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Background: Information on bullous pemphigoid in an Indian context is scarce. Aim: To report clinico-demographic profile, associated comorbidities and prescription pattern of bullous pemphigoid patients in India. Methods: This was a retrospective study, where past records of all bullous pemphigoid patients diagnosed and treated between November 2013 and October 2019 were accessed and analysed. Patients having a compatible clinical presentation with either histopathological and/or direct immunofluorescence evidence of bullous pemphigoid were included. Results: There were 96 bullous pemphigoid patients, with a male: female ratio of 1.6:1. The mean age at diagnosis was 62.5 ± 2.2 years, with mean duration of illness 27.5 ± 4.5 months before presentation. Comorbidities were present in 80 (83%) patients, with type 2 diabetes mellitus (38.5%), hypertension (36.4%) and neurological illness (16.7%) being the commonest ones. Clinically, blisters were the predominant presentation in 81 (84.4%) patients. The majority (87.5%) of patients showed a predominant eosinophilic infiltrate on histopathology. Direct immunofluorescence revealed immunoglobulin G deposits with complement C3 in 77 (80.2%) cases. The majority of patients (77.1%) were treated with oral prednisolone, either alone (11.5%) or in combination (65.6%) with other topical and systemic agents. Topical steroids were used in 29.1%, azathioprine in 28%, dapsone in 16.7% and omalizumab in 6.2% of patients. Limitations: The study is retrospective. Immunofluorescence on salt split skin, direct immunofluorescence serration pattern analysis, and immunoblotting were not performed. Hence, there is a possibility that a few included cases were suffering from other subepidermal autoimmune bullous diseases like epidermolysis bullosa acquisita or anti-p200 pemphigoid. Conclusion: Bullous pemphigoid patients in this study had a younger age of onset and showed male preponderance. Comorbidities like type 2 diabetes, hypertension and neurological disorders were freq
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Introdução: A angina bolhosa hemorrágica (ABH) é uma condição rara, benigna e geralmente assintomática, caracterizada pelo surgimento súbito de bolhas preenchidas com sangue nas superfícies mucosas da cavidade oral e orofaringe. Objetivo: Este trabalho tem como propósito fornecer uma análise abrangente das características clínicas, etiológicas e histopatológicas da angina bolhosa hemorrágica, além de abordar métodos de diagnóstico e opções de tratamento. Materiais e métodos: Foi realizada uma busca por artigos científicos publicados de 2010 a 2023, nas bases de dados Scientific Electronic Library Online (SciELO), US National Library of Medicine (PubMed) e ScienceDirect. Foram coletados artigos em inglês e português utilizando as palavras-chave "angina bolhosa hemorrágica", "estomatite bolhosa hemorrágica benigna", "hemorrhagic bullous angina" e "benign hemorrhagic bullous stomatitis". Conclusão: A ABH é escassamente documentada na literatura, com muitos dados ausentes ou subnotificados. Embora seja uma condição benigna com rápida evolução espontânea, o procedimento diagnóstico deve ser rigoroso para descartar outras possíveis lesões.
Introduction: Hemorrhagic bullous angina (ABH) is a rare, benign and generally asymptomatic condition, characterized by the sudden appearance of blisters filled with blood on the mucous surfaces of the oral cavity and oropharynx. Objective: This work aims to provide a comprehensive analysis of the clinical, etiological and histopathological characteristics of hemorrhagic bullous angina, in addition to addressing diagnostic methods and treatment options. Materials and methods: A search was carried out for scientific articles published from 2010 to 2023, in the Scientific Electronic Library Online (SciELO), US National Library of Medicine (PubMed) and ScienceDirect databases. Articles were collected in English and Portuguese using the keywords "hemorrhagic bullous angina", "benign hemorrhagic bullous stomatitis", "hemorrhagic bullous angina" and "benign hemorrhagic bullous stomatitis". Conclusion: ABH is scarcely documented in the literature, with many data missing or underreported. Although it is a benign condition with rapid spontaneous evolution, the diagnostic procedure must be rigorous to rule out other possible lesions.
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Background: Bullous pemphigoid is the most common subepidermal autoimmune blistering disease. Till now, the reported prognostic factors in bullous pemphigoid vary considerably. Aims: The purpose of this study was to determine the overall survival rate and prognostic factors in bullous pemphigoid. Methods: We conducted a retrospective cohort study on newly diagnosed bullous pemphigoid patients between July 2001 and November 2019 in a referral unit for autoimmune blistering skin diseases in Romania. Results: One hundred forty-eight patients were included in the study. The Kaplan–Meier overall survival rates at 1, 3, 5 and 10 years were respectively 74.2% (95% confidence interval, 67.5–81.6%), 53.4% (45.7–62.2%), 43.6% (35.9–53%) and 31.3% (23.5–41.7%). The median follow-up among survivors was 48 months (interquartile range: 11–150). Ninety (60.8%) patients died during the follow-up period; of them, 38 (42.2%) had active disease at the time of death. Advanced age, neurological diseases, valvular heart disease, malignancies, use of statins, skin infections and extensive cutaneous involvement were linked to poorer outcomes, while the use of topical corticosteroids was associated with increased overall survival. Limitations: This study lacks a control cohort to validate the obtained results. It was conducted in a retrospective manner in a single centre. In addition, indirect immunofluorescence microscopy was not performed in all patients. Conclusion: Beyond ageing and neurological comorbidities, the prognosis of bullous pemphigoid patients was significantly influenced by the presence of skin infections, valvular heart disease, use of statins and extensive cutaneous involvement. Topical corticosteroid treatment was associated with increased survival in these patients
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Context: Bullous pemphigoid (BP) and “Pemphigus diseases” (PD) can have overlapping clinical manifestations and accurate distinction is crucial for appropriate management. Aims: The study aimed at analyzing the utility of simple hematological markers of systemic inflammation like neutrophil-to-lymphocyte ratio (NLR), neutrophil-to-eosinophil ratio (NER), and platelet-to-lymphocyte ratio (PLR) in clinical decision making in the setting of clinical differentials of BP and PD in a particular case. Methods: This single-centre based retrospective observational analytical study included adult subjects newly diagnosed to have BP (n=66) or PD (n=53), confirmed with direct immune-fluorescence testing, over a period of six years. Blood counts performed using Coulter™ hematology analyser, at the time of their initial presentation, were retrieved from the hospital medical records, and the leucocyte ratios were calculated.Statistical Analysis: The data were compared between the two groups, using Mann–Whitney U test and chi-square test /Fisher's exact test. ROC curve analysis was performed to estimate cut-off values. Results: The BP group had a significantly higher NLR, total leukocyte counts (TLC), absolute eosinophil counts (AEC), and absolute lymphocyte counts (ALC), and lower NER values compared to the PD group (P < 0.05). Areas under ROC for NLR, NER, TLC, AEC, and ALC were between 0.5 and 0.7. NLR ? 7, AEC ? 2055/cumm, and TLC ? 15,000/cumm had a specificity of 90.6, 100, and 100% respectively for identifying BP patients out of the two groups, but with a low sensitivity of 22.7, 21, and 22.7%, respectively. Conclusions: NLR can be a valuable diagnostic adjunct in subtyping autoimmune bullous disorders, albeit in a small proportion of cases.
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Introducción: El penfigoide ampolloso es una dermatosis vesicular ampollosa autoinmunitaria subepidérmica, asociada a la formación de autoanticuerpos que reconocen autoantígenos en la zona de la membrana basal. El tratamiento inmunomodulador con corticoides es la primera línea en el control de la enfermedad. Objetivo: Presentar el caso clínico de un paciente con diagnóstico de penfigoide ampolloso de origen idiopático. Caso clínico: Paciente masculino de 81 años con lesiones ampollosas dolorosas a la palpación de contenido serohemático, tamaño variado, bordes regulares y base eritematosa a nivel generalizado. La evaluación inicial sugiere diagnóstico de penfigoide ampolloso e infección bacteriana activa de las lesiones en la piel. Se solicitan exámenes de laboratorio e imágenes diagnósticas, se descartan etiologías infecciosas, autoinmunes o neoplásicas asociadas, se inicia tratamiento con corticosteroides intravenosos con adecuada evolución clínica. Finalmente, no se identifican enfermedades asociadas a las lesiones ampollosas del paciente. Conclusión: El penfigoide ampolloso es una entidad poco frecuente, con una elevada tasa de mortalidad si se realiza un diagnóstico y tratamiento tardío. Reconocer las principales manifestaciones y variantes clínicas de esta enfermedad permite un oportuno enfoque diagnóstico y terapéutico, este último basado en el control de la respuesta inflamatoria contra la piel y otros órganos.
Introduction: Bullous pemphigoid is a subepidermal autoimmune bullous vesicular dermatosis associated with the formation of autoantibodies that confirm autoantigens in the basement membrane area. Immunomodulatory treatment with corticosteroids is the first line in the control of the disease. Objective: To present the clinical case of a patient diagnosed with a bullous pemphigoid of idiopathic origin. Clinical case: 81-year-old male patient with blistering lesions that are painful on palpation with serohematic content, varied in size, regular borders and a generalized erythematous base; that the initial evaluation suggests a diagnosis of bullous pemphigoid and active bacterial infection of the skin. Laboratory tests and diagnostic images are requested, infectious, autoimmune or associated neoplastic etiologies are ruled out, treatment with intravenous corticosteroids is started with adequate clinical evolution. Finally, no diseases associated with the patient's blistering lesions were identified. Conclusion: Bullous pemphigoid is a rare entity, with a high mortality rate if a late diagnosis and treatment is performed. Recognizing the main manifestations and clinical variants of this disease allows for a timely diagnostic and therapeutic approach, the latter based on the control of the inflammatory response against the skin and other organs.
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Abstract Background Significant association between bullous pemphigoid (BP) and stroke has been reported. This study aimed to evaluate the level of anti-BP180 antibody in stroke patients to explore the relationship between BP and stroke in their pathogenesis. Methods We collected serum samples from stroke patients and matched controls between February 2019 and June 2020. The anti-BP180 antibody levels were measured by enzyme-linked immunosorbent assay (ELISA). Results A total of 1183 stroke patients including 970 with cerebral infarction (CI), 192 with intracerebral hemorrhage (ICH), 21 with CI and ICH, and 855 controls were enrolled in this study. Anti-BP180 autoantibody values were significantly higher in stroke patients than in controls (p < 0.001). Anti-BP180 autoantibody-positive rates were 12.51% (148) in stroke patients and 4.68% (40) in controls (p < 0.001, OR = 2.65). In anti-BP180 autoantibody-positive subjects, the values were significantly higher in stroke patients than in controls (p < 0.001). However, only 10 (6.76%) stroke patients and 3 (7.5%) controls had high values (> 100 RU/mL) (p = 0.87). Stratified analysis showed that anti-BP180 antibody positive rates were independent of age, sex, and stroke subtypes in the stroke group. Positive rates in patients with both CI and ICH were nearly two times higher than those in patients with either CI or ICH alone (p = 0.11, OR = 1.94). Study limitations This study had a limited sample size and lacked quantitative criteria for stroke severity. Conclusions Anti-BP180 antibody values and positive rates were higher in stroke patients than in controls, suggesting that stroke patients may have higher of developing BP.
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Spontaneous pneumothorax (SP) is a serious and life-threatening condition often caused by ruptured apical lung bulla in young male individuals. It is commonly associated with different syndromes but also occurs in healthy individuals. In this case report, we aim to discuss the etiology, clinical course, and surgical treatment of a 21-year-old male kickboxer with a right-sided pneumothorax that occurred during a sparring session. A chest tube with negative suction was inserted to resolve the pneumothorax. Because there was no visible resolution, video-assisted thoracoscopic surgery (VATS) was performed. During VATS, a large, apically placed, ruptured lung bulla, was revealed and removed. One month after surgery, the patient is in great clinical condition. There are no signs of a recurrence of SP.
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Objective:To investigate and analyze the clinical manifestations and imaging features of the eyes with bullous retinal detachment.Methods:Retrospective case series study. Eleven eyes of 11 patients with bullous retinal detachment diagnosed in Department of Ophthalmology, Peking University People's Hospital from July 2015 to September 2021 were enrolled. There were 10 males and 1 female, with the mean age of (39.27±6.81) years. All patients had monocular bullous retinal detachment, with mean duration ranged from 3 months to 14 years. The basic information and medical history of all patients were collected. All patients underwent best corrected visual acuity (BCVA), indirect ophthalmoscopy, color fundus photography, optical coherence tomography (OCT), fundus fluorescein angiography (FFA), indocyanine green angiography (ICGA) and B-scan ultrasonography. BCVA was performed using a standard logarithmic visual acuity chart, which was converted to logarithm of the minimum angle of resolution (logMAR) visual acuity. The clinical data and imaging features of BCVA, OCT, FFA and ICGA were retrospectively analyzed and summarized.Results:The mean logMAR BCVA of the 11 eyes was 0.91±0.45. Nine patients had bilateral disease, but bullous retinal detachment occurred in only 1 eye, and CSC manifestations were present in the contralateral eye. Six patients had received systemic or topical hormone therapy prior to onset. Yellowish-white material was observed in 6 eyes and retinal folds were observed in 5 eyes. OCT examination showed serous retinal detachment in the macular area with granular or patchy hyperreflective signals in the subretinal area in all eyes, and a few granular hyperreflective substances in the neuroretina in 6 eyes. Neuroretina cystoid degeneration was observed in 6 eyes, adhesion between the detached neuroretina and retinal pigment epithelial (RPE) was observed in 6 eyes, RPE tear was observed in 6 eyes, and different forms of retinal pigment epithelial detachment (PED) were observed in 6 eyes. FFA showed multiple fluorescence leakage spots in 10 eyes, and the average number of fluorescence leakage spots in all eyes was 3.82±2.44. There were multiple diffuse RPE lesions in 9 eyes. The results of ICGA examination showed that choroidal vessels were dilated and multiple hyperfluorescent leaks were observed in all eyes. B-scan ultrasonography examination of all affected eyes showed retinal detachment. Retinal reattachment can be achieved at (2.0±1.0) months after photodynamic therapy (PDT), while SRF can be completely absorbed at (2.36±0.81) months. The mean logMAR BCVA can be improved to 0.50±0.33, and no recurrence was found in the follow-up period up to 6 months.Conclusions:Bullous retinal detachment is often associated with the use of hormones, while yellow-white material in the subretina and hyperreflective material in the OCT are common. It is characterized by neuroretina cystoid degeneration in the macular area, adhesion between the neuroretina and RPE, RPE tear and PED, with multiple fluorescence leakage spots and diffuse RPE lesions. PDT is an effective treatment for bullous retinal detachment.
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Epitope spreading often occurs in patients with autoimmune bullous diseases (ABDs), resulting in exposure of more antigenic epitopes, aggravation or transformation of pre-existing diseases, or concurrence of other diseases. With the increase in the immunological evidence for epitope spreading, more and more scholars have realized that epitope spreading plays an important role in the development of ABDs. This review introduces the phenomenon of epitope spreading in ABDs from 4 aspects, including the concurrence of or transformation between different types of pemphigoid, different types of pemphigus, pemphigus and pemphigoid, as well as between ABDs and other skin diseases.
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Objective:To investigate differences in clinical characteristics between bullous pemphigoid (BP) patients with stroke and those without, and their relationship with the prognosis of stroke.Methods:A retrospective analysis was performed on medical records of 330 BP inpatients in the First Affiliated Hospital of Zhengzhou University from September 2012 to April 2020. These patients were divided into BP + stroke (ST) group and BP - ST group according to whether they were accompanied by stroke, and clinical manifestations and relevant laboratory examination results were compared between the two groups. According to the stroke outcome score assessed by modified Rankin Scale (mRS), patients in the BP + ST group were further divided into good-prognosis ST group (mRS ≤ 2 points) and poor-prognosis ST group (mRS > 2 points), and subgroup analysis was conducted. Correlations between measurement data (such as age, disease course and laboratory examination results) and mRS scores were analyzed.Results:In the BP - ST group (256 cases), 151 were males and 105 were females, and their age ranged from 19 to 92 (66.8 ± 13.6) years; in the BP + ST group (74 cases), 45 were males and 29 were females, and their age ranged from 48 to 92 (74.6 ± 9.6) years; Compared with the BP - ST group, the BP + ST group showed older age ( t = -5.57, P < 0.001), shorter disease course of BP ( Z = -3.07, P = 0.002), and higher anti-BP180 IgG antibody levels (215.0 [157.2, 283.1] U/ml vs. 155.0 [63.9, 279.8] U/ml; Z = -2.12, P = 0.034). The distribution of skin lesions significantly differed between the two groups ( χ2 = 10.51, P = 0.015), and the BP + ST group showed a significantly lower proportion of patients with generalized lesions ( P<0.05), but a higher proportion of patients with lesions on the limbs ( P<0.05). Subgroup analysis showed significant differences in the patients′ age, BP course, lesion distribution and anti-BP180 IgG antibody levels among the good-prognosis ST group, poor-prognosis ST group and BP - ST group ( F = 10.83, P<0.001; Z = 17.24, P<0.001; χ2 = 15.57, P = 0.026; Z = 6.29, P = 0.043, respectively). There was no significant difference in the age between the good-prognosis ST group and poor-prognosis ST group (adjusted P = 1.000), but the patients were significantly older in the two above groups than in the BP - ST group (adjusted P = 0.001, 0.007, respectively) ; the poor-prognosis ST group showed significantly shorter BP courses (adjusted P = 0.016, < 0.001, respectively) and a higher proportion of patients with lesions on the limbs (both P < 0.05) compared with the good-prognosis ST group and BP - ST group, and significantly higher serum anti-BP180 IgG antibody levels compared with the BP - ST group (226.2 [163.6, 285.8] U/ml vs. 155.0 [63.9, 279.8] U/ml; adjusted P = 0.037). There were no significant differences in the gender distribution, lesional morphology, percentages and counts of peripheral blood eosinophils, serum total IgE levels, and anti-BP230 IgG antibody levels between the BP + ST group and BP - ST group (all P > 0.05), or among the good-prognosis ST group, poor-prognosis ST group and BP - ST group (all P > 0.05). Correlation analysis in the BP + ST group showed a significantly negative correlation between the BP course and mRS scores ( r = -0.33, P = 0.004), and a significantly positive correlation between the anti-BP180 IgG antibody levels and mRS scores ( r = 0.34, P = 0.032) . Conclusion:There were differences in the patients′ age, BP course, lesion distribution, and anti-BP180 IgG antibody levels between the BP patients with stroke and those without, and the differences were more obvious between the poor-prognosis ST group and BP - ST group.
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Objective:To retrospectively analyze clinical characteristics and treatment of pemphigus/bullous pemphigoid (BP) complicated by herpes simplex virus (HSV) infection.Methods:Inpatients with pemphigus/BP complicated by HSV infection were collected from Wuhan No.1 Hospital from 2016 to 2021, and their clinical characteristics, treatment and follow-up results were retrospectively analyzed.Results:Among the 8 patients with pemphigus/BP complicated by HSV infection, there were 2 males and 6 females, and their age was 50.6 ± 8.3 years. Five of them were diagnosed with pemphigus vulgaris (PV), 1 with pemphigus foliaceus (PF), and 2 with BP. Seven were infected with HSV-1, and 1 with HSV-2. All the 8 patients were given systemic glucocorticoids and immunosuppressive agents for the treatment of pemphigus or BP, and were admitted to the hospital due to resistance to the treatment. Seven patients presented with exacerbation or recurrence of primary lesions, and 1 presented with enlarged lesions all over the body. HSV infection-induced lesions were located on the trunk in 4 cases, on the oral mucosa in 4, on the scalp in 3, and on the face in 2; lesions mainly manifested as irregular erosions with blood crusts, and some centrally umbilicated pustules; 7 patients had obvious pain at the lesional sites. During HSV infection, anti-desmoglein 1 antibody levels decreased in all the 6 patients with pemphigus, and anti-desmoglein 3 antibody levels decreased in 4 of the 5 patients with pemphigus vulgaris; anti-BP180 antibody levels decreased in 1 patient with BP, but increased in the other one with BP. After antiviral therapy at adequate doses for adequate durations (7- to 14-day treatment with valacyclovir alone or in combination with ganciclovir), HSV infection was controlled, the autoimmune bullous skin disorder intensity scores decreased compared with those before the antiviral therapy, and pain was significantly relieved in all the patients. No dose adjustment of glucocorticoids or other immunosuppressive agents was made during antiviral therapy in all patients.Conclusion:HSV infection should be considered when patients with pemphigus/BP suffer from recurrence or exacerbation and poorly respond to conventional treatment; for patients with pemphigus/BP complicated by HSV infection, systemic antiviral therapy at adequate doses can be used to control the disease condition without modifying the conventional immunosuppressive regimen.
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A 10-day-old male infant presented with skin erythema and blisters for 6 days. Skin examination showed scattered or confluent erythema all over the body, tense blisters of varying sizes on the normal skin or an erythematous base, and some blisters were ulcerated and erosive; bloody bullae and erythematous erosive patches could be seen on the oral mucosa. Histopathological examination revealed subepidermal blisters, and there were some neutrophils and a few eosinophils in the blisters. Direct immunofluorescence assay showed homogeneous linear IgA and granular C3 deposits along the basement membrane zone, without IgG deposits. The diagnosis of neonatal linear IgA bullous dermatosis was confirmed. After comprehensive treatments including nutritional support and anti-infection treatment, skin erythema and blisters subsided, and the mucosal damage was attenuated. The telephone follow-up 16 months after discharge showed that the infant was in good general condition with normal growth and development, and the oral mucosal lesions had subsided and healed, without new skin lesions.
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RESUMEN La dermatosis ampollar por IgA lineal del adulto (DLA) es una enfermedad autoinmune adquirida infrecuente, caracterizada por el depósito lineal de anticuerpos IgA en la membrana basal. La mayoría de los casos reportados son de causa idiopática, pero esta entidad también se ha visto asociada a ciertos fármacos, siendo la vancomicina el más frecuente. Se presenta un caso de DLA asociada a vancomicina, con extensa afectación cutánea y compromiso mucoso, tratado con dapsona y corticoides sistémicos con buena respuesta.
ABSTRACT Adult linear IgA bollous dermatosis (LABD) is a rare acquired autoimmune disease characterized by linear deposition of IgA antibodies on the basement membrane. Most of the reported cases are of idiopathic cause, but this entity has also been associated with certain drugs, vancomycin being the most frequent. We present a case of LABD associated with vancomycine, with extensive skin and mucosal involvement, treated with dapsone and systemic corticosteroids with a good response.
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Tuberculosis, although an ancient disease, affecting humans for millennia; continues to present in atypical ways. Whilst the development of bullous lung disease in patients suffering from Pulmonary Tuberculosis, has been reported, it remains an extremely rare phenomenon. The underlying mechanisms remain unknown, and potential causes have been hypothesized, including the role of Isoniazid as part of antitubercular therapy. We reportedone such case of a rapid development of bullous lung as a complication of Pulmonary Tuberculosis.
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Abstract Background: Bullous pemphigoid (BP) associated with milia lesions has been increasingly reported, but its prevalence has not been reported in a Brazilian BP population yet. Objectives: To describe the occurrence and clinical-laboratorial findings of BP-milia association in a southeastern Brazilian sample. Methods: A descriptive study based on the medical charts of 102 BP patients was accomplished. Clinical and laboratory data of BP-milia patients were compiled. Total serum IgE measurements, immunoblot assays based on basement membrane zone antigens, and HLA-DQ alleles typing were performed. Results: Milia was evident in 8 (7.8%) BP patients, five males, aged between 46 and 88 years. Increased total IgE levels were determined in 7 (87.5%) of the eight patients. In five of eight patients, immunoblotting showed IgG reactivity against the BP180-NC16a domain but not against collagen VII or laminin-332; it also revealed reactivity against the BP180 C-terminal domain or LAD-1, or both in four of them. The HLA-DQB1*03:01 and HLA-DQA1*05:05 alleles were identified in three of five BP-milia patients. Moreover, three of five cases presented the HLA-DQB1*06 allelic group. Study limitations: HLA determination was performed in five patients. Conclusions: Milia formation in BP patients seems to be less uncommon than previously admitted. Laboratory data revealed increased IgE; autoantibodies against the BP180 C-terminal domain or LAD-1, or both; and the HLA-DQB1*06 allelic group, described for the BP-milia association. Careful determination of antibodies against basement membrane zone molecules and HLA characterization in different populations may provide further insights into this association. © 2022 Sociedade Brasileira de Dermatologia. Published by Elsevier España, S.L.U. This is an open access article under the CC BY license (https://creativecommons.org/licenses/by/4.0/).
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Scabies is an ectoparasitic infection caused by Sarcoptes scabiei var. hominis in humans. Accurate figures of the prevalence of scabies are difficult to obtain, it seems to affect around 100�0 million people worldwide. Scabies is usually transmitted by close intimate physical contacts, such as the sharing of a bed or prolonged hand-holding. Scabies mites survive for 24� h outside of human bodies. Itching is the most obvious manifestation of scabies, which is usually sparing the face in adult classic scabies. The onset occurs 3�weeks after acquiring the infection. Bullous pemphigoid-like scabies is one of the atypical and rare forms of scabies that may be overlooked and mismanaged, where only 44 cases worldwide with this mode of presentation were reported. Here, we reported a series of three cases of bullous pemphigoid-like scabies, which presented with nocturnal itching and vesiculobullous lesions that are to the best of our knowledge the first cases reported in our country.
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Introduction: Bullae are air filled spaces in lung parenchyma which develop in various diseases and cause respiratory compromise. Bulla which occupies one third of hemithorax is considered as a giant bulla. Case series: This case series consists of eleven cases of giant bullae with varied clinical and radiological features. Common presenta?ons include breathlessness, associated para-septal emphysema and complica?ons like pneumothorax. Conclusion: Pa?ents presen?ng with acute chest symptoms should also be evaluated for bullous disease and Computed tomography of chest can differen?ate pneumothorax from giant bullae.
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Hypoglycemic drugs in the dipeptidyl peptidase-4 (DPP-4) inhibitor class are used as a second-line treatment for type 2 diabetes mellitus. With DPP-4 inhibitors, there have been a few reports of cutaneous side effects such as bullous response, fixed drug eruption, and photosensitivity. There is no definitive pathophysiology for the above mentioned allergic reactions. Sitagliptin phosphate belongs to the DPP-4 inhibitor class. This is a case report of a sitagliptin-induced bullous drug reaction manifesting three weeks after starting therapy. He had bullous pemphigoid-like eruptions all over the body. The patient showed improvement once sitagliptin was discontinued alon with oral and topical steroid treatment.
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Objective:To retrospectively analyze the efficacy and safety of dupilumab in the treatment of bullous pemphigoid (BP) .Methods:Clinical data were collected from BP patients who received injections of dupilumab at an initial dose of 600 mg followed by an every-2-week regimen at a dose of 300 mg (the frequency of injections could be increased if necessary) in Department of Dermatology, Peking University First Hospital from October 2020 to October 2021, and their clinical manifestations and changes in laboratory indices were analyzed.Results:A total of 21 BP patients treated with dupilumab were included in this study. Nineteen (90.5%) patients achieved complete or marked disease control after 2-week treatment with dupilumab; 12 patients were followed up for 16 weeks, and all maintained complete disease control at 16 weeks. All patients had a bullous pemphigoid disease area index (BPDAI) score of 122.5 ± 51.1 points at baseline, which decreased to 30.6 ± 27.4 points after 2-week treatment with dupilumab ( t = 8.53, P < 0.001) , and continued to decrease to 12.7 ± 9.1 points after 4-week treatment ( t = 9.73, P < 0.001) . Pruritus was markedly relieved in all the 21 patients within 4-week treatment with dupilumab. Among 10 patients with elevated eosinophil counts at baseline, the eosinophil counts markedly decreased in 9 after treatment. The serum IgE level was elevated in 7 patients at baseline, which markedly decreased in 6 after treatment. Viral conjunctivitis occurred in 1 (4.8%) patient, and no adverse reactions were observed in other patients. Conclusion:Dupilumab is effective in the control of BP and relief of pruritus, with a favorable safety profile.
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There are overlaps in the pathogenesis of autoimmune bullous diseases (AIBDs) and psoriasis. In recent years, there have been many reports on correlations between psoriasis and AIBDs. Bullous pemphigoid is the most common AIBD associated with psoriasis, followed by pemphigus vulgaris, pemphigus erythematosus and linear IgA bullous dermatosis. AIBDs occur after the onset of psoriasis in most cases, but simultaneously with or before the onset of psoriasis in some patients. This review summarizes correlations between AIBDs and psoriasis, as well as possible mechanisms.