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.

Quality of systematic reviews on the treatment of vesiculobullous skin diseases. A meta-epidemiological study

Abstract Background Systematic reviews of Randomized Controlled Trials (RCTs) are considered high-level evidence to support a decision on therapeutic interventions, and their methodological quality is essential to provide reliable and applicable results. Objective This meta-epidemiological study aimed to map and critically appraise systematic reviews assessing treatments for vesiculobullous skin diseases. Methods We conducted a comprehensive search strategy on MEDLINE (via Pubmed) in December 2022 without restrictions to find systematic reviews evaluating pharmacological interventions for vesiculobullous skin diseases. The methodological quality was assessed using the AMSTAR-2 tool, and additional information was extracted. We identified nine systematic reviews published between 2002 and 2021, seven assessing pemphigus. Results According to the AMSTAR-2 tool, 55.6% were classified as critically low quality, 22.2% as moderate quality, 11.1% as low and 11.1% as high quality. No review assessed the certainty of the evidence (GRADE); 86% of pemphigus reviews had at least two overlapping RCTs. There were some limitations regarding methodological flaws and the AMSTAR-2 tool use Conclusions These findings reveal a frail methodological quality of systematic reviews about vesiculobullous diseases treatment that may impact the results. Therefore, methodological rigor is mandatory for future systematic reviews to avoid duplication of effort and increase the certainty of the evidence supporting decision-making.

Autoimmune subepidermal bullous diseases characterized by annular erythema and blisters: a retrospective analysis of 25 cases / 中华皮肤科杂志

Objective:To summarize clinical, histopathological, immunoserological, and therapeutic features of patients with autoimmune subepidermal bullous diseases characterized by annular erythema and blisters.Methods:Clinical data were collected from patients with autoimmune subepidermal bullous diseases characterized by annular erythema and blisters in the Hospital of Dermatology, Chinese Academy of Medical Sciences and Peking Union Medical College from 2015 to 2022, and their clinical, pathological, immunoserological and therapeutic characteristics were retrospectively analyzed.Results:A total of 25 patients were enrolled, including 10 males and 15 females, aged 39.21 ± 24.65 years; there were 9 patients with linear IgA bullous dermatosis (LABD), 7 with bullous pemphigoid (BP), 5 with anti-P200 pemphigoid and 4 with epidermolysis bullosa acquisita (EBA), and 20 (80%) patients suffered from pruritus to different extents. Dermal eosinophil infiltration was observed in 15 (60%) patients, elevated peripheral blood eosinophil counts in 11 (44%), and both were observed in 7 (28%). Indirect immunofluorescence on salt-split skin and Western blot analysis showed that both IgG and IgA anti-basement membrane zone antibodies were present in 9 patients, including 4 with BP, 1 with LABD, 2 with anti-P200 pemphigoid, and 2 with EBA; and multiple antibodies against different basement membrane zone antigens were present in 5. All the 7 BP patients were treated with systemic glucocorticoids, including 5 (71.4%) receiving combined immunosuppressive agents, and 2 receiving combined minocycline; the patients with LABD, anti-P200 pemphigoid or EBA were sensitive to anti-inflammatory drugs and dapsone.Conclusion:Multiple types of autoimmune subepidermal bullous diseases may manifest as annular erythema and blisters, and it is necessary to make a differential diagnosis based on immunoserological tests.

Clinical features of coronavirus disease 2019 in patients with autoimmune blistering diseases / 中华皮肤科杂志

Objective:To analyze clinical features of coronavirus disease 2019 in patients with autoimmune blistering diseases (AIBDs), and to explore risk factors for the development of severe coronavirus disease 2019 in patients with AIBDs.Methods:Clinical data were collected from patients with AIBDs who were hospitalized in Zhongda Hospital, Southeast University from August 2021 to December 2022, and were retrospectively analyzed. Independent sample t-test, Mann-Whitney U test, or chi-square test (Fisher′s exact test) were used to analyze factors associated with severe coronavirus disease 2019 in AIBD patients. Results:A total of 30 patients with AIBDs accompanied by coronavirus disease 2019 were included, they were aged 62.90 ± 15.72 years, and included 16 males and 14 females; pemphigus vulgaris (12 cases, 40.00%) and bullous pemphigoid (13 cases, 43.33%) predominated in the disease spectrum of AIBDs in these patients. The common clinical manifestations of coronavirus disease 2019 were cough (25 cases, 83.33%), fatigue (22 cases, 73.33%), and fever (15 cases, 50.00%) ; most patients had mild clinical symptoms, while 7 (23.33%) developed into severe coronavirus disease 2019, including 2 out of 14 aged 30 - 59 years and 5 out of 16 elderly patients aged ≥ 60 years, and 2 out of the 5 elderly patients finally died. According to the clinical classification criteria of coronavirus disease 2019, the 30 patients were divided into the mild-to-moderate group (23 cases) and severe group (7 cases) ; the duration of AIBDs was significantly shorter in the severe group (10.71 ± 10.72 months) than in the mild-to-moderate group (27.61 ± 19.67 months, t = 2.16, P = 0.040) ; the remission rate of AIBDs was significantly higher in the mild-to-moderate group (21/23) than in the severe group (4/7, χ2 = 4.36, P = 0.037) ; the average daily dosage of methylprednisolone during the last month was significantly lower in the mild-to-moderate group ( M[ Q1, Q3]: 8 [4, 14] mg) than in the severe group (24 [12, 47] mg, U = 133.50, P = 0.007), and the proportion of patients treated with methylprednisolone at an average dosage of > 8 mg was significantly higher in the severe group (6/7) than in the mild-to-moderate group (8/23, χ2 = 5.59, P = 0.031) . Conclusions:Among the patients with AIBDs accompanied by coronavirus disease 2019, the incidence and mortality of severe coronavirus disease 2019 were higher in elderly patients than in young patients. The short course and no-remission of AIBDs, and the average daily dosage of methylprednisolone being>8 mg/d during the last month were risk factors for the development of severe coronavirus disease 2019 in the patients with AIBDs.

Is transition between subtypes of pemphigus possible? A series of pemphigus vulgaris patients showing the transition to pemphigus foliaceus

Abstract Background Pemphigus vulgaris (PV) and pemphigus foliaceus (PF) are subtypes of pemphigus with distinct clinical and laboratory features. The transition between these two subtypes has rarely been reported previously. Methods The data of PV patients who exhibited clinical and immunoserological transition to PF during the follow-up period were retrospectively evaluated regarding their demographical, clinical, and laboratory characteristics. Results Among 453 patients diagnosed with PV, 13 (2.9%) patients exhibited clinical and immunoserological transition from PV to PF. The mean age of PV patients at the time of diagnosis was 39.8 ± 14.7 (19‒62) years and 7 (53.8%) of them were female. These patients showed clinical and immunoserological transition from PV to PF after a period ranging from 4 months to 13 years (mean 36.2 ± 41 months). In addition to typical clinical features of PF, all patients had positive anti-desmoglein-1 and negative anti-desmoglein-3 antibody levels after the clinical transition had occurred without any mucosal involvement. During a mean 7.8 ± 5.8 (2‒21) years of follow-up period after the transition from PV to PF, only one female patient had experienced a re-transition to PV characterized by a relapse of disease involving mucosal surfaces with positive anti-desmoglein-3 antibody levels following a 5-year period of remission period without treatment. Study limitations Single-center study with a retrospective study design. Conclusion Our series is the largest group of patients reported to show the transition from PV to PF to date with a long follow-up period. The reason behind the disappearance of anti-desmoglein-3 antibodies and the pathogenesis of this phenomenon is not yet elucidated.

Histopathological finding of vesiculobullous lesions of skin in relation to their clinical presentation: Prospective study from a tertiary care center

Background: Vesiculobullous disorders (VBDs) are extant with diverse clinical manifestations. Vesicles and bullae are fluid-filled cavities present within or beneath the epidermis. They are autoimmune blistering disorders in which autoantibodies are directed against target antigens present in the epidermis and dermo-epidermal junction. Objective: Evaluation of the various clinicodemographic profile of patients with a pattern of distribution (subtypes) of VBDs of the skin and assess the association between clinical aspects and histological changes in vesiculobullous lesions of the skin. Materials and Methods: The study material constituted 93 cases of VBDs out of 936 skin biopsies reported over two and a half years (January 2016 to June 2018) from the tertiary care center. A detailed history of the patients was taken, and a complete physical and dermatological examination with findings including clinical diagnosis was recorded. Histopathological examination (incisional/excisional/punch biopsy) was done in each case. The clinico-demographic evaluation was done and the results were correlated with histopathological findings. Results: Vesiculobullous lesions constituted 10.06% of all skin biopsies. The majority of cases were of pemphigus vulgaris (PV) 30 (32.25%) followed by 16 (17.2%) of bullous pemphigoid. In 83 cases (89.24%) histopathology findings were consistent with clinical diagnosis. Out of 34 cases that were diagnosed clinically as PV , the histopathological study proved 30 cases (88.23%) as PV. Conclusion: Vesiculobullous lesions of the skin are a heterogeneous group of disorders. It is essential to differentiate each pattern of subtype based on clinical examination and histopathological findings. Histopathological diagnosis with clinical correlation plays a major role in arriving at the diagnosis.

Epidermolysis bullosa acquisita

Abstract Epidermolysis bullosa acquisita is a rare autoimmune disease, characterized by the synthesis of anti-collagen VII autoantibodies, the main component of hemidesmosome anchoring fibrils. The antigen-antibody binding elicits a complex inflammatory response, which culminates in the loss of dermo-epidermal adhesion of the skin and/or mucous membranes. Skin fragility with bullae, erosions, and milia in areas of trauma characterizes the mechanobullous form of the disease. In the inflammatory form of epidermolysis bullosa acquisita, urticarial inflammatory plaques with tense bullae, similar to bullous pemphigoid, or mucosal lesions can determine permanent scars and loss of functionality in the ocular, oral, esophageal, and urogenital regions. Due to the similarity of the clinical findings of epidermolysis bullosa acquisita with other diseases of the pemphigoid group and with porphyria cutanea tarda, the diagnosis is currently confirmed mainly based on the clinical correlation with histopathological findings (pauci-inflammatory subepidermal cleavage or with a neutrophilic infiltrate) and the demonstration of the presence of anti-collagen VII IgG in situ by direct immunofluorescence, or circulating anti-collagen VII IgG through indirect immunofluorescence and/or ELISA. There is no specific therapy for epidermolysis bullosa acquisita and the response to treatment is variable, usually with complete remission in children and a worse prognosis in adults with mucosal involvement. Systemic corticosteroids and immunomodulators (colchicine and dapsone) are alternatives for the treatment of mild forms of the disease, while severe forms require the use of corticosteroid therapy associated with immunosuppressants, intravenous immunoglobulin, and rituximab.

Desquamative Gingivitis as the First Clinical Sign of Pemphigus Vulgaris: A Case Report

ABSTRACT@#Desquamative gingivitis is characterised by desquamation of the gingiva with painful erosion and ulceration. It is predominantly a manifestation of several vesiculobullous diseases. Delayed diagnosis or misdiagnosis often led to disease progression. Pemphigus vulgaris is a chronic, life-threatening autoimmune disease resulting in blistering of the mucosa and skin. Oral lesions normally preceded skin lesions. Early diagnosis and treatment are important to prevent involvement of the skin, as the treatment and prognosis varies with extraoral involvement. Clinical, histopathological examination and direct immunofluorescent are necessary for the diagnosis of pemphigus vulgaris. Treatment of desquamative gingivitis involves improving oral hygiene, reduce irritation to the lesions and specific therapy to the underlying disease. This paper describes a case of a patient with desquamative gingivitis for one year, whom is ultimately diagnosed as having pemphigus vulgaris.

Correlation between autoimmune bullous diseases and psoriasis / 中华皮肤科杂志

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.

Linear IgA bullous dermatosis / 中华皮肤科杂志

Linear IgA bullous dermatosis (LABD) is a rare autoimmune subepidermal blistering disease. Currently, researches on LABD are still limited, and most are case reports. This review summarizes research advance in etiology and pathogenesis, clinical and histopathological manifestations, diagnosis and treatment of LABD.

Autoimmune bullous diseases in pregnancy: clinical and epidemiological characteristics and therapeutic approach

Abstract Autoimmune bullous dermatoses are a heterogeneous group of diseases with autoantibodies against structural skin proteins. Although the occurrence of autoimmune bullous dermatoses during pregnancy is low, this topic deserves attention, since the immunological and hormonal alterations that occur during this period can produce alterations during the expected course of these dermatoses. The authors review the several aspects of autoimmune bullous dermatoses that affect pregnant women, including the therapeutic approach during pregnancy and breastfeeding. Gestational pemphigoid, a pregnancy-specific bullous disease, was not studied in this review.

Drug-induced chronic bullous disease of childhood in a two-year-old Filipino male triggered by cefaclor or cefuroxime: A case report

Introduction@#Chronic bullous disease of childhood (CBDC) is a rare immune-mediated subepidermal vesiculobullous eruption, characterized by linear IgA deposition along the basement membrane zone of the skin. Although mostly idiopathic, CBDC may be triggered by factors such as infection, and drugs. Clinical and immunohistopathological features of drug-induced cases are heterogeneous and indistinguishable from the idiopathic form.@*Case report@#A two-year-old Filipino male presented with pruritic vesicles and bullae on the back several days after finishing a course of cefuroxime, and cefaclor. Examination revealed multiple tense vesicles and bullae, some coalescing into a rosette pattern with central crusts on the perioral, scalp, neck, back, perineal, and perianal areas. Histopathology showed a subepidermal split with neutrophilic and eosinophilic infiltrates. Direct immunofluorescence revealed strong linear deposition of IgA, and granular deposits of C3 and IgM at the basement membrane zone, thus confirming the di- agnosis of CBDC. Dapsone at 2mg/kg/day was started, with oral prednisolone (1.3mg/kg/day), and cloxacillin syrup (40mg/kg/day). Topical care with betamethasone dipropionate and mupirocin ointment was included. After eight weeks, patient showed significant im- provement with few vesicles and resolved lesions healing with post-inflammatory hyperpigmentation.@*Conclusion@#We report a case of a two-year-old male presenting with vesiculobullous lesions after a course of cefuroxime, and cefaclor. As both were given and withdrawn in a period of close proximity, it is difficult to determine the probable culprit drug. Spontaneous resolution upon withdrawal of the suspected drug is variable. Systemic therapy such as dapsone may be necessary for treatment.

Clinical analysis of 1 057 patients with critical illnesses in a dermatological ward / 中华皮肤科杂志

Objective:To summarize clinical characteristics of and treatment experience with patients with critical illnesses in a dermatological ward.Methods:All patients with serious or life-threatening conditions, who were hospitalized at the dermatological ward of the Second Xiangya Hospital of Central South University from July 9, 2011 to December 31, 2020, were collected, and their clinical data were retrospectively analyzed. Demographic characteristics, disease types and proportions, main complications, causes of serious or life-threatening conditions, important treatment measures and outcomes were summarized, and causes of death were also analyzed and discussed.Results:A total of 1 057 patients with critical illnesses were collected, with a male-to-female ratio of 1∶1.11, and 64.81% of them aged 18 to 65 years. The types of diseases mainly included drug eruptions (332 cases) , connective tissue diseases (226 cases) , bullous skin diseases (104 cases) , psoriasis (57 cases) , erythroderma (45 cases) , infectious skin diseases (67 cases) , etc. Among them, psoriasis (39 cases) and erythroderma (32 cases) mostly occurred in males, and connective tissue diseases (168 cases) mostly occurred in females. Common complications mainly involved infections, important organ damage or dysfunction, hypoalbuminemia, and fluid, electrolyte and acid-base imbalances. A total of 94 patients were diagnosed with life-threatening conditions, which were found to be mainly caused by primary skin diseases, hematologic abnormalities, respiratory failure, nervous system abnormalities, renal failure, sepsis, fluid, electrolyte and acid-base imbalances, etc. During the management of critical illnesses, 43 patients were treated with high-dose glucocorticoid pulse therapy, 264 were treated with gamma-globulin pulse therapy, 355 were transfused with other blood products, and 34 received special therapies such as hemoperfusion/immunoadsorption therapy, plasma exchange, dialysis, artificial liver support therapy; 42 patients were transferred to the intensive care unit (ICU) , 12 were transferred to the department of surgery for operations, and 12 were transferred to the department of obstetrics and gynecology for delivery or induction of labor. After treatment, 989 patients (93.57%) achieved improvement and were discharged. A total of 14 patients (1.32%) died, of whom 7 died of secondary sepsis, 2 died of severe pulmonary infections, 2 died of asphyxia caused by respiratory mucosa shedding-induced airway obstruction, the other 3 died of gastrointestinal hemorrhage, cerebral hemorrhage and neuropsychiatric systemic lupus erythematosus, respectively.Conclusions:Critical cases in the dermatological ward mainly suffered from serious skin diseases such as severe drug eruptions, connective tissue diseases and bullous skin diseases, as well as complications such as severe underlying diseases, severe organ dysfunction, sepsis or severe fluid, electrolyte and acid-base imbalances. In terms of treatment, it is of critical significance to make a clear diagnosis and assess the severity of disease as early as possible, monitor and prevent possible complications, and to consult with specialists in relevant disciplines in time.

Caso clínico: Herpes Zoster diseminado en paciente adulta mayor inmunocompetente / Case Report: Disseminated herpes Zoster, in an immunocompetent elderly patient

INTRODUCCIÓN: El Herpes Zoster cutáneo diseminado (HZCD) se define como la pre-sencia de más de 20 vesículas por fuera del área y adyacencias del dermatoma prima-riamente comprometido, afectación de más de 2 dermatomas contiguos o que haya compromiso sistémico. Es una patología con una prevalencia entre 10 y 40% entre pacientes inmunocomprometidos, pero en pacientes inmunocompetentes se presen-tan caso aislados. Se presenta un caso de una paciente adulta mayor, sin antecedentes patológicos de importancia, inmunocompetente que desarrolló HZCD. CASO CLÍNICO: Paciente femenina de 89 años, sin antecedentes patológicos, consultó por lesiones dolorosas, pruriginosas y ampollosas en la cara interna del muslo derecho y el tercio proximal de la pierna ipsilateral de 10 días de evolución. Días después del inicio del cuadro las lesiones se diseminan y la paciente recibe tratamiento tópico que no especificó; 72 horas después empeora su estado general por lo que es traslada al Servicio de Emergencia. EVOLUCIÓN: La paciente fue diagnosticada de Herpes Zoster diseminado. Presentó evolución favorable, tras la administración del tratamiento antiviral intravenoso y fue dada de alta sin complicaciones, al día 10 de hospitalización. CONCLUSIÓN: El diagnóstico de herpes zoster diseminado debe ser considerado tanto en pacientes inmunocompetentes como inmunodeprimidos. La evolución depende en gran medida de la rapidez con la que se instale el tratamiento específico.(au)

BACKGROUND: Disseminated cutaneous Herpes Zoster is defined as the presence of more than 20 vesicles outside the area and adjacencies of the primarily affected dermatome, the involvement of more than 2 contiguous dermatomes or the presence of systemic disease. Its prevalence among immunodepressed patients is 10 - 40%, but between immunocompetent patients only isolated cases are reported. We present a case of an elderly immunocompetent patient, with no pathological history, that developed HZCD. CASE REPORT: An 89 year old woman, with no medical background, presented with 10 day evolution itchy, painful blistering lesions on the inner right thigh and the proximal third of the ipsilateral leg. Days after the lesions disseminated and the patient received topical treatment, she couldn't specify the treatment; after 3 days her general condition worsened, and she was brought to the Emergency Room. EVOlUTION: Patient was diagnosed with Disseminated Herpes Zoster. She had a good outcome, after completing the intravenous antiviral treatment and she was discharged with no complications, after 10 days of being hospitalized. CONClUSION: Diagnosis of disseminated herpes zoster should be considered in both immunocompetent and immunocompromised patients. The outcome strongly depends on the promptness of the specific therapy instauration.(au)

Familial "benign" pemphigus? Erythroderma and fatal outcome

Abstract Hailey-Hailey disease, or familial benign pemphigus, is a rare bullous genodermatosis that usually presents with flaccid blisters, erosions, and maceration limited to flexural areas, resulting in increased morbidity and reduced quality of life for affected patients. The authors report an unusual case of generalized Hailey-Hailey disease with erythroderma and fatal outcome.

Diagnostic approach of eosinophilic spongiosis

Abstract Eosinophilic spongiosis is a histological feature shared by some distinct inflammatory disorders, and is characterized by the presence of intraepidermal eosinophils associated with spongiosis. Most often, isolated eosinophilic spongiosis indicates the early stages of a subjacent autoimmune bullous dermatosis, such as the pemphigus group and bullous pemphigoid. Herein, the main causes of eosinophilic spongiosis are discussed, as well as the supplementary investigation needed to elucidate its etiology.

Vesiculobullous exanthema in a 3-month-old child with probable acute chikungunya infection / Exantema vesiculobolhoso em criança de 3 meses com provável infecção aguda por chikungunya

Objective: to report a rare case of a 3-month-old child with acute chikungunya infection presenting as vesiculobullous rash. Case description: herein we report a case of a 3-month-old infant which developed a diffuse erythematous rash involving the trunk and limbs within two days after sudden acute fever, and also presented striking irritability with inconsolable crying, hyporexia, cough, and coryza. Hematological and biochemical investigations were undertaken and were found to be normal. Serum samples were tested for Chikungunya revealing positive IgM. He was treated with supportive therapy, mainly with antipyretics, venous hydration and rest evolving with entirely resolution of the symptoms after four days. Comments: Chikungunya fever is a disease caused by the Chikungunya virus which can be found in endemic areas such as Brazil. Atypical manifestations can affect various systems including the skin and may be more frequent in children. The role of dermatological manifestations related to Chikungunya infection is not completely understood. The reported case is an unusual case of Chikungunya infection which highlights the importance of to know and to be aware of this manifestation in children mainly in new endemic areas such as Brazil.

Objetivo: relatar o caso raro de uma criança de 3 meses de idade com infecção aguda por chikungunya apresentando-se como erupção vesiculobolhosa. Descrição do caso: aqui relatamos o caso de uma criança de 3 meses que evoluiu com erupção eritematosa difusa envolvendo tronco e membros dois dias após febre aguda repentina, além de apresentar irritabilidade marcante com choro inconsolável, hiporexia, tosse e coriza. Investigações hematológicas e bioquímicas foram realizadas e foram normais. Amostras de soro foram testadas para Chikungunya revelando IgM positiva. Ele foi tratado com terapia de suporte, principalmente com antipiréticos, hidratação venosa e descanso evoluindo com resolução completa dos sintomas após quatro dias. Comentários: a febre Chikungunya é uma doença causada pelo vírus Chikungunya, que pode ser encontrada em áreas endêmicas como o Brasil. Manifestações atípicas podem afetar vários sistemas, incluindo a pele, e podem ser mais frequentes em crianças. O papel das manifestações dermatológicas relacionadas à infecção por Chikungunya não é completamente compreendido. O caso relatado é um caso incomum de infecção por Chikungunya, que destaca a importância de conhecer e ter consciência dessa manifestação em crianças, principalmente em novas áreas endêmicas como o Brasil.

Paraneoplastic pemphigus: a clinical, laboratorial, and therapeutic overview

Abstract: Paraneoplastic pemphigus is a rare and severe autoimmune blistering disease characterized by mucocutaneous lesions associated with benign and malignant neoplasms. Diagnostic criteria include the presence of chronic mucositis and polymorphic cutaneous lesions with occult or confirmed neoplasia; histopathological analysis exhibiting intraepidermal acantholysis, necrotic keratinocytes, and vacuolar interface dermatitis; direct immunofluorescence with intercellular deposits (IgG and C3) and at the basement membrane zone (IgG); indirect immunofluorescence with intercellular deposition of IgG (substrates: monkey esophagus and simple, columnar, and transitional epithelium); and, autoreactivity to desmogleins 1 and 3, desmocollins 1, 2, and 3, desmoplakins I and II, envoplakin, periplakin, epiplakin, plectin, BP230, and α-2-macroglobulin-like protein 1. Neoplasias frequently related to paraneoplastic pemphigus include chronic lymphocytic leukemia, non-Hodgkin lymphoma, carcinomas, Castleman disease, thymoma, and others. Currently, there is no standardized treatment for paraneoplastic pemphigus. Systemic corticosteroids, azathioprine, mycophenolate mofetil, cyclosporine, rituximab, cyclophosphamide, plasmapheresis, and intravenous immunoglobulin have been used, with variable outcomes. Reported survival rates in 1, 2, and 5 years are 49%, 41%, and 38%, respectively.

Assessment of the quality of life of Egyptian and Tunisian autoimmune bullous diseases' patients using an Arabic version of the autoimmune bullous disease quality of life and the treatment of autoimmune bullous disease quality of life questionnaires

Abstract: Background: The Autoimmune Bullous Disease Quality of Life (ABQOL) and the Treatment of Autoimmune Bullous Disease Quality of Life (TABQOL) questionnaires proved to be reliable tools that measure the disease and treatment burden. Objectives: We aimed to assess the ABQOL and TABQOL in the Arabic population. Methods: The English questionnaires were translated into the Arabic language by a certified translation agency. Eighty autoimmune bullous disease (AIBD) patients were included in this study. Patients were asked to answer 2 questionnaires. After 1 week the same patients were asked to answer the same questionnaires again. Results: The age of the patients ranged from 19 to 81 years (mean=46), 19 males, 61 females. The ABQOL ranged from 0-37 (mean=16.4±9.2). The TABQOL ranged from 2-43 (mean=21.5±9.4). Test-retest reliability was acceptable, Cronbach's alpha was 0.76 for ABQOL and 0.74 for TABQOL. There was no significant correlation between the age of the patients and ABQOL, r =-0.2, p value was 0.183. There was a significant negative correlation between the age of the patients and the TABQOL, r=-0.2, p value was 0.039. There was a significant negative correlation between the education of the patients and the TABQOL, r=-0.3, p value was 0.007. Study limitations: Small sample size of some AIBDs and patients with severe disease. Conclusion: Objective and valuable measurements such as ABQOL and TABQOL are now available to help physicians understand their patient's distress and should be used in every patient with AIBD. Younger and less educated patients appear to have more effects on their QOL from the treatments.