both humans and animals, the arsenal of data for human AIBDs far exceeds those of their animal counterpart. Therefore, the main purpose of this review is to highlight existing knowledge, and recent advances in the diagnosis and management of AIBDs in humans - to serve as a road map for veterinary dermatologists. AREAS COVERED: Recent findings include complement-independent pathways in the pathogenesis of bullous pemphigoid, as well as the role of desmoglein and desmocollin autoantibodies in inducing acantholysis. Systemic glucocorticoids are the mainstay of treatment for AIBDs in humans, yet their long-term use is associated with severe adverse effects and complications, thereby limiting their use. Therefore, researchers have been exploring new and safer alternative therapeutic options for human AIBDs such as anti-CD20 monoclonal antibodies (Rituximab), Bruton's tyrosine kinase inhibitors (BTKi) and neonatal Fc receptor (FcRn) blockers. EXPERT OPINION: Randomised controlled trial (RCT) level evidence show that Rituximab and short-course GC regimes are more effective and safer than traditional GC treatment for human AIBDs. FcRn blockers such as SYNT001 have shown positive results in preliminary phase 2 clinical trials for treatment of human pemphigus; further trials are required. Rilzabrutinib (PRN1008), an orally administered BTKi, has recently completed phase 2 trials in pemphigus and is in a phase 3 RCT in humans., (© 2021 ESVD and ACVD.)

Background: Concurrent vasculopathic lesions in dogs with pemphigus foliaceus (PF) have been observed anecdotally yet not reported in the literature. Any association with prognosis is unclear., Hypothesis/objectives: To compare clinical features and outcome of PF in dogs with and without vasculopathic lesions., Animals: Archived, formalin-fixed, paraffin-embedded biopsy samples of 41 dogs with PF., Methods and Materials: Archived, formalin-fixed, paraffin-embedded biopsy samples with a histological diagnosis of PF were selected and re-evaluated independently. Dogs were assigned to groups following histological evaluation: Group 1 (no vasculopathic lesions) and Group 2 (vasculopathic lesions present). Group 2 was subdivided into Group 2a (vasculopathic lesions without vasculitis - i.e. vasculopathy) and Group 2b (overt vasculitis). Medical records from identified cases were reviewed retrospectively for data on clinical presentation, treatment and outcome., Results: Time to remission was longer in Group 2b (93.8 days) compared to Group 1 (41.8 days) (P = 0.047). Dogs in groups 2a and 2b were more likely to have systemic signs of illness at presentation (P = 0.028 and P = 0.032, respectively) compared to Group 1. Dogs in Group 2b were more likely to have adverse effects associated with treatment than dogs in Group 1 (P = 0.004). There were no significant differences in lesion type, distribution, rates of remission, recurrence or corticosteroid dosage between groups., Conclusions and Clinical Importance: Dogs with PF and concurrent vasculitis took longer to achieve remission and were more likely to have systemic signs of illness or adverse effects associated with treatment than dogs with PF without concurrent vasculopathic lesions., (© 2021 the European Society of Veterinary Dermatology and the American College of Veterinary Dermatology.)

Pemphigus foliaceous (PF) is a pustular, immune-mediated skin disease characterised by acantholytic cells and commonly treated with high doses of glucocorticoids. This report describes one case of feline PF successfully controlled using oral oclacitinib, suggesting a possible therapeutic alternative to glucocorticoids in some cases., (© 2021 ESVD and ACVD.)

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.

Background: Bruton's tyrosine kinase (BTK) is important in B-cell signalling. Efficacy has been reported for BTK inhibitors (BTKi) in human autoimmune diseases. Canine pemphigus foliaceus (cPF) is one of the most common canine autoimmune skin diseases., Objectives: To determine the safety and efficacy of the BTKi PRN1008 in the treatment of cPF., Animals: Four privately owned dogs., Methods and Materials: Four dogs diagnosed with PF were administered BTKi PRN1008. Initial dosages approximated to 15 mg/kg once daily, increased to twice daily if inadequate response was seen. Treatment continued for 20 weeks, attempting to decrease to every other day. Dogs were monitored with complete blood counts, serum biochemistry panels and urinalyses, and evaluated with a modified version of a validated human Pemphigus Disease Activity Index (cPDAI). Serum anti-desmocollin-1 (DSC-1) and desmoglein-1 (DSG-1) immunoglobulin (Ig)G titres were performed before and after the treatment period. Drug bound to target was measured in peripheral blood mononuclear cells (PBMC)., Results: All four dogs showed reduction in lesions and cPDAI score during the first two weeks of treatment. Three dogs continued to improve and sustained near complete remission by 20 weeks, at which point three responses were considered "good" and one "fair". Final daily dosages were in the range 17-33 mg/kg. Anti-DSC-1 IgG titre decreased dramatically in one dog, was undetectable in two and was uninterpretable in one dog. No dogs had detectable IgG to DSG1. A possible adverse event occurred in one dog., Conclusions and Clinical Importance: BTKi PRN1008 monotherapy may have some beneficial effects in some cases of cPF., (© 2020 ESVD and ACVD.)

Background: Circulating anti-keratinocyte immunoglobulin (Ig)G targeting desmosomal proteins have been identified in people and dogs with pemphigus foliaceus (PF). By contrast, detection attempts in PF-affected cats have been largely unsuccessful., Hypothesis/objectives: To detect circulating anti-keratinocyte autoantibodies in PF-affected cats and determine their titres and tissue-staining patterns., Animals: Thirty PF-affected cats were compared to 11 specific-pathogen free, 15 healthy and 31 allergic cats., Methods and Materials: Sera were tested by indirect immunofluorescence on canine footpad and buccal mucosal substrates., Results: Circulating, anti-keratinocyte IgG with a suprabasilar, web-like (intercellular) pattern were detected in the majority of PF-affected cats (23 of 30, 77%), some allergic cats (six of 31, 19%) and one healthy cat (7%). Both footpad epidermis and buccal mucosa were positive in the majority of seropositive PF-affected cats (21 of 23, 91%), and in only one of six (17%) seropositive allergic cats. Staining was limited to the footpad in the remaining seropositive PF-affected and allergic cats and one seropositive healthy cat. Reciprocal IgG titres were significantly higher in PF-affected cats compared to controls (Dunn's post-test, P < 0.0001). Anti-keratinocyte IgM, IgA or IgE were not detected in any sera., Conclusions and Clinical Importance: These results confirm the presence of circulating anti-keratinocyte IgG in a majority of PF-affected cats and in a small percentage of healthy and allergic cats. Although the molecular target and pathogenic nature of the antibodies remains unknown, the detection of positive immunostaining on buccal mucosal tissue, in addition to the footpad, suggests that the major target antigen of feline PF differs from that reported in dogs., (© 2020 ESVD and ACVD, Veterinary Dermatology.)

Background: Polyautoimmunity, the concurrent expression of two or more distinct autoimmune diseases (ADs) in a single individual, is a known phenomenon in humans and has been rarely reported in dogs. To the best of the authors' knowledge, comorbid pemphigus foliaceus (PF) and generalized discoid lupus erythematosus (GDLE) has not been reported in dogs., Hypothesis/objectives: To describe the clinical, histological and immunological features and treatment outcome of two unrelated dogs with comorbid PF and GDLE., Animals: One 10-year-old, spayed German shepherd dog and one 8-year-old, castrated American Staffordshire terrier presented for evaluation of a symmetrical, facial- and/or pedal-dominant pustular dermatitis with concurrent, truncal scaly plaques., Methods: For each dog, clinicopathological characterization included physical examination, lesion cytological evaluation, bacterial culture and sensitivity testing, skin histopathological investigation and direct and indirect immunofluorescence testing. Additional diagnostic imaging and haematological testing was performed to exclude extracutaneous disease., Results: Both dogs exhibited lesions clinically and histologically compatible with PF and GDLE. Moreover, one dog exhibited generalized leucotrichia and chronic superficial keratitis. Remission was achieved with immunosuppressive dosages of prednisolone [high-dose pulse (Case 1) or standard immunosuppressive dosage (Case 2)] and ciclosporin (5-6 mg/kg/day). Tissue-bound antikeratinocyte immunoglobulin (Ig)G and IgM were detected in both dogs. A weak basement membrane zone deposit of C3 was seen in one dog. Circulating antikeratinocyte and anti-desmocollin-1 IgG were detected in one dog., Conclusions and Clinical Importance: Cutaneous polyautoimmunity can occur in the dog. Depending on the specific disease combinations, overlapping clinical features may present diagnostic and/or therapeutic challenges. Moreover, these cases should be monitored for development of additional cutaneous or extra-cutaneous AD(s)., (© 2020 ESVD and ACVD.)

Background: Bruton's tyrosine kinase (BTK) is important in B-cell signalling. Efficacy has been reported for BTK inhibitors (BTKi) in human autoimmune diseases. Canine pemphigus foliaceus (cPF) is the most common canine autoimmune skin disease., Objectives: To determine the safety and efficacy of a BTKi in cPF treatment., Animals: Nine privately owned dogs., Methods and Materials: Nine dogs diagnosed with PF were administered BTKi PRN473. Initial dosages were ≈15 mg/kg once daily, increased to twice daily if inadequate response was seen. Treatment continued for a maximum of 20 weeks, attempting decrease to every other day. Dogs were monitored with complete blood counts, serum biochemistry panels, urinalyses and evaluated with a modified version of a validated human Pemphigus Disease Activity Index (cPDAI). Anti-desmocollin-1 (DSC-1) and desmoglein-1 (DSG-1) immunoglobulin G (IgG) titres were performed before and after the treatment period. Drug bound to target was measured in peripheral blood mononuclear cells., Results: All nine dogs showed reduction in lesions and cPDAI score during the first two weeks of treatment. At the end of the study, four responses were considered "good", two "fair", two "poor" and one dog withdrawn due to recurrence of a previously excised mast cell tumour. Four dogs continued to improve by Week 4; three sustained near complete remission by study's end. The anti-DSC-1 IgG titre decreased in three dogs, increased in two, was undetected in three and was not performed in the withdrawn dog. No dogs had detectable IgG to DSG1. Possible adverse effects occurred in three dogs., Conclusions and Clinical Importance: Bruton's tyrosine kinase inhibitor monotherapy may have beneficial effects in some cases of cPF., (© 2020 ESVD and ACVD.)

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.

Background: Few studies have described the pathophysiology, clinical course, treatment outcomes and quality of life (QoL) of cats with pemphigus foliaceus (PF)., Objective: Describe clinicopathological features, treatment outcomes and impacts on QoL in feline PF., Animals: Forty-nine client-owned cats with PF that presented to a veterinary teaching hospital between 1987 and 2017., Methods and Materials: Medical records and histopathological reports were reviewed to obtain clinicopathological data and treatment outcomes. Owners were contacted and requested to complete a questionnaire to obtain long-term follow-up and evaluate the impacts of PF on QoL of cats and owners., Results: Domestic short/medium/long hair breeds were most commonly affected, with pinnae, head, haired face, nasal planum and ungual folds most frequently involved. Associated pruritus and systemic signs of illness were common. Vasculopathological changes were noted in a small proportion of cats. Corticosteroid monotherapy was sufficient to induce complete remission in the majority of cats. Pemphigus foliaceus and its management had a negative impact on QoL of both cats and owners. Receiving/administering medications, attending veterinary appointments, and financial and time commitments were cited sources of stress for affected cats and/or owners., Conclusions and Clinical Importance: Results illustrate that affected cats generally respond favourably to treatment but do require long-term therapy. The exact aetiology of the vasculopathological changes was unclear; it may reflect the stage or severity of disease or suggest the presence of a cutaneous adverse drug reaction. Clinicians managing cats with PF should be aware of the potential negative impact on QoL of owners and cats and adjust management accordingly., (© 2019 ESVD and ACVD.)

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.