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Background: Pemphigus is a life-threatening autoimmune disease characterised by blistering skin and/or mucous membranes. The present study aimed to determine the prevalence of fungal infections in the pemphigus population., Methods: Different databases were searched to gain access to all studies on the prevalence of fungal infections published up to the 31st of May 2024. The pooled rate prevalence of fungal infections with a 95% confidence interval (CI) was calculated using the random effects model., Results: A total of 1012 studies were identified, out of which 18 were included in meta-analyses. The random-effects estimates of the prevalence of fungal infection in the pemphigus population were 0.18% (95% CI: 9%-31%). Based on the findings, it was determined that four genera of fungal infections were related to mucocutaneous lesions of individuals diagnosed with pemphigus. Among these fungi, the most prevalent were those belonging to the Candida species, with a particular emphasis on Candida albicans (87.61%). Additional isolated fungal species include Trichophyton rubrum (4.5%), Aspergillus species (1.8%), Pneumocystis jirovecii (1.2%), Malassezia furfur (0.78%), Microsporum canis (0.75%), Trichophyton mentagrohytes (0.65%), Microsporum audouinii (0.60%), and Trichophyton concentricum (0.45%)., Conclusions: The current study highlights the significance of fungal infection in individuals with pemphigus, suggesting that pemphigus and administration of immunosuppressive medicines such as corticosteroids may trigger an increased risk of fungal infections. Additionally, prompt diagnosis of fungal infections in individuals with pemphigus could help healthcare professionals prevent and treat serious infections, such as Pneumocystis jirovecii pneumonia (PJP), and potentially alleviate the economic impact of this condition., (© 2024 Wiley‐VCH GmbH. Published by John Wiley & Sons Ltd.)

Background: Pemphigus is a group of autoimmune blistering disorders that have been associated with dementia in previous studies. Mild cognitive impairment (MCI) can be the first stage of progression into dementia. The objective of the present study was to evaluate the frequency of MCI in pemphigus patients compared to a control group., Methods: This case-control study included 80 patients with pemphigus referred to the dermatology clinics of Shohadaye Tajrish and Loghman Hakim hospitals, Tehran, Iran, in 2021. A group of 80 individuals without pemphigus who visited the same clinics for cosmetic consultation or interventions were regarded as controls. Age, sex, marital status, and education were recorded for all participants. Disease duration, medications, and severity were noted for pemphigus patients. The Persian version of the Montreal Cognitive Assessment (MoCA) test was used to assess cognitive function., Results: MCI was significantly more frequent in pemphigus patients than in controls (55% vs. 37.5%, P = 0.026). Furthermore, the total MoCA score was significantly lower in pemphigus patients compared to controls (23.98 ± 3.77 vs. 25.21 ± 3.45, P = 0.032); however, among MoCA's different domains, only the executive functions score was significantly lower in pemphigus patients (P = 0.010). After adjustment, multivariable logistic regression analysis revealed that every 1-year higher education in patients decreased the odds of MCI by 52% (adjusted odds ratio = 0.483, 95% confidence interval 0.326; 0.715, P < 0.001)., Conclusions: The frequency of MCI was found to be significantly higher, and overall scores of the MoCA test, as well as its executive function domain, were significantly lower among pemphigus patients in this study compared to the control group. Additionally, a higher level of education was associated with decreased odds of MCI in pemphigus patients. Identifying pemphigus patients with MCI through the use of the MoCA test can facilitate early intervention, enabling them to seek help and support., (© 2024 the International Society of Dermatology.)

Pemphigus is a group of autoimmune mucocutaneous bullous disorders characterized by acantholysis resulting from autoantibodies targeting epithelial cell surface antigens. Studies reflect the presence of nail manifestations in some patients and suggest a potential correlation with clinical severity. This study examines the overall prevalence and characterizes the diverse manifestations of nail changes in pemphigus. We searched Cochrane, MEDLINE, EMBASE, and LILACS from 1990 to June 26, 2023 for studies reporting different nail changes in pemphigus patients. Data were collected and pooled to obtain proportions of the prevalence of nail changes in patients with pemphigus and subgroup analysis for pemphigus foliaceous and pemphigus vulgaris. The risk of bias was assessed with the Joanna Briggs Institute Checklist. Of 321 studies screened, 14 studies with 1,208 patients were included. Paronychia (n = 185) and Beau's lines (n = 104) were the most common nail changes identified. The pooled prevalence of nail disease in pemphigus patients was 0.389 (number of studies; [95% CI]: n = 9; [0.160-0.680], with high heterogeneity between studies (I 2  = 95.0%, P < 0.001). Subgroup analysis revealed the highest prevalence in pemphigus foliaceous at 0.342 (n = 3; [0.109-0.688]) and pemphigus vulgaris at 0.396 (n = 5; [0.114-0.769]). Nail changes exhibited varied temporal relationships with disease onset and flares, preceding, concurrent, or following these events. Correlation with disease severity was noted, although discrepancies between studies were reported. Nail changes in pemphigus, particularly pemphigus vulgaris and pemphigus foliaceous, may be underrecognized. Observations regarding temporal associations and potential correlations with disease severity highlight the diagnostic and prognostic implications of nail changes in pemphigus. The limitations of this study include study heterogeneity and possible bias. Further research to establish the correlation of the presence and severity of nail changes on the overall disease course would be helpful., (© 2024 the International Society of Dermatology.)

Background: Pemphigus vulgaris (PV) is a potentially life-threatening mucocutaneous autoimmune disease that affects desmoglein-1 and desmoglein-3, leading to intraepithelial vesiculobullous lesions. In the oral mucosa, PV lesions can mimic other diseases such as mucous membrane pemphigoid, other forms of pemphigus, recurrent aphthous stomatitis, erythema multiforme, Stevens-Johnson syndrome, and virus-induced ulcers like herpes simplex virus (HSV), making diagnosis challenging. The co-occurrence of PV with Crohn's disease is rare and predominantly seen in younger patients. The therapeutic mainstay for both PV and Crohn's disease usually involves systemic corticosteroids combined with immunosuppressants and immunobiological drugs. Literature indicates that the use of these drugs, particularly TNF-alpha inhibitors, for managing autoimmune diseases like Crohn's can potentially induce other autoimmune diseases known as autoimmune-like syndromes, which include episodes of lupus-like syndrome and inflammatory neuropathies. There are few cases in the literature reporting the development of PV in individuals with CD undergoing infliximab therapy., Case Report: A young female with severe Crohn's disease, treated with the TNF-alpha inhibitor infliximab, developed friable pseudomembranous oral ulcerations. Histopathological and immunofluorescence analyses confirmed these as PV. The treatment included clobetasol propionate and low-level photobiomodulation, which resulted in partial improvement. The patient later experienced severe intestinal bleeding, requiring intravenous hydrocortisone therapy, which improved both her systemic condition and oral lesions. Weeks later, new ulcerations caused by herpes virus and candidiasis were identified, leading to treatment with oral acyclovir, a 21-day regimen of oral nystatin rinse, and photodynamic therapy, ultimately healing the oral infections. To manage her condition, the gastroenterologists included methotrexate (25 mg) in her regimen to reduce the immunogenicity of infliximab and minimize corticosteroid use, as the patient was in remission for Crohn's disease, and the oral PV lesions were under control., Conclusion: Young patients with Crohn's disease should be referred to an oral medicine specialist for comorbidity investigation, as oral PV and opportunistic infections can arise during immunosuppressive therapy. The use of TNF-alpha inhibitors in patients treated for inflammatory bowel disease, such as Crohn's, should be carefully evaluated for potential side effects, including oral PV., (© 2024. The Author(s).)

Paraneoplastic pemphigus is a rare, life-threatening autoimmune disease that is clinically characterized by mostly extensive and refractory mucosal erosions and polymorphous skin lesions. We report here on a 16-year-old girl with isolated oral erosions, in whom mucosal pemphigoid was initially suspected and after treatment with prednisolone and dapsone marked improvement was achieved. However, a few months later the patient developed massive respiratory insufficiency as a result of bronchiolitis obliterans, so that a lung transplant was planned. As part of the preparatory diagnostic workup, unicentric, abdominally localized Castleman's disease was diagnosed, which ultimately led to the diagnosis of paraneoplastic pemphigus as evidenced by envoplakin autoantibodies. Tumor resection and subsequent lung transplantation achieved good results with sustained mucocutaneous remission., (© 2024. The Author(s).)

Background: Autoimmune blistering disorders (ABDs) might elevate cardiovascular risk, but studies are lacking., Objective: The objective of this study was to examine if ABDs elevate the risk of atherosclerotic cardiovascular disease, heart failure, arrhythmia, venous thromboembolism, and cardiovascular death., Methods: A population-based cohort of Danish patients with ABD (≥18 years of age) diagnosed during 1996-2021 (n = 3322) was compared with an age- and sex-matched comparison cohort from the general population (n = 33,195)., Results: Compared with the general population, patients with ABDs had higher 1-year risks of atherosclerotic cardiovascular disease (3.4% vs 1.6%), heart failure (1.9% vs 0.7%), arrhythmia (3.8% vs 1.3%), venous thromboembolism (1.9% vs 0.3%), and cardiovascular death (3.3% vs 0.9%). The elevated risk persisted after 10 years for all outcomes but arrhythmia. The hazard ratios associating ABDs with the outcomes during the entire follow-up were 1.24 (1.09-1.40) for atherosclerotic cardiovascular disease, 1.48 (1.24-1.77) for heart failure, 1.16 (1.02-1.32) for arrhythmia, 1.87 (1.50-2.34) for venous thromboembolism, and 2.01 (1.76-2.29) for cardiovascular death. The elevated cardiovascular risk was observed for both pemphigus and pemphigoid., Limitations: Our findings might only generalize to patients with ABDs without prevalent cardiovascular diseases., Conclusion: Patients with ABDs had an elevated cardiovascular risk compared with age- and sex-matched controls., Competing Interests: Conflicts of interest None disclosed., (Copyright © 2024 American Academy of Dermatology, Inc. Published by Elsevier Inc. All rights reserved.)

Bullous pemphigoid (BP) is a rare blistering disease often considered a primary sign of a paraneoplastic syndrome. Retrospective studies have established its link with hematological malignancies, particularly lymphoproliferative disorders. Here, we present what we believe to be the inaugural case of successful simultaneous management of BP and de novo acute myeloid leukemia (AML) in a 28-year-old male patient. Given the rarity and severity of both conditions, our treatment strategy aimed to maximize efficacy by combining immunosuppressive therapy (initially plasmapheresis with high-dose corticosteroids, followed by anti-CD20 monoclonal antibody and intravenous immunoglobulins 2 g/m 2 ) with lymphodepleting antileukemic chemotherapy utilizing Fludarabine (FLAG-IDA induction regimen). Following diagnosis, considering the patient's youth and the concurrent presence of two rare and potentially life-threatening diseases, we opted for an aggressive treatment. Upon achieving complete morphological remission of AML with measurable residual disease (MRD) negativity, despite incomplete resolution of BP, we proceeded with high-dose cytarabine consolidation followed by peripheral stem cell harvest and autologous stem cell transplantation (ASCT). Our conditioning regimen for ASCT involved Bu-Cy with the addition of anti-thymocyte globulins. At day + 100 post-ASCT, bone marrow evaluation confirmed morphological remission and MRD negativity. Meanwhile, BP had completely resolved with normalization of BP180 antibody levels., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Purpose: To evaluate ocular surface involvement, tear cytokine levels, and histopathological changes in pemphigus and pemphigoid patients., Methods: A total of 22 patients (15 pemphigus and 7 pemphigoids) and 21 non-diseased controls were enrolled in our study. All participants underwent ocular surface evaluation, which included ocular surface disease index test, slit lamp observation, dry eye-related examination, tear multicytokine analysis, and conjunctival impression cytology., Results: Pemphigus and pemphigoid patients presented much more severe conjunctivochalasis, corneal epithelial defects, corneal opacity, symblepharon   and dry eye. Severe ocular surface squamous metaplasia and a significant increase of tear macrophage inflammatory protein-1beta, tumor necrosis factor-alpha, interleukin (IL)-1β, IL -6, and IL-8 occurred in pemphigus and pemphigoid patients., Conclusions: Our results revealed that ocular surface inflammation and dry eye persist in most pemphigus and pemphigoid patients, and do not occur in parallel with the systemic course. Regular ophthalmological examinations and local anti-inflammatory should be provided for pemphigus and pemphigoid patients.

Myiasis is the infestation of live vertebrates by dipterous larvae. Cutaneous myiasis is the most common form, although many organs can be infected by these larvae. Cutaneous myiasis is divided into 3 forms: localized furuncular, migratory, and wound myiasis, which have a worldwide distribution, but tropical and subtropical countries have a heavier burden of the disease. Herein, we report a case of scalp wound myiasis in a patient with pemphigus vulgaris caused by Muscidae domestica (M. domestica) in Riyadh, Saudi Arabia. Cases of M. domestica myiasis are limited in the literature. We would like to raise awareness regarding the possibility of cutaneous myiasis in M. domestica in Riyadh, Saudi Arabia., (Copyright: © Saudi Medical Journal.)

We report a case of refractory paediatric pemphigus vulgaris with sepsis, treated successfully with intravenous immunoglobulin (IVIG) and amniotic membrane dressing. The patient was initially started on oral prednisolone (1 mg/kg/day) and dapsone 50 mg once daily. Azathioprine 50 mg orally was then used in place of dapsone due to rapid disease progression with extensive skin involvement. However, the patient developed sepsis and azathioprine had to be discontinued. Because of rapidly progressive disease and sepsis, the patient was put on IVIG at a dose of 2 g/kg in divided doses over 3 days along with amniotic membrane dressing. There was marked improvement after 2 weeks of follow-up., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2023. No commercial re-use. See rights and permissions. Published by BMJ.)

Nocardia brain abscess is an uncommon but potentially life threatening opportunistic infection that generally occurs in immunocompromised patients. Nocardia cyriacigeorgica is a recently described species rarely reported as a cause of human disease. Pemphigus vulgaris is managed with immunosuppression. There have been four prior reports of brain abscess caused by Nocardia cyriacigeorgica .

Background: Diagnosis of a severe condition may have a strong emotional impact on patients. Specific emotions experienced when receiving the diagnosis of a bullous disease have not been investigated., Methods: Adult patients diagnosed with a bullous condition were recruited through the Italian Association of patients with pemphigus and pemphigoid (ANPPI). Information was collected online on sociodemographic and clinical data. We asked which emotions the patient experienced at the time of the diagnosis, i.e., isolation, anger, confusion, sadness, despair, disregard, fear, avoidance, and challenge. Also, the patients reported to whom they talked as soon as they had the diagnosis., Results: Data were collected on 105 patients, most of whom were affected by pemphigus vulgaris. The emotion most frequently experienced at diagnosis was confusion (47.6% of patients). More than 30% of patients reported sadness and fear, 12.5% anger, and 10.5% despair. A significantly higher percentage of women than men experienced isolation and despair. Despair was more frequent in patients who were older at diagnosis. Patients with children experienced more sadness and despair, and less avoidance and challenge, while those who had a relative with a bullous disease reported less fear, and more challenge., Conclusions: Clinicians should be aware of the emotions of the patient when communicating the diagnosis of severe conditions, such as bullous diseases. Active listening and empathy are necessary to provide patients with correct information on the disease, so that they are not overwhelmed with negative emotions.

Competing Interests: The authors declare no conflicts of interest.

Pemphigus is a class of rare autoimmune diseases that causes incredibly painful blistering of the skin and significantly impacts patients' day-to-day lives and well-being. Many strides have been made in treating pemphigus; however, no comprehensive literature exists on how to treat the pain that accompanies the disease. It is important to remember that treating pemphigus involves a two-fold treatment plan assessing both the underlying autoimmune disease and the pain involved with the lesions. This literature review explores novel therapies that have been shown to be effective in treating pain in pemphigus., (© 2022 the International Society of Dermatology.)

Autoimmune bullous skin diseases (AIBDs), such as bullous pemphigoid (BP) and pemphigus, are characterized and caused by autoantibodies targeting structural proteins. In BP, clinical experience and recent systematic evaluation identified pruritus to be common and an important cause of impaired quality of life. Furthermore, chronic pruritus may be the sole clinical symptom of BP. In pemphigus, a retrospective study recently documented a high prevalence of pruritus. The temporal relation between pruritus and BP/pemphigus are, however, unknown. Likewise, the presence of pruritus in AIBDs other than BP and pemphigus is unknown. To address this, we performed propensity-matched retrospective cohort studies using TriNetX, providing real-world patient data to (i) assess the risk to develop AIBDs following the diagnosis of pruritus and (ii) vice versa. We assessed this in eight AIBDs: BP, mucous membrane pemphigoid (MMP), epidermolysis bullosa acquisita, dermatitis herpetiformis, lichen planus pemphigoides (LPP), pemphigus vulgaris, pemphigus foliaceous, and paraneoplastic pemphigus (PNP). For all AIBDs, pruritus was associated with an increased risk for the subsequent diagnosis of each of the eight investigated AIBDs in 1,717,744 cases (pruritus) compared with 1,717,744 controls. The observed hazard ratios ranged from 4.2 (CI 3.2-5.5; p < 0.0001) in MMP to 28.7 (CI 3.9-211.3; p < 0.0001) in LPP. Results were confirmed in two subgroup analyses. When restricting the observation time to 6 months after pruritus onset, most HRs noticeably increased, e.g., from 6.9 (CI 6.2-7.9; p < 0.0001) to 23.3 (CI 17.0-31.8; p < 0.0001) in BP. Moreover, pruritus frequently developed following the diagnosis of any of the eight AIBDs, except for PNP. Thus, all AIBDs should be considered as differential diagnosis in patients with chronic pruritus.

In dermatology, biologics that block signaling pathways of TNF-α, IL-4/IL13, IL-17s, and IL-23 are widely used for the treatment of several inflammatory skin diseases, such as atopic dermatitis and psoriasis. They have shown excellent efficacy with an acceptable safety profile. However, these biologics targeting pathogenic cytokines and their receptors could modulate immunological balance, leading to the development of other inflammatory or autoimmune skin diseases in some cases. In this study, we present a patient who suffered pemphigus vegetans and showed an exacerbation of pemphigus foliaceus after secukinumab loading for the treatment of complicated generalized pustular psoriasis and pyoderma gangrenosum., (© 2022 Japanese Dermatological Association.)

Immunoglobulin A (IgA) pemphigus, also known as intercellular IgA dermatosis, is a rare autoimmune bullous disease presenting with IgA anti-keratinocyte cell surface autoantibodies. Concomitant lymphoproliferative disorders have been reported in IgA pemphigus, including IgA monoclonal gammopathy of undetermined significance and IgA type multiple myeloma (MM). A 35-year-old Japanese woman with a 3-year history of pruritic papulovesicles on her lower legs and trunk was referred to our department. Histopathological examination revealed acantholytic blisters, and results of both direct and indirect immunofluorescence were negative. Direct and indirect immunofluorescence were still negative 3 years and 7 months later. Approximately 7 years after her first visit, the patient was re-referred to us because of disease exacerbation. Histopathological findings revealed subcorneal blistering with acantholysis, in which neutrophil-dominant inflammatory cells were present. Indirect immunofluorescence was positive for IgA on the epidermal cell surface and both desmoglein (Dsg) 1/3 and (Dsc) desmocollin 1-3 enzyme-linked immunosorbent assays (ELISAs) for IgA were positive. The histological findings and positive Dsc1 IgA ELISA led to the diagnosis of subcorneal pustular dermatosis (SPD)-type IgA pemphigus. Further examination revealed hyper-IgA globulinemia, increased serum IgA-κ protein, and increased plasma cells in the bone marrow, enabling the diagnosis of IgA type MM. Daratumumab, lenalidomide, and dexamethasone (DLd) therapy was effective for both the MM and the skin lesions, resulting in negative results on Dsg1/3 and Dsc1-3 IgA ELISAs. The association between IgA pemphigus and IgA type multiple myeloma remains unclear, and only seven cases including the present case have been reported. Literature review revealed associations between SPD-type and IgA κ chain in IgA pemphigus and MM, and that in most cases the onset or diagnosis of MM was simultaneous or occurred after the diagnosis of IgA pemphigus. Therefore, clinicians should be aware of the development of multiple myeloma during the clinical course of patients with SPD-type IgA pemphigus., (© 2022 Japanese Dermatological Association.)

Pemphigus vulgaris is a potentially fatal disease within the epidermis with rare noncutaneous manifestations. Heterotopic ossification has not been previously described as one of the inherent complications of this skin pathology. A 44-year-old man presented with severe pemphigus vulgaris involving 80 to 90% of his body surface area. He had an extended time to wound closure of 5 months, as well as two additional months bed bound due to related infectious and respiratory complications. He progressively developed a worsening range of motion at his bilateral elbows. X-ray demonstrated bilateral anterior elbow heterotopic ossification. Passive and active range of motion exercises were initiated early in the course of his treatment and only yielded a small positive effect. Thus, screening for heterotopic ossification may be warranted when a significant joint range of motion is lost in cases of autoimmune cutaneous disease, and even more in the presence of severe open wounds with delayed wound closure., (© The Author(s) 2022. Published by Oxford University Press on behalf of the American Burn Association. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.)

Introduction: Paraneoplastic pemphigus (PNP) is a rare, often fatal, autoimmune blistering disease of the skin and mucous membranes. In children, PNP is frequently associated with Castleman disease (CD). This series describes five cases of PNP associated with CD., Methods: Data were collected retrospectively from the medical records of patients with a diagnosis of PNP and CD from January 2013 to June 2022. Patients ≤22 years old with clinical and immunopathologic evidence of PNP were included; CD was diagnosed histopathologically., Results: Two children, two adolescents, and one young adult (two males, three females) were included. The average age at disease presentation was 11.8 years (range: 7-22 years). Oral (n = 5) and anogenital (n = 3) mucositis were common. Four patients had "unicentric" CD (UCD); one patient had "multicentric" CD (MCD). Castleman tumors were in the retroperitoneum (n = 4) or axilla (n = 1). One patient had myasthenia gravis without thymoma. Three patients had bronchiolitis obliterans (BO). Three patients had complete resection of their CD; two had partial resection. Three patients remain alive with a median follow-up of 13 months (range: 12 months to 13 years); two are clinically stable with resolution of mucocutaneous lesions; one has persistent BO requiring ongoing ventilatory support. Patients who remain alive had UCD with complete resection; all deceased patients had partial resection and BO., Conclusion: Most patients had UCD, and the retroperitoneum was the most common location. Patients with MCD, incomplete resection, and BO died; patients with UCD and complete resection remain alive, even in the setting of BO. Consideration of PNP is critical when pediatric patients present with mucositis as PNP may be clinically indistinguishable from more common causes of mucositis., (© 2022 Wiley Periodicals LLC.)

Abstract: The combination of paraneoplastic pemphigus and prostate cancer is extremely unusual and has not been reported yet. Paraneoplastic pemphigus is caused by tumor-induced autoantibodies, which cause damage to the skin and mucosa. The essential treatment is active tumor control. Our patient received a robot-assisted radical prostatectomy and glucocorticoid therapy to improve his condition and relieve his skin lesions., Competing Interests: The authors declare no conflicts of interest. The patient gave his written informed consent for reporting and publishing his case. Disclosure statement: No potential conflict of interest was reported by the author(s)., (Copyright © 2022 Wolters Kluwer Health, Inc. All rights reserved.)

Introduction: The ocular presentation of paraneoplastic pemphigus (PNP) has rarely been reported in the literature. In this report, we describe a 61-year-old male presenting with eruptive skin lesions associated with underlying non-Hodgkin's lymphoma who had rapid progressive corneal perforation with secondary endophthalmitis in the setting of PNP., Case Description: A 61-year-old male presented to the emergency department complaining of skin eruption mimicking Stevens-Johnson syndrome, which was later found to be related to PNP. Initially, the patient complained of progressive ocular surface dryness in both eyes. Meanwhile, he developed mild pain in the right eye associated with blurry vision in both eyes and was managed with lubricants and topical antibiotics. A few days later, he was found to have corneal perforation with features suggestive of left endophthalmitis with possible early panophthalmitis. Intravenous antibiotic was administered, and primary evisceration of the left globe was performed. Histopathology revealed acute necrotizing keratitis and endophthalmitis. Vitreous analysis showed numerous gram-negative bacilli and a positive culture of Morganella morganii . The patient continued to be managed with frequent lubrications and punctual plugs in the fellow eye during the follow-up period., Conclusion: We describe the first case of endophthalmitis developing secondary to PNP-induced corneal melting and perforation. Anticipating unusual infectious sequelae in the setting of PNP might be warranted to actively detect and successfully manage dry eye disease before devastating complications develop.

Background: There is currently no information on the anatomical risk factors for splenic artery aneurysm rupture, specifically the location or size of the lesion; therefore, reporting this entity to obtain data and ultimately reduce morbidity and mortality is essential. Here we report a case of a male patient with spontaneous rupture of a large splenic artery aneurysm presenting with abdominal pain., Case Presentation: A 59-year middle-eastern male, with known pemphigus vulgaris presented with a chief complaint of headache and syncope, followed by abdominal pain along with severe metabolic acidosis. A contrast-enhanced computed tomography scan of the abdomen and pelvic showed a splenic artery aneurysm of 33 × 30 mm with a 150 × 90 mm hematoma formation around the aneurysm site. The patient underwent an operation and splenectomy, with confirmation of the diagnosis of ruptured splenic artery aneurysm., Conclusion: It is essential to consider splenic aneurysm rupture as a second-line differential diagnosis, especially among patients with comorbid diseases, as this can lead to timely and appropriate lifesaving intervention., (© 2022. The Author(s).)

Objective: Critically ill patients with solid tumors complicated with paraneoplastic pemphigus are usually treated in intensive care units (ICU) for perioperative management after surgical treatment. In this study, the clinical characteristics and predictors of long-term prognosis of these critically ill patients were analyzed., Methods: the clinical and laboratory data of 63 patients with solid tumors complicated with paraneoplastic pemphigus admitted to ICU from 2005 to 2020 were retrospectively analyzed, and the survival status of the patients were followed up., Results: Among the 63 patients, 79.4% had Castleman disease as the primary tumor, and 20.6% with other pathological types; 69.8% had severe-extensive skin lesions, and 30.2% had other skin lesions; the patients with bronchiolitis obliterans accounted for 44.4%, and 55.6% were not merged. Postoperative fungal infection occurred in 23.8% of the patients, and 76.2% without fungal infection. The median follow-up time was 95 months, and 25 patients died during the study period. The 1-year, 3-year and 5-year survival rates were 74.6% (95% CI 63.8%-85.4%), 67.4% (95% CI 55.6%-79.2%) and 55.1% (95% CI 47.9%-62.3%), respectively. The log-rank univariate analysis showed that the patients had age>40 years ( P =0.042), preoperative weight loss>5 kg ( P =0.002), preoperative albumin < 30 g/L ( P < 0.001), paraneoplastic pemphigus complicated with bronchiolitis obliterans ( P =0.002), and perioperative fungal infection ( P < 0.001) had increased mortality. Cox univariate analysis showed that preoperative weight loss >5 kg ( P =0.005), preoperative albumin < 30 g/L ( P < 0.001), paraneoplastic pemphigus complicated with bronchiolitis obliterans ( P =0.009), preoperative bacterial pulmonary infection ( P =0.007), prolonged surgical time ( P =0.048), postoperative oxygenation index ( P =0.012) and low albumin ( P =0.010) and hemoglobin concentration ( P =0.035) in ICU, acute physiology and chronic health evaluation (APACHE Ⅱ) score ( P =0.001); sequential organ failure assessment (SOFA) score ( P =0.010), and postoperative fungal infection ( P < 0.001) were risk factors for long-term survival. Cox regression model for multivariate analysis showed that preoperative weight loss > 5 kg ( HR 4.44; 95% CI 1.47-13.38; P =0.008), and preoperative albumin < 30 g/L ( HR 4.38; 95% CI 1.72-11.12; P =0.002), bronchiolitis obliterans ( HR 2.69; 95% CI 1.12-6.50; P =0.027), and postoperative fungal infection ( HR 4.85; 95% CI 2.01-11.72; P < 0.001) were independent risk factors for postoperative mortality., Conclusion: The 5-year survival rate of critically ill patients undergoing surgery for paraneoplastic pemphigus combined with solid tumors is approximately 55.1%, with preoperative weight loss > 5 kg, albumin < 30 g/L, bronchiolitis obliterans and postoperative fungal infection were associated with an increased risk of near- and long-term postoperative mortality.

Pemphigus is a rare autoimmune bullous disease which affects the mucous membranes and skin. Pemphigus vulgaris (PV) is the most prevalent type of pemphigus. PV presents with extremely painful, non-healing oral erosions. However, there are only a handful of reports addressing the pain management of PV. We would like to present a case of a painful PV, which was successfully managed., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2022. No commercial re-use. See rights and permissions. Published by BMJ.)

Autoimmune skin diseases are a group of disorders that arise due to the dysregulated immune system attacking self-antigens, causing multiple tissue and organ lesions. With disease progression, the physical and psychological health of patients may be seriously damaged. High-frequency ultrasound is non-invasive, reproducible, and suitable for visualizing the fine structure of external organs. The usage of high-frequency ultrasound has increased in recent years in the auxiliary diagnosis and monitoring of various skin diseases; it serves as a promising tool for dermatological disease assessment. This review summarizes the characteristics of high-frequency ultrasound imaging in common autoimmune skin diseases, including systemic lupus erythematosus, scleroderma, psoriasis, dermatomyositis, and pemphigus/pemphigoid. The objective of this review is to provide new ideas and strategies for dermatologists to diagnose and track the prognosis of autoimmune skin diseases.

Competing Interests: None

Background: Pemphigus is associated with several autoimmune, dermatological, and psychiatric diseases. Previous studies have reported an increasing incidence of pemphigus in Finland, particularly pemphigus foliaceus and erythematosus., Objectives: The aim of this study was to determine the clinical presentation and associated comorbidities in pemphigus patients., Materials & Methods: We retrospectively assessed 66 pemphigus patients in Helsinki University Hospital and, with an age-standardised control group, performed a comparison of the studied comorbidities., Results: The patients displayed a 0.8 female:male distribution and a mean age of 57.4 years. Pemphigus vulgaris (41%), foliaceus (30%), and erythematosus (15%) were the most common subtypes. Hypertension (30%) and dyslipidaemia (21%) were the most prevalent comorbidities. We found a statistically significant association between pemphigus and a past history of, or concurrent malignancies and atopic dermatitis (p = 0.002 and p = 0.028, respectively). No significant difference was observed in the prevalence of cardiovascular disease, asthma, chronic obstructive lung disease, type I or II diabetes mellitus, inflammatory bowel disease, depression, or anxiety. Erosions (65%), bullae (59%), and crusted lesions (55%) were observed in most patients. Half of the patients experienced pruritus before or at diagnosis. Pruritus was associated with pemphigus without mucosal involvement (p = 0.01)., Conclusion: We found a significant association between pemphigus and atopic dermatitis and a history of malignancy. The clinical picture frequently included pruritus. These results support the findings of some recent studies of pruritus occurring more frequently in patients with pemphigus foliaceus and cutaneous pemphigus vulgaris.

Purpose: To describe a case of paraneoplastic pemphigus (PNP) presenting as spontaneous bilateral corneal perforations in a patient with follicular dendritic cell sarcoma., Methods: Retrospective chart reviewResults: A 73-year-old Greek woman with a history of follicular dendritic cell sarcoma (FDCS) presented with bilateral corneal perforations and a cicatrizing conjunctivitis. Her diagnosis was consistent with PNP with corneal and conjunctival involvement after a change in her chemotherapy regimen from intravenous cyclophosphamide to gemcitabine. She was treated with a multilayered amniotic membrane in the right eye and cyanoacrylate glue in the left eye. Systemic intravenous cyclophosphamide and oral prednisone were re-started. Both perforations healed but the patient passed away soon after precluding further follow-up., Conclusions: Ocular manifestations of PNP can rarely present with spontaneous corneal perforations. This is the first case of FDCS-associated PNP with corneal involvement. Such cases should be diagnosed expediently and managed with aggressive systemic immunosuppressive therapy.