Pemphigus vulgaris versus Mucous Membrane Pemphigoid: Case Series

Pemphigus vulgaris (PV) and mucous membrane pemphigoid (MMP) are bullous autoimmune diseases that reach the oral mucosa and have common clinical features. The objective of the study was to present and compare the clinical manifestations of PV and MMP and the results of applied treatments. A case series of a stomatology service from 1985 to 2018. Data collection included epidemiological data, comorbidities, medications in use, duration of symptoms before the first visit, previous treatment, symptomatology, clinical description of lesions, presumptive diagnosis, histopathological description, extraoral manifestations, final diagnosis, treatment and follow-up. The medical records of 25 patients were analysed, 19 of whom were diagnosed with MMP and 6 with PV. The female gender was prevalent in MMP (84 %) and the male gender in PV (67 %). More than 60 % of patients complained of pain at their first visit. Patients with MMP took on average 6 months to seek professional help and patients with PV, about 2 months. Desquamative gingivitis was the most common lesion (63 %) in MMP and non-gingival ulcers (67 %) in PV. Minimal therapy was effective in all cases of MMP, and in PV one individual required minimal adjuvant therapy due to worsening of the case. Patients with PV have more intense signs and oral symptoms and may need more intensive treatment than patients with MMP. The use of topical and/or systemic corticosteroids was sufficient for most cases in both diseases.

Pénfigo vulgar (PV) y Penfigoide de la Membrana Mucosa (PMM) son enfermadades autoinmunes ampollosas que llegan a la mucosa oral y tienen características clínicas comunes. El objetivo de este estudio fue presentar y comparar las manifestaciones clínicas de PV y PMM y los resultados de los tratamientos aplicados. En el análisis se incluyó una serie de casos de un servicio de estomatología de 1985 a 2018. La recolección de información incluyó datos epidemiológicos, comorbilidades, medicamentos en uso, duración de los síntomas antes de la primera visita, tratamientos previos, sintomatología, descripción clínica de las lesiones, diagnóstico presuntivo, descripción histopatológica, manifestaciones extraorales, diagnóstico final, tratamiento y seguimiento. Se analizaron las historias clínicas de 25 pacientes, 19 de los cuales fueron diagnosticados de PMM y 6 de PV. El sexo feminino fue prevalente en PMM (84 %) y el sexo masculino en PV (67 %). Más del 60 % de los pacientes se quejaron de dolor durante la primera consulta. Los pacientes con PMM tardaron en promedio 6 meses en buscar ayuda profesional y los pacientes con PV, alrededor de 2 meses. La gingivitis descamativa fue la lesion más común (63 %) en PMM y las úlceras non gengivales (67 %) en PV. La terapia mínima fue efectiva en todos los casos de PMM, y en PV un individuo requirió terapia adyuvante mínima debido al empeoramiento del caso. Los pacientes con PV tienen signos y síntomas orales más intensos y pueden necesitar un tratamiento más intensivo que los pacientes con PMM. El uso de corticosteroides tópicos y/o sistémicos fue suficiente para la mayoría de los casos en ambas enfermedades.

Epidemiological and clinical study of cases of endemic pemphigus foliaceus and pemphigus vulgaris in a reference center in the state of Minas Gerais, Brazil

Abstract Background: Pemphigus constitutes a group of autoimmune bullous diseases. A reduction in the incidence of endemic pemphigus foliaceus and an increase in pemphigus vulgaris has been described, but there are no studies in Minas Gerais that address the subject. Objective: To describe the epidemiological and clinical profile of patients with pemphigus treated at the Dermatology Service of a public University Hospital in the state of Minas Gerais, Brazil. Methods: An observational, descriptive, and cross-sectional study was carried out of cases of endemic pemphigus foliaceus and pemphigus vulgaris, for a period of six months. A questionnaire was filled out with epidemiological and clinical data on the disease. Results: A total of 122 patients were included in the study, 64 with endemic pemphigus foliaceus and 58 with pemphigus vulgaris. When comparing patients with endemic pemphigus foliaceus and those with pemphigus vulgaris, a statistical difference was observed between the median age of initial disease manifestation (p = 0.001), patient occupation (p = 0.010), area of residence (p = 0.000), forests (p = 0.000) and rivers/streams close to the dwelling (p = 0.001) and the number of systemic medications required to control the disease (p = 0.002). When comparing patients with endemic pemphigus foliaceus to those evaluated in a study carried out at the same service in 2008, there was a statistical difference in the area of residence (p = 0.030). Study limitations: The assessed population comes from a tertiary care service that is not a reference for the entire state. Conclusions: Patients with endemic pemphigus foliaceus and pemphigus vulgaris maintain statistically significant differences regarding their main variables in the literature, such as age and area of residence. Historically, there has been a reduction in cases of endemic pemphigus foliaceus and an increase in cases of pemphigus vulgaris in this population.

Analysis of current status and risk factors of multidrug-resistant bacteria wound infections in patients with autoimmune bullous diseases / 中华皮肤科杂志

Objective:To investigate the current status of multidrug-resistant bacteria (MDRO) wound infections in patients with autoimmune bullous diseases (AIBDs), and to analyze their risk factors.Methods:A retrospective study was conducted, and inpatients with AIBDs accompanied by wound infections were collected from Hospital of Dermatology, Chinese Academy of Medical Sciences from January 2020 to December 2022. A descriptive analysis was carried out to analyze the basic characteristics of these patients and pathogenic characteristics of MDRO. Univariate and binary logistic regression models were used to analyze independent risk factors for MDRO infections in patients with AIBDs. Differences between the MDRO infection group and common bacterial infection group were analyzed by using t test, Mann-Whitney U test and chi-square test. Results:Totally, 271 patients with AIBDs accompanied by wound infections were included, including 159 males (58.7%) and 112 females (41.3%), and 142 patients (52.4%) were aged over 60 years. Most patients with AIBDs were diagnosed with pemphigus vulgaris (131 cases, 48.3%), or bullous pemphigoid (99 cases, 36.5%). Bacterial culture was positive in all the patients, and 74 (27.3%) were infected with MDRO; a total of 108 strains of MDRO were detected, with relatively high detection rates of Staphylococcus (82 strains, 75.9%) and Enterobacter (15 strains, 13.9%). Significant differences were observed between the MDRO infection group and the common bacterial infection group in the duration of hospitalization, involved body surface area, proportions of patients self-modificating drug dosage, proportions of patients topically using antibiotic ointments, proportions of patients using immunosuppressants, duration of glucocorticoid use, maximum dose of glucocorticoids and the first albumin level at admission (all P < 0.05), while there were no significant differences in the gender, age, proportions of patients at first hospitalization, types of AIBDs, duration of education, body mass index, disease duration, proportions of smoking patients, proportions of drinking patients, proportions of patients with comorbid chronic diseases, surgical history, prevalence of hypoalbuminemia, prevalence of mucosal involvement, proportions of patients receiving topical glucocorticoids, proportions of patients using biological agents, duration of antibiotic use, and the first total protein level at admission between the two groups (all P > 0.05). Logistic regression analysis showed that the use of topical antibiotic ointments, use of immunosuppressants, maximum dose of glucocorticoids, and self-modification of drug dosage were independent risk factors for MDRO infections (all P < 0.05) . Conclusions:The patients with AIBDs were prone to develop MDRO infections in wounds, and Staphylococcus infections were the most common. The use of topical antibiotic ointments, use of immunosuppressants, high dose of glucocorticoids, and self-modification of drug dosage may increase the risk of infections in patients with AIBDs.

A case of Psoriasis and Pemphigus Foliaceous in a 55-year-old Filipino

@#Pemphigus foliaceous is a rare autoimmune blistering disease, while psoriasis is a common immune‑mediated inflammatory skin disease. The coexistence of psoriasis and pemphigus foliaceous has rarely been reported. We report a case of a 55‑year‑old Filipino female with an 8‑year history of chronic plaque‑type psoriasis biopsy‑proven. After 5 years, she developed generalized flaccid bullae and crusted erosions over the face, trunk, and extremities, with no mucous membrane involvement. Skin punch biopsy, direct immunofluorescence, and enzyme‑linked immunosorbent assay were consistent with pemphigus foliaceous. The combination of topical corticosteroids and oral methotrexate was selected as the therapeutic approach, leading to a notable improvement in the patient’s condition. This case report underscores the significance of identifying the simultaneous presence of psoriasis alongside autoimmune blistering diseases like pemphigus foliaceous. Examining predisposing and triggering factors, performing re‑biopsy, and further work‑up as the disease evolves may yield more profound insights. Nonetheless, effectively managing this condition poses a significant challenge.

Progress in plasma exchange for severe immune-related dermatoses / 中国输血杂志

Plasma exchange (PE) is effective in a part of autoimmune diseases, and its main action mechanisms include removing pathogenic factors from the patient's blood, regulating immune function, replenishing normal plasma components. The use of PE in the treatment of severe immune-related skin diseases, such as pemphigus vulgaris, toxic epidermal necrolysis and clinically amyopathic dermatomyositis, has become more widespread. This review provides an overview of the progress of PE application in severe immune-related skin diseases at home and abroad in recent years to provide new ideas for the treatment of clinical severe skin diseases.

Manifestações bucais e diagnóstico precoce do pênfigo vulgar: relato de caso / Oral manifestations and early diagnosis of pemphigus vulgaris: a case report / Manifestaciones orales y diagnóstico precoz del pénfigo vulgar: informe de un caso

O pênfigo vulgar (PV) é uma doença autoimune que acomete o tecido epitelial dos indivíduos afetados. As manifestações orais são frequentemente os primeiros sinais observados da doença. Objetivou-se apresentar um caso clínico de pênfigo vulgar em uma paciente de 39 anos de idade, atendida em um serviço hospitalar de referência do estado de Pernambuco, Brasil. A paciente queixava-se de lesões dolorosas na cavidade bucal há aproximadamente 1 mês, de progressão rápida. Ao exame clínico intrabucal, realizou-se o teste de Nikolsky, com resultado positivo. Em seguida, realizou-se biópsia incisional, confirmando a hipótese diagnóstica clínica de PV. A paciente recebeu o tratamento multiprofissional e foi medicada para o controle da doença, com melhora significativa do quadro clínico. O diagnóstico precoce da doença pelo profissional é importante, seguida de uma abordagem multiprofissional para um correto diagnóstico da patologia, possibilitando remissão da sintomatologia e melhora da qualidade de vida dos pacientes... (AU)

Pemphigus vulgaris (PV) is an autoimmune disease that affects the epithelial tissue of affected individuals. Oral manifestations are often the first signs observed of the disease. We aimed to present a clinical case of pemphigus vulgaris in a 39-year-old patient seen at a reference hospital service in the state of Pernambuco, Brazil. The patient complained of painful lesions in the oral cavity for about 1 month, with rapid progression. Upon intraoral clinical examination, the Nikolsky test was performed, with a positive result. An incisional biopsy was then performed, confirming the clinical diagnosis of PV. The patient received multiprofessional treatment and was medicated to control the disease, with significant improvement in the clinical picture. The early diagnosis of the disease by the professional is important, followed by a multiprofessional approach for a correct diagnosis of the pathology, enabling remission of the symptoms and improving the quality of life of patients... (AU)

El pénfigo vulgar (PV) es una enfermedad autoinmune que afecta al tejido epitelial de los individuos afectados. Las manifestaciones orales suelen ser los primeros síntomas observados de la enfermedad. El objetivo es presentar un caso clínico de pénfigo vulgar en una paciente de 39 años de edad, atendida en un servicio hospitalario de referencia del estado de Pernambuco, Brasil. El paciente se quejaba de lesiones dolorosas en la cavidad oral desde hacía aproximadamente un mes, con una rápida progresión. Tras el examen clínico intraoral, se realizó la prueba de Nikolsky, con resultados positivos. Posteriormente, se realizó una biopsia incisional que confirmó el diagnóstico clínico de PV. El paciente recibió tratamiento multiprofesional y fue medicado para controlar la enfermedad, con una mejora significativa del cuadro clínico. Es importante el diagnóstico precoz de la enfermedad por parte del profesional, seguido de un abordaje multiprofesional para un correcto diagnóstico de la patología, que permita la remisión de los síntomas y la mejora de la calidad de vida de los pacientes... (AU)

Pénfigo foliáceo, variedad seborreica, reporte de caso / Pemphigus foliaceus, seborrheic variety, case report

Presentación del caso. Se expone el caso de un paciente masculino de 48 años de edad, sin antecedentes médicos conocidos, que presentó múltiples lesiones en forma de placas eritematocostrosas fácilmente descamativas, inicialmente en tórax anterior, que se esparcían sobre el rostro y cuero cabelludo sin afectar las mucosas. Intervención terapéutica. El manejo hospitalario se basó fundamentalmente en el uso de esteroides tópicos y sistémicos, así como el manejo de las infecciones sobreagregadas a las lesiones dermatológicas y el apoyo psicológico del paciente. Se tomó biopsia de piel donde se evidenció la presencia de acantólisis, confirmando el diagnóstico de esta enfermedad autoinmunitaria. Evolución clínica. Luego del tratamiento se logró una reducción de las múltiples lesiones descamativas, el control de la infección local y la recuperación de la piel del paciente, la cual a pesar de aún presentar cicatrices se encontraba con sus funciones restituidas

Case presentation. a 48-year-old male with no known medical history who presented multiple lesions in the form of easily desquamative erythematous and crusted plaques, initially on the anterior thorax, which spread over the face and scalp without affecting the mucous membranes. Treatment. In-hospital management was mainly based on topical and systemic steroids, the management of infections superadded to the dermatologic lesions, and psychological support for the patient. A skin biopsy was taken where acantholysis was evidenced, confirming the autoimmune disease diagnosis. Outcome.After treatment, the multiple scaly lesions were reduced, the local infection was controlled, and the patient's skin recovered although it still had scars, its functions were restored

Oral lesions after infection by SARS CoV 2: two case reports / Lesiones bucales posteriores a infección por SARS CoV 2 presentación de dos casos

Introduction: The SARS CoV 2 infection has resulted in several health, economic, and social crises in all areas. The disease shows a substantial biological diversity in humans causing a series of sequels in the trans- or post-infection period in the entire organism. Case Report: The manifestations that occur in the oral cavity and pharynx have not been evaluated. In this study, two clinical cases are reported. The first patient, a 67-year-old male, presents erosive lesions on the dorsal surface of his tongue after SARS CoV 2 infection. Results: Therapy consisting of reinforcing oral cleaning, use of antifungal solutions, mouthwashes containing superoxidation solution and B complex was given to the patient. The reported lesions improved satisfactorily. The second case, a 47-year-old male patient, presented vesiculobullous lesions on the lingual and labial mucosa accompanied by severe painful symptoms after SARS CoV 2 infection. An incisional biopsy was performed. The histopathological result was compatible with pemphigus vulgaris, and the treatment protocol was started with 0.1% topical mometasone and 2g miconazole gel, observing adequate involution of the lesions after 20 days. Conclusions: The aim of this study is to report on the lesions affecting the oral cavity and pharynx in post-COVID patients with the aim of carrying out a thorough intraoral examination, establishing a clinical or histopathological diagnosis to implement a specific treatment plan in each case to improve the health and quality of life of the patients. Keywords: SARS-CoV-2; Oral manifestations; Oral ulcer; Pemphigus; Mouth; Mucous membrane.

Introducción: La infección por virus de SARS CoV 2 ha dejado a su paso una estela de crisis en materia de salud, económica, social y en todos los ámbitos a la fecha seguimos realizando la observación del comportamiento de la enfermedad en los seres humanos con una diversidad biológica importante y que ha traído como consecuencia una serie de secuelas que se presentan en el periodo trans o posterior a la infección en toda la economía corporal. Reporte de Caso: Se ha evaluado poco las manifestaciones que se presentan en la cavidad bucal y faringe; se presentan dos casos clínicos el primero paciente masculino de 67 años de edad posterior a la infección por SARS CoV 2 presenta diluciones de continuidad en bordes laterales de la lengua se indica terapia y refuerza limpieza bucal, antimicótico, colutorios con solución de superoxidación y complejo B, las úlceras involucionan de manera satisfactoria. Resultados: El segundo caso masculino de 47 años posterior a la infección por SARS CoV 2 debuta con lesiones vesículo-ampollosas en mucosa lingual, labial con sintomatología dolorosa severa, se realiza biopsia incisional donde el resultado histopatológico es compatible con pénfigo vulgar, se inicia protocolo de tratamiento con mometasona tópica al 0.1% y miconazol gel 2g observándose una adecuada involución de las lesiones a los 20 días. Conclusiones: El objetivo de este trabajo es poner en contexto de la comunidad médica y científica las lesiones concernientes a la cavidad bucal y faringe que están presentando los pacientes postcovid con el objetivo de realizar una exhaustiva exploración intraoral, establecer un diagnóstico clínico o histopatológico y con base en esto instaurar un plan de tratamiento específico en cada caso en particular con el fin fundamental de mejorar la salud y calidad de vida del paciente.

Role of neutrophil-to-lymphocyte, neutrophil-to-eosinophil and platelet-to-lymphocyte ratios in the diagnosis of bullous pemphigoid and Pemphigus disease

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.

Penfigoide gestacional. Reporte de caso

El penfigoide gestacional, antes conocido como herpes gestacional, es una dermatosis rara, ampollosa, autoinmune y específica del embarazo. Aunque la etiología no se conoce del todo, la mayoría de los pacientes desarrollan anticuerpos contra una proteína hemidesmosomal transmembrana de 180 kDa. Los síntomas que definen la enfermedad son el picor intenso y las lesiones urticariales que evolucionan hacia ampollas dolorosas. Las lesiones comienzan en la región periumbilical en el 90% de los casos y se extienden rápidamente a otras zonas del cuerpo, a excepción de la cabeza y las mucosas. Es más frecuente en el segundo o tercer trimestre. En las muestras de biopsia de piel, la tinción de inmunofluorescencia directa valida el diagnóstico. Los corticoides tópicos pueden ser utilizados para tratar los síntomas leves, mientras que los corticoides orales y los antihistamínicos deben usarse para tratar los casos graves. Se presenta un caso de penfigoide gestacional.

Pemphigoid gestationis, formerly known as herpes gestationis, is a rare, blistering, autoimmune, pregnancy-specific dermatosis. Although the etiology is not fully understood, most patients develop antibodies against 180 kDa transmembrane hemidesmosomal protein. The defining symptoms of the disease are intense itching and urticarial lesions that evolve into painful blisters. Lesions begin in the periumbilical region in 90% of cases and spread rapidly to other areas of the body, except for the head and mucous membranes. It is most frequent in the second or third trimester. In skin biopsy specimens, direct immunofluorescence staining validates the diagnosis. Topical corticosteroids can be used to treat mild symptoms, while oral corticosteroids and antihistamines should be used to treat severe cases. A case of pemphigoid gestationis is presented.

Subcorneal pustular dermatosis type of IgA Pemphigus in a 35-year-old female: A case report

Introduction@#IgA pemphigus is a rare, distinct variant of Pemphigus characterized by vesiculopustular eruptions mediated by IgA autoantibodies targeting keratinocyte cell surface antigens, desmocollins 1-3 and sometimes desmogleins 1 and 3. Its classical features have been described in literature but atypical cases have also been documented. This report presents such case posing a diagnostic dilemma.@*Case Report@#A 35-year-old female presented with a 16-year history of intermittent eruptions of multiple hyperpigmented, annular and circinate, desquamating plaques and coalescing flaccid pustules on erythematous bases on the scalp, neck, trunk, and extremities. Histopathologic examination revealed subcorneal pustular dermatitis, and direct immunofluorescence was positive for granular intercellular IgG and IgA deposits in the epidermis. Antinuclear antibody test was negative and C3 level was normal. Antibody tests against desmogleins 1 and 3 were both negative. Topical potent corticosteroid therapy resulted in complete resolution of all lesions in three weeks.@*Conclusion@#Diagnostic dilemmas arise when laboratory results do not correlate with clinical findings. Findings of IgA autoantibodies in patients with pemphigus-like skin eruptions led to the diagnosis of subcorneal pustular dermatosis type of IgA pemphigus. Dapsone is the treatment of choice although topical potent corticosteroid alone may provide complete remission in some cases, avoiding the potential adverse effects of systemic therapy

The clinical characteristics of nail involvement in pemphigus patients and the correlation between nail involvement and the severity of pemphigus / 中国医师杂志

Objective:To analyze the clinical characteristics of nail involvement in patients with pemphigus and the correlation between nail damage and the severity of pemphigus.Methods:The clinical data of 23 patients with pemphigus combined with nail damage admitted to the People′s Hospital of Xinjiang Uygur Autonomous Region from January 2011 to August 2019 were retrospectively analyzed, the manifestations of pemphigus combined with nail damage were summarized. The nail damage number of patients with pemphigus complicated with nail damage of different genders were analyzed, as well as the distribution of nail damage in nail and toenail. The titer of anti-desmocoglycoprotein(dsg) antibody was detected in all patients. The relationship between nail damage and disease severity and course in pemphigus patients was analyzed.Results:A total of 132 damage nails were found in 23 patients (14 males and 9 females) with pemphigus, including 66 nails in male, 66 nails in female, 82 nails and 50 toenails. There were 10 forms of nail damage, of which chronic paronychia was the most common. The number of damage nails between different genders in pemphigus patients was statistically significant (χ 2=9.183, P<0.001). The distribution of nail and toenail damage in pemphigus patients was statistically significant (χ 2=10.880, P<0.001). Of the 23 patients, only 3 were positive for dsg1 and 1 was positive for dsg3. There were 19 patients with both positive for dsg1 and dsg3. The titers of dsg1 and dsg3 were compared in 14 patients before and after nail damage. The results showed that the titers of anti-dsg antibody in pemphigus patients after nail damage were significantly higher than before. Thirteen of the 23 patients had nail damage at the time of the initial onset of pemphigus. The nail damage occurred from 6 weeks before the onset to 4 weeks after the onset. The nail damage occurred in 10 patients when the disease recurred. The nail damage occurred within 4 weeks before or at the same time with the blister. Conclusions:The number of damage nails per capita in female patients with pemphigus and nail damage was significantly higher than that in male patients, and nail damage was more common. The titers of anti-dsg antibody will be at a high level when pemphigus patients with nail damage, and the condition gets worse. Nail involvement is positive to the severity of the disease, and it can prolong the time of disease.

Effect of different immersion bath methods on the healing of skin lesions in patients with pemphigus / 中华皮肤科杂志

Objective:To analyze effects of ozone bath, 1∶8 000 potassium permanganate bath and 1∶5 000 potassium permanganate bath on the recovery of skin lesions in patients with pemphigus.Methods:Patients with pemphigus, who received immersion bath treatment during their hospitalization, were retrospectively collected from Department of Dermatology, Peking University First Hospital from January 2016 to June 2021. The efficacy and safety of different immersion bath methods including ozone bath, 1∶8 000 potassium permanganate bath and 1∶5 000 potassium permanganate bath were compared. Categorical variables were compared using chi-square test or Fisher′s exact test, and univariate and multivariate logistic regression models were used to analyze relationships between candidate variables and patients′ condition at discharge.Results:A total of 74 patients with pemphigus were included, including 45 (60.81%) males and 29 (39.19%) females, their age ( M[ Q1, Q3]) was 52 (41, 60) years, and the median percentage of primary skin lesion area in body surface area was 40%. There were no significant differences in hospital stays, disease duration (time from onset to admission) , gender ratio or skin lesion areas among patients receiving ozone bath (32 cases) , patients receiving 1∶8 000 potassium permanganate bath (25 cases) and those receiving 1∶5 000 potassium permanganate bath (17 cases, all P > 0.05) . These patients still received other treatments, including glucocorticoids, gamma globulin, plasma transfusion, rituximab, immunosuppressants, topical antibiotics, etc., and there was no significant difference in the proportions of patients using the above therapies among the three groups (all P > 0.05) , while the ozone bath group showed a lower proportion of patients using systemic antibiotics compared with the two potassium permanganate bath groups (both P < 0.01) . At discharge, in the ozone bath group, 1 case was improved, 21 were nearly cured, and 10 were cured; in the 1∶8 000 potassium permanganate bath group, 4 cases were improved, 13 were nearly cured, and 8 were cured; in the 1∶5 000 potassium permanganate bath group, 5 were improved, 8 were nearly cured, and 4 were cured. The proportion of well-recovered patients was significantly higher in the ozone bath group (31/32 cases) than in the potassium permanganate bath group (33/42 cases, P = 0.036) . Univariate and multivariate analyses showed that ozone bath significantly affected the patients′ condition at discharge ( P < 0.05) , and was an independent predictor of "good recovery" at discharge ( HR = 8.455, 95% CI: 1.011 - 70.672, P = 0.049) . Conclusion:Ozone bath therapy can facilitate recovery of skin lesions in patients with pemphigus.

Clinical characteristics and treatment of eight cases of pemphigus/bullous pemphigoid complicated by herpes simplex virus infection / 中华皮肤科杂志

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.

Detection of ATP2C1 gene mutations in a family with generalized familial benign chronic pemphigus / 中华皮肤科杂志

A 60-year-old female proband presented with recurrent erythema, blisters and erosions all over the body for 30 years, which had been aggravated 10 days prior to the presentation. Skin examination showed erythematous swelling of the bilateral eyelids with scattered dark red crusts, scattered erythema and erosions on the nasolabial folds and chin, large areas of erythema and erosions on the neck, bilateral axillae, left cubital fossa, perineum and perianal area, accompanied by bright red granulation tissues and positive Nikolsky′s sign. The proband had two sons, both of whom occasionally presented with erythema and erosions on the axillae and groin, and had not been diagnosed or treated. Blood samples were collected from the proband and her two sons, and genomic DNA was extracted and subjected to whole-exome sequencing. A heterozygous deletion mutation c.955_957del (p.A319del) was identified in the ATP2C1 gene in the proband and her two sons, which had not been previously reported. The patient was finally diagnosed with generalized familial benign chronic pemphigus.

Epitope spreading in autoimmune bullous diseases / 中华皮肤科杂志

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.

A Case of Paraneoplastic Pemphigus with Malignant Thymoma and Obliterated Bronchiolitis / 罕见病研究

Paraneoplastic pemphigus (PNP) is a rare and life-threatening autoimmune blistering disease characterized by severe mucosal erosions and polymorphous cutaneous eruptions associated with lymphoproliferative neoplasmas. Castleman's disease, non-Hodgkin's lymphoma, thymoma, follicular dendritic cell sarcoma and chronic lymphocytic leukemia are the commonly associated neoplasmas in PNP, whereas malignant thymoma is rare. The prognosis of PNP is poor due to severe infections during immunosuppressive treatment, underlying malignancies and bronchiolitis obliterans mediated by autoimmunity. A 58-year-old woman who experienced recurrent oral erosive ulcer for 3 years, systemic erythema for 10 months, and dyspnea for 3 months was diagnosed with PNP with malignant thymoma and obliterated bronchiolitis by clinical, radiological, histopathological, and immunological examinations. She died of respiratory failure 3 weeks after resection of the malignant thymoma.

Considerations during the treatment of pemphigus vulgaris under special circumstances / 中华皮肤科杂志

Pemphigus vulgaris is the most common and serious type of pemphigus, and timely treatment can change its prognosis. This review comprehensively analyzes considerations in the treatment of pemphigus vulgaris during pregnancy and lactation as well as during the prevention and control of COVID-19 pandemic, including treatment particularities and comprehensive nursing care, in order to provide better guidance and treatment for patients.

Qualitative Study on the Emotional Experience of Family Caregivers of Inpatients with Pemphigus / 中国医学伦理学

【Objective:】 To study the emotional experience of family caregivers of patients with Pemphigus, and to provide reference for them to formulate targeted intervention and support. 【Methods:】 Using the phenomenological research method in qualitative research and purposive sampling method, in-depth semi-structured interviews were conducted with 13 family caregivers of patients with pemphigus from September 2021 to June 2022. Colaizzi analysis method was used for data analysis. 【Results:】 The emotional experience of family caregivers of inpatients with pemphigus was summarized into five themes: lack of cognition, high financial and psychological burden, more concerns about the use of hormone drugs in treatment, provide positive guidance and support for patients, negative effect on the quality of life. 【Conclusion:】 Nursing staff should pay attention to the emotional experience of family caregivers, enhance disease knowledge and medication explanation, conduct psychological counseling, and increase treatment confidence, so as to achieve the purpose of promoting the rehabilitation of patients.

Measurement of pesticides in hair samples from pemphigus foliaceus and pemphigus vulgaris patients in Southeastern Brazil

Abstract Background Pesticides, mainly organophosphates (OP), have been related to increased risk of pemphigus vulgaris (PV) and pemphigus foliaceus (PF), nevertheless, their measurement has not been determined in pemphigus patients. Objective To evaluate pesticide exposure and pesticide measurement, comparing PV, PF and control groups in Southeastern Brazil. Methods Information about urban or rural residency and exposure to pesticides at the onset of pemphigus was assessed by questionnaire interview; hair samples from the scalp of PV, PF, and controls were tested for OP and organochlorines (OC) by gas-phase chromatography coupled to mass spectrometry. Results The minority of PV (2 [7.1%] of 28) and PF (7 [18%] of 39), but none of the 48 controls, informed living in rural areas at the onset of pemphigus (p = 0.2853). PV (33.3%), PF (38.5%), and controls (20%) informed exposure to pesticides (p = 0.186). Twenty-one (14.8%) of 142 individuals tested positive for OP and/or OC: PV (2 [6.3%] of 32) and PF (11 [25.6%] of 43) had similar pesticides contamination as controls (8 [11.9%] of 67) (p = 0.4928; p = 0.0753, respectively), but PF presented higher contamination than PV (p = 0.034). PV did not present any positivity for OP. Three (7%) PF tested positive for both OP and OC. Some PF tested positive for three or four OP, mainly diazinon and dichlorvos. Study limitation Lack of data for some controls. Conclusion Although the frequency of PV and PF patients exposed to pesticides was similar, pesticides were more frequently detected in hair samples from PF compared to PV. The cause-effect relationship still needs to be determined.