Airway management of a patient with linear immunoglobulin A bullous dermatosis:A case report

BACKGROUND There is limited literature on managing the airway of patients with linear immunoglobulin A(IgA)bullous dermatosis,a rare mucocutaneous disorder that leads to the development of friable bullae.Careful clinical decision making is necessary when there is a risk of bleeding into the airway,and a multidisciplinary team approach may lead to decreased patient morbidity during these high-risk scenarios,especially when confronted with an unusual cause for bleeding.CASE SUMMARY A 45-year-old African American female presented to our ambulatory surgical center for right corneal transplantation due to corneal perforation after blunt trauma in the setting of cicatricial conjunctivitis and diffuse corneal neovascularization from linear IgA bullous dermatosis.The diagnosis of IgA dermatosis was recent,and the patient had been lost to follow-up.The severity of the disease and extent of airway involvement was unknown at the time of the surgery.Significant airway bleeding was noticed upon intubation and the otorhinolaryngology team had to be called to the operating room.The patient required transfer to the intensive care unit where a multidisciplinary team was involved in her case.The patient was extubated on postoperative day 4.CONCLUSION A multidisciplinary approach to treating this disease is the best course of action before a surgical procedure.In our case,key communication between the surgery,anesthesia,and dermatology teams led to the quick and safe treatment of our patient’s disease.Ambulatory surgery should not be considered for these cases unless they are in full remission and there is no mucous membrane involvement.

Bilateral malignant glaucoma with bullous keratopathy:A case report

BACKGROUND Malignant glaucoma,caused by aqueous misdirection,is a challenging postsurgical complication presented with normal/high intraocular pressure and shallowing of the central and peripheral anterior chambers.Its incidence is about 0.6%-4.0%.It can be secondary to filtering surgeries,laser iridotomy,and cataract surgery.Short axial length and a history of angle closure glaucoma are its main risk factors.Here,we report a bilateral malignant glaucoma with bullous keratopathy in the patient’s left eye.CASE SUMMARY We present a case of bilateral malignant glaucoma.The cause of malignant glaucoma for each eye of this patient was different.Hence,the management strategy and selection of surgical methods were also different.However,the normal anterior chamber was ultimately maintained,and maximum visual function was preserved.Even though the left eye received multiple surgeries and corneal endothelial decompensation occurred,the formation of a retroendothelial fibrous membrane partially compensated for the function of the corneal endothelium.CONCLUSION The formation of a retroendothelial fibrous membrane partially compensated for the function of the corneal endothelium.

Rituximab Therapy for Persistent, Severe and Extensive Idiopathic Bullous Pemphigoid

Background: Idiopathic Bullous Pemphigoid (IBP) is a rare blistering autoimmune disease. Its morbidity and mortality have remained high owing to complications of extensive skin involvement as well as its conventional steroid therapy. We reviewed the medical literature and found indicators of an autoimmune etiology for its pathogenesis triggering genetically predisposed patients. Objective: to evaluate, prospectively, the role of Rituximab (R) therapy in its persistent, severe and extensive form. Patients and methods: A total of 12 patients, with disease duration of 6 ± 1 months, were treated with yearly R infusions (1 g followed by 1 g 2 weeks later). Results: Significant clinical improvement was achieved as documented by decrease in total score of Bullous Pemphigoid Disease Area Index from 60 ± 3 to 6 ± 2 that persisted for 26 ± 11 months of follow up. Moreover, IBP autoantibodies (anti-BP 180 and anti-320 IgG) levels fell from to 91 ± 3 and 81 ± 2 to 8 ± 2 and 9 ± 2, respectively. Conclusions: R is a safe and effective treatment for severe IBP and such response further confirms its autoimmune pathogenesis.

Recent advancement on autoantigens, autoantibodies and inflammatory cells in subepidermal autoimmune bullous diseases

Subepidermal autoimmune bullous diseases （SABD） are some autoimmune skin diseases that can present in a variety of forms and can be a challenging disease to treat. An overview of the different forms of SABD are discussed including bullous pemphigoid （BP）, epidermolysis bullosa acquisita （EBA）, cicatricial pemphigoid （CP）, bullous systemic lupus erythematosus （BSLE）, and Anti-p200 pemphigoid. Emphasis on recent advancement is presented. In recent years, improved knowledge of the mechanisms of intercellular and cell-matrix adhesion has led to better understanding of the blistering process in some SABD. Defects of such structures cause the subepidermal bullous diseases and have also led to the discovery of new diseases （e.g. anti-p200-pemphigoid）. Recent studies have outlined the important role of autoantibodies, mast cell lymphocytes and their cytokines in pathogenesis of SABD.

Urticarial Bullous Pemphigoid: A New Case Report

Background: Bullous pemphigoid (BP) is the most common autoimmune bullous disease and it primarily affects the elderly population. It typically presents with tense bullae and severe pruritus. Non-bullous pemphigoid is a subtype of BP characterized by lacking the typical bullae formation with different presentations including erythematous, eczematous, urticarial, polycyclic, targetoid, nodular, lichenoid, vesicular and erythrodermic. Aim: to document a new case presentation of urticarial BP who was treated for several years as chronic urticarial and chronic eczema. Case Report: A 56-year-old male patient presented with a history of recurrent severely pruritic urticarial wheals for 4 years duration involving the lower abdomen, lower back, upper and lower extremities associated with excoriations, keratosis, and post-inflammatory hyperpigmentations on resolved sites, diagnosed as urticarial BP on histopathology & direct immunofluorescence study (DIF) and was improved on systemic doxycycline therapy. Conclusion: BP can be presented with atypical manifestation. Urticarial BP is a rare variant of non-bullous pemphigoid that should be taken into consideration in the differential diagnosis of an atypical urticarial wheal not responding to conventional therapy.

Esophagitis dissecans superficialis and autoimmune bullous dermatoses: A review

Esophagitis dissecans superficialis(EDS)is a rare and severe endoscopic finding characterized by sloughing of large fragments of esophageal mucosal lining.Although EDS has been reported in association with serious illnesses and certain medications,the pathophysiological association of autoimmune bullous dermatoses with EDS has gained remarkable attention.Among these dermatoses,pemphigus vulgaris and pemphigoid frequently present with various types of esophageal involvement including EDS.We review the pathophysiology and clinical features of this involvement with the presentation of our experiences.The importance of endoscopic evaluation of this entity is discussed.

Combined Descemet stripping automated endothelial keratoplasty and intravitreal dexamethasone implant for concomitant pseudophakic bullous keratopathy and cystoid macular edema

Dear Editor,Endothelial cell density decreases with age and in various ocular conditions,including corneal endotheliitis,uveitis,pseudoexfoliation syndrome,and birth injury(1)The reduction of endothelial cell density is exacerbated over time after intraocular surgery(1)Descemet stripping automated endothelial keratoplasty(DSAEK)is considered the primary procedure for patients with only endothelial dysfunction.

Staphylococcal bullous impetigo in a neonate

An otherwise healthy, full-term neonate presented at day 15 of life to the pediatric emergency with generalized papulo-pustular rash for 2 d. This was finally diagnosed as bullous impetigo caused by Staphylococcus aureus(S. aureus). The skin lesions decreased significantly after starting antibiotic therapy and drainage of blister fluid. There was no recurrence of the lesions on followup. This case of generalized pustular eruption due to S. aureus in a neonate is reported, as it poses a diagnostic dilemma and can have serious consequences if left untreated.

A Retrospective and Comparative Study of Familial and Non-familial Bullous Lichen Planus

In order to compare the clinical characteristics of familial and non-familial bullous lichen planus (BLP), the archival data of 36 BLP patients with positive family history and 21 BLP patients with negative family history diagnosed according to the clinical features and histopathology were collected in our department from 1956 to 2003. The clinical features were analyzed and compared. There were significant differences between familial and non-familial BLP in age of onset, duration of disease and extension of eruption (P<0.01). It was concluded that familial BLP appeared to differ from the non-familial form in its earlier age of onset, longer duration of the disease, more extensive eruption and more tendency to involve nails. Hereditary factors may play a role in the pathogenesis of familial BLP.

Intravitreous injection of conbercept for bullous retinal detachment:A case report

BACKGROUND Diffuse retinal pigment epitheliopathy(DRPE)associated with bullous retinal detachment is a severe variant of DRPE that is frequently misdiagnosed and often improperly treated.CASE SUMMARY A 36-year-old female patient complained of"painless vision decline in the left eye with obscuration for 10 d".Slit-lamp microscopic fundus examination revealed white-yellow subretinal exudates in the posterior pole in both eyes,retinal detachment with shifting subretinal fluid in the left eye,and no retinal hiatus.Fundus fluorescein angiography revealed multiple subretinal leakage foci and localized hypofluorescent lesions with patched hyperfluorescence.There was fluorescence leakage in the retinal vessels in the retinal detachment area and occluded blood vessels in the lower and peripheral areas.Indocyanine green angiography revealed multifocal lamellar hyperfluorescence in the middle stage and low fluorescence in the retinal detachment area in the late stage.Retinal anatomical reduction significantly improved with intravitreal conbercept injections.CONCLUSION Intravitreal injection of conbercept can anatomically reattach the retina in patients with bullous retinal detachment.

Experience with the surgical treatment of patients with both esophageal carcinoma and bullous emphysema

Objective： We aimed to investigate the security and feasibility of the simultaneous surgery for patients with both esophageal carcinoma and bullous emphysema. Methods： We described simultaneous surgery performed on 49 cases with both esophaoeal carcinoma and buUous emphysema, accounting for 2.5% of all esophagectomy patients from January 2000 to January 2003. Radical resection of upper and mid-thoracic esophageal cancer was performed in 31 cases, including three approaches from the right chest, left neck and midsection. Thirty-six patients were underwent cervical anastomosis and 13 cases were operated by intrathoracic anastomosis. Results： No perioperative period death occurred. And postoperative com- plications were as follows： cervical anastomotic leakage in 9 cases, lung infection in 11 cases, pulmonary air leak in 13 cases （2 cases lasted for 4 weeks）, recurrent laryngeal nerve damage in 4 cases, supraventricular tachycardia in 4 cases. Patients all recovered and left the hospital with average hospitalization time of 17.5 days. Conclusion： Patients with both esophageal carcinoma and bullous can perform the esophageal carcinoma resection and lung volume reduction surgery （LVRS） simulta- neously. It will not increase the mortality rate and show the feasibility and safety in patients.

Topical use of ozone effectively alleviates the acute symptoms and quality of life of patients with moderate to severe bullous pemphigoid: a randomized controlled trial

Objective:To evaluate the adjuvant treatment of acute stage symptoms and quality of life of patients with bullous pemphigoid(BP)with ozone.Methods:We included 74 patients with moderate and severe BP hospitalized from July 2018 to December 2020.The participants were randomly divided into the control group and the ozone group,with 37 patients in each group(n=37).The control group used tap water to wash the whole body,and mupirocin ointment was used for the erosions.The ozone group used ozone water to wash the whole body,and the erosion place was coated with ozone oil.The bullous pemphigoid disease area index(BPDAI)score of BP disease was used to observe the clinical symptoms and skin lesions of the two groups before and on day 3,7,and 14 after treatment.Pruritus and sleep were observed before and on day 3,7,and 14 after treatment.The Dermatology Life Quality Index(DLQI)was used to observe the quality of life before and after treatment and the adverse reactions were recorded.Results:The total BPDAI score and erythema score in the ozone group were significantly different from those in the control group on day 3,7,and 14 after treatment(P<0.050).The scab drying time and erosion drying time of ozone group were shorter than that of control group(P<0.001).There were statistically significant differences between the ozone group and the control group in pruritus score and sleep score on day 3,7,and 14 after treatment(P<0.001).There was statistically significant difference in skin disease quality of life between the two groups 14 days after treatment(P<0.001).No significant adverse reactions and complications were observed in both groups.Conclusions:Ozone external use can rapidly improve the acute stage of edema erythema,erosion,and itching of BP,improve the quality of life of patients,is an effective treatment method,and wor thy of clinical promotion.

Clinical Efficacy of Plasma Exchange Therapy for Treatment of Autoimmune Bullous Skin Disease

Objective:To investigate the clinical efficacy of plasma exchange therapy for autoimmune bullous skin disease.Methods:Fifty patients with autoimmune bullous skin disease enrolled in our hospital from January 2018 to January 2019 were selected.The patients were grouped by treatment method:25 control group patients were given conventional hormone therapy,while 25 experimental group patients were treated with plasma exchange therapy;efficacy of treatment was compared between two groups of patients.Results:Initial dose,maximum dose,and cumulative dose of glucocorticoids were lower in experimental group patients than those in control group(P<0.05).Incidence of complication was lower in experimental group patients than those in control group(P<0.05);the difference was significant.There was no significant difference in short-term efficacy between the two groups(P>0.05).Conclusion:The application of plasma exchange therapy was effective for treatment of autoimmune bullous skin disease.It could reduce dosage amount of glucocorticoids and incidence of complications;its application can be promoted.

Expression of MMP-2 and MMP-13 in Bullous Pemphigoid

Objective:To investigate the expression and significance of matrix metalloproteinase-2(MMP-2)and matrix metalloproteinase-13(MMP-13)in bullous pemphigoid(BP)skin lesions.Methods:Immunohistochemical SP method was used to detect the expression of MMP-2 and MMP-13 in 32 BP skin lesions,and compared with 15 normal skin tissues.Results:The expression of MMP-2 in the case group was significantly increased(38.56±10.06)compared to the normal control group(21.20±5.98);the expression of MMP-13 in the case group was significantly augmented(18.62±5.90)compared to the normal control group(11.47±8.484).The expressions of MMP-2 and MMP-13 in the skin lesions of patients with bullous pemphigoid were statistically different from those of normal people(both P<0.05).Compared with the expression of MMP-2 and MMP-13 in bullous pemphigoid,the expression of MMP-2 and MMP-13 was moderately correlated(correlation coefficient was 0.523).Conclusion:The expression of MMP-2 and MMP-13 is significantly increased in bullous pemphigoid skin lesions,suggesting that they may play an important role in the pathogenesis of BP.There is a certain correlation between the expression of MMP-2 and MMP-13,suggesting that the high expression of MMP-13 may play a role in the mechanism that further leads to the high expression of MMP-2.

Rare bullous pemphigoid during PD-1 inhibitor therapy:a case report

Immunotherapy is an important treatment modality in cancer,but it can also cause adverse reactions,with skin toxicity being the most common.The increasing number of immune checkpoint inhibitors being used in the clinic will inevitably cause an increase in the rate of adverse skin reactions that markedly affect the patient's quality of life.A 58-year-old patient with intrahepatic cholangiocareinoma developed bullous pemphigoid(BP)nearly a year after using immune checkpoint inhibitors,which is different from what has been reported inthe literature within two weeks of treatment.Pathologically,the skin biopsy diagnosis was epidermal hyperplasia and focal sub-epidermal pustule formation,consistent with drug-induced dermatitis.The patient was treated with methylprednisolone,minocyeline,colchicine,nicotinamide,triamcinolone,and traditional Chinese medicine decoction.No new blisters developed after 1 week of treatment.The medication was gradually discontinued,and BP did not recur.Clinicians should carefully consider the risk-benefit ratio when using PD-1 inhibitors,particularly concerning rash severity.Further studies are needed to investigate the relationship between adverse skin reactions and drug efficacy.

Bullous Pemphigoid Induced by Doxycycline: Case Report and Literature Review

Bullous pemphigoid (BP) is the most prevalent autoimmune sub-epidermal blistering disease that affects mainly the elderly and could lead to serious morbidity. It has numerous risk factors and triggers, including an aging population with several comorbidities and drug exposure. In the published paper, we reported a case about a 32 years old male patient with unknown medical conditions who presented with erythematous patches and plaques on the scalp, face, and trunk with scattered blisters two weeks after initiating doxycycline treatment for folliculitis. The exact pathogenesis of the drug-reaction in drug- associated bullous pemphigoid (DABP) remains controversial. In conclusion, it is crucial for clinicians to be aware of DABP when prescribing doxycycline. The purpose of this case report is to raise awareness of the possible association between bullous pemphigoid and doxycycline.

Characteristics and Risk Factors of Infections among Patients with Autoimmune Bullous Diseases: A Retrospective Single-Center Study in China

Background: Since infections can lead to adverse outcomes in autoimmune bullous diseases (AIBD), this study aimed to analyze infection characteristics in AIBD, predict infection risk factors and provide probable suggestions to infection prevention, which will spot a light on early discovery and prevention of infections in AIBD patients. Methods: This is a retrospective study of the medical records of inpatients diagnosed with AIBD at the Department of Infectious Diseases and Dermatology, Huashan Hospital from January 2017 to December 2021. We collected patients’ clinical manifestations and laboratory examination results. Risk factors for infections were evaluated using multivariate logistic regression. Results: A total of 263 AIBD patients were included in the study. The overall incidence of infection was 42.2% (111/263). Pemphigus had a higher infection rate (74/142, 52.1%) than bullous pemphigoid (37/121, 30.6%). Among the patients with infections, 49.5% (55/111) had bacterial infections, 13.5% (15/111) had fungal infections, 2.7% (3/111) had virus infections, and 34.2% (38/111) had mixed infections. Staphylococcus aureus and Candida were the most common pathogens in localized infections, while Cryptococcus and Candida were predominant in systemic infections. In the multivariate logistic model, pemphigus (odds ratio [OR] = 2.56, 95% confidence interval [CI] 1.41-4.63), hypoalbuminemia (OR = 3.78, 95% CI 1.68-8.50), and systemic glucocorticoid treatment (OR = 4.67, 95% CI 2.51-8.68) were independent risk factors for infection in AIBD patients. Bullous pemphigoid (OR = 3.99, 95% CI 1.03-15.45), high-dose (average > 24 mg/day) compared to low-dose (average ≤ 6 mg/day) and medium-dose (average 6-24 mg/day) systemic glucocorticoid treatment (OR = 19.31, 95% CI 3.32-112.28, and OR = 5.71, 95% CI 1.37-23.79) were associated with increased risk factors of systemic infection in AIBD patients with systemic glucocorticoid treatment. A methylprednisolone dose averaging 14 mg/day over the past three months had a 75.0% positive predictive value for infections in AIBD patients. Conclusions: The incidence of infection in AIBD patients is high, with a broad spectrum of pathogens and infection locations. AIBD patients undergoing systemic glucocorticoid treatment, especially those receiving high doses in the previous three months, should be cautious about infection.

Preliminary Application of High-Frequency Ultrasound in the Differentiation of Pemphigus and Bullous Pemphigoid:An Observational Study

Objective:Ultrasonography is a diagnostic imaging technique used to visualize subcutaneous body structures for identification of possible pathologies.In this study,we aimed to explore the clinical significance of high-frequency ultrasound in differentiating the location of blisters between pemphigus and bullous pemphigoid.Methods:Eighteen patients were recruited in Department of Dermatology,Zhongda Hospital from 2020 to 2021 and divided into a pemphigus group(n=8)and a bullous pemphigoid group(n=10)according to the diagnostic criteria for each.Ultrasonographic images were collected using a 50-MHz high-frequency ultrasound system.The indexes of ultrasonic evaluation were the layer(epidermis or dermis),size,shape,internal echo,and boundary of the blister.Categorical variables are expressed as n(%),and differences were compared using Fisher’s exact test.Results:The ultrasonographic images in the pemphigus group showed an intraepidermal semi-arc or irregular anechoic or hypoechoic areas.The inferior borders were situated above the characteristic thin linear hyperechoic bands of the epidermis.A linear hypoechoic band was present at the dermoepidermal junction in some cases.In the ultrasonographic images of the bullous pemphigoid group,the linear hyperechoic bands of the epidermis were continuous and intact.An oval fluid anechoic area(subepidermal blister)was present immediately below the bands.The location of the blisters in the ultrasonographic images was significantly different between the 2 groups(P<0.001).Conclusion:High-frequency ultrasound can be used as an auxiliary means to differentiate the location of blisters between pemphigus and bullous pemphigoid.

Bullous pemphigoid associated with acquired hemophilia A: A case report

BACKGROUND Acquired hemophilia A (AHA) is a rare and potentially severe bleeding disordercaused by circulating autoantibodies against factor Ⅷ (FⅧ). In approximately50% of the patients, the condition is associated with autoimmune diseases,cancers, medication use, pregnancy, and the post-partum period. Bullous pemphigoid(BP) is a chronic autoimmune subepidermal blistering disease associatedwith tissue-bound and circulating autoantibodies against BP antigens 180 (BP180)and 230 (BP230). AHA-associated BP has a high mortality rate;hence, the understandingof this disease must improve.CASE SUMMARY A 69-year-old man presented with erythema, blisters, blood blisters, and crustsaccompanied by severe pruritus for more than 20 days, and ecchymosis andswelling on his left upper arm for 3 days. Pathological examination revealed asubepidermal blister that contained eosinophils. Laboratory tests showed that theBP180 autoantibody levels had increased, isolated activated partial thromboplastintime was notably prolonged (115.6 s), and coagulation FⅧ activity wasextremely low (< 1.0%). Furthermore, the FⅧ inhibitor titer had greatlyincreased (59.2 Bethesda units). Therefore, the patient was diagnosed as having BP associated with AHA, prescribed 0.05% topical halometasone cream, and transferred to a higher-level hospitalfor effective treatment;however, he died after 2 days.CONCLUSION AHA associated BP is rare, dangerous, and has a high mortality rate. Therefore, its timely diagnosis and effectivetreatment are necessary.

Pilomatricoma With Bullous Appearance:A Case Report

Introduction:Pilomatricoma is usually a solitary,deep dermal firm nodule with an overlying normal epidermis.Bullous pilomatricoma is rarely reported;thus,its pathogenesis has not been elucidated.We report a case presenting the pilomatricoma with bullous change.Case presentation:A 19-year-old female presented a firm nodule covered by a bulla on her right upper arm.Histopathological examination showed the tumor was composed of basophilic basal-like cells and shadow cells.No cellular atypia or abnormal mitotic figures were observed.All these clinical characteristics and pathological results point to the diagnosis of bullous pilomatricoma.The tumor was surgically removed by enlargement.Discussion:We hypothesize that a history of trauma and rapid growth may contribute to the diagnosis of the bullous pilomatricoma.The appearance of the bullous can be explained by the dilated lymphatic vessels seen in histopathologic examination.We give a case report help clinicians to better diagnose this entity and decrease the rate of misdiagnosis.Conclusion:We observed a rare bullous variant of pilomatricoma.Hence,when identifying a patient of bullous pilomatricoma,a detailed medical history and physical examination are essential.