RESUMO
A neonate presented to a tertiary clinic with blisters and erosions over his trunk and extremities. The mother had multiple erosions with crusts affecting the scalp, oral cavity and trunk present since the first trimester and worse since delivery. Skin biopsy for histopathology and direct immunofluorescence confirmed pemphigus vulgaris (PV) in the mother. Indirect immunofluorescence assays for serum antibodies against desmogleins 1 and 3 were positive in both mother and baby confirming a diagnosis of neonatal PV. The baby's lesions healed spontaneously within 2 weeks, and the mother was clear at 2 months following treatment with rituximab.
Assuntos
Pênfigo , Rituximab , Humanos , Pênfigo/diagnóstico , Pênfigo/tratamento farmacológico , Pênfigo/patologia , Recém-Nascido , Feminino , Rituximab/uso terapêutico , Masculino , Gravidez , Fatores Imunológicos/uso terapêutico , Adulto , Complicações na Gravidez/tratamento farmacológico , Complicações na Gravidez/diagnósticoRESUMO
Bruton's tyrosine kinase (BTK), a nonreceptor tyrosine kinase, plays a remarkable role in the transmission and amplification of extracellular signals to intracellular signaling pathways. Various types of cells use the BTK pathway to communicate, including hematopoietic cells particularly B cells and T cells. The BTK pathway plays a role in controlling the proliferation, survival, and functions of B cells as well as other myeloid cells. First, second, and third-generation BTK inhibitors are currently being evaluated for the treatment of immune-mediated diseases in addition to B cell malignancies. In this article, the available evidence on the action mechanisms of BTK inhibitors is reviewed. Then, the most recent data obtained from preclinical studies and ongoing clinical trials for the treatment of autoimmune diseases, such as pemphigus vulgaris, pemphigus foliaceus, bullous pemphigoid, systemic lupus erythematosus, Sjögren's disease, rheumatoid arthritis, systemic sclerosis, multiple sclerosis, myasthenia gravis, and inflammatory diseases such as psoriasis, chronic spontaneous urticaria, atopic dermatitis, and asthma are discussed. In addition, adverse effects and complications associated with BTK inhibitors as well as factors predisposing patients to BTK inhibitors complications are discussed.
Assuntos
Tirosina Quinase da Agamaglobulinemia , Doenças Autoimunes , Pênfigo , Inibidores de Proteínas Quinases , Transdução de Sinais , Humanos , Tirosina Quinase da Agamaglobulinemia/antagonistas & inibidores , Doenças Autoimunes/tratamento farmacológico , Inibidores de Proteínas Quinases/uso terapêutico , Pênfigo/tratamento farmacológico , Pênfigo/imunologia , Pirimidinas/uso terapêutico , Piperidinas/uso terapêutico , Artrite Reumatoide/tratamento farmacológico , Artrite Reumatoide/imunologia , Miastenia Gravis/tratamento farmacológico , Esclerose Múltipla/tratamento farmacológico , Pirazóis/uso terapêutico , Pirazóis/farmacologia , Nitrilas/uso terapêutico , Lúpus Eritematoso Sistêmico/tratamento farmacológico , Lúpus Eritematoso Sistêmico/imunologia , Adenina/análogos & derivados , Adenina/uso terapêutico , Asma/tratamento farmacológico , Linfócitos B/imunologia , Síndrome de Sjogren/tratamento farmacológico , Psoríase/tratamento farmacológico , Psoríase/imunologia , Escleroderma Sistêmico/tratamento farmacológico , Proteínas Tirosina Quinases/antagonistas & inibidores , Benzamidas , Imidazóis , PirazinasRESUMO
Pemphigus foliaceus (PF) is a superficial form of pemphigus. Treatment options for PF resemble pemphigus vulgaris, including glucocorticosteroids, immunosuppressive agents and rituximab et al. These treatment approaches can effectively improve the condition but may also be accompanied by high risks of side effects. Therefore, it is crucial to find a safe and effective treatment options for patients with PF. It will not only benefit/be necessary for patients who refuse glucocorticosteroids or immunosuppressive agents treatments, but also for patients who cannot be treated with glucocorticosteroids or immunosuppressive agents. Herein, we reported a case of PF that was treated with apremilast without systemic glucocorticosteroids or immunosuppressive agents. A 54-year-old woman presented with itchy erythema and erosions on the trunk for more than 1 month. The patient applied mometasonefuroate cream without improvement for a duration of two weeks. The past history of diabetes mellitus and atrophic gastritis was reported. Physical examination revealed scattered erythematous macules and erosions on the trunk. No mucosal involvement was observed. The condition was assessed by the pemphigus disease area index and numerical rating scale, with baseline scores of 7 and 8, respectively. Histopathological examination showed acantholysis and intraepithelial blister. Direct immunofluorescence revealed the presence of IgG and Complement 3 deposition between the acanthocytes with the reticular distribution. Based on enzyme-linked immunosorbent assay results, the levels of Dsg1 and Dsg3 antibodies were 28.18 and 0.26 kU/L respectively. The diagnosis of PF was made. This patient was successfully treated with apremilast without systemic glucocorticosteroids or immunosuppressive agents. The patient has continued with apremilast 30mg once daily for maintenance and no adverse events related to apremilast such as gastrointestinal side effects were observed during the 9-month follow-up period. In conclusion, apremilast therapy without systemic glucocorticosteroids nor immunosuppressive agents might provide an effective alternative to management of mild PF without obvious side effect.
Assuntos
Pênfigo , Talidomida , Humanos , Pênfigo/tratamento farmacológico , Feminino , Pessoa de Meia-Idade , Talidomida/análogos & derivados , Talidomida/uso terapêutico , Imunossupressores/uso terapêutico , Resultado do Tratamento , Glucocorticoides/uso terapêutico , Anti-Inflamatórios não Esteroides/uso terapêuticoRESUMO
BACKGROUND: Pemphigus vulgaris (PV) is a potentially life-threatening mucocutaneous autoimmune disease that affects desmoglein-1 and desmoglein-3, leading to intraepithelial vesiculobullous lesions. In the oral mucosa, PV lesions can mimic other diseases such as mucous membrane pemphigoid, other forms of pemphigus, recurrent aphthous stomatitis, erythema multiforme, Stevens-Johnson syndrome, and virus-induced ulcers like herpes simplex virus (HSV), making diagnosis challenging. The co-occurrence of PV with Crohn's disease is rare and predominantly seen in younger patients. The therapeutic mainstay for both PV and Crohn's disease usually involves systemic corticosteroids combined with immunosuppressants and immunobiological drugs. Literature indicates that the use of these drugs, particularly TNF-alpha inhibitors, for managing autoimmune diseases like Crohn's can potentially induce other autoimmune diseases known as autoimmune-like syndromes, which include episodes of lupus-like syndrome and inflammatory neuropathies. There are few cases in the literature reporting the development of PV in individuals with CD undergoing infliximab therapy. CASE REPORT: A young female with severe Crohn's disease, treated with the TNF-alpha inhibitor infliximab, developed friable pseudomembranous oral ulcerations. Histopathological and immunofluorescence analyses confirmed these as PV. The treatment included clobetasol propionate and low-level photobiomodulation, which resulted in partial improvement. The patient later experienced severe intestinal bleeding, requiring intravenous hydrocortisone therapy, which improved both her systemic condition and oral lesions. Weeks later, new ulcerations caused by herpes virus and candidiasis were identified, leading to treatment with oral acyclovir, a 21-day regimen of oral nystatin rinse, and photodynamic therapy, ultimately healing the oral infections. To manage her condition, the gastroenterologists included methotrexate (25 mg) in her regimen to reduce the immunogenicity of infliximab and minimize corticosteroid use, as the patient was in remission for Crohn's disease, and the oral PV lesions were under control. CONCLUSION: Young patients with Crohn's disease should be referred to an oral medicine specialist for comorbidity investigation, as oral PV and opportunistic infections can arise during immunosuppressive therapy. The use of TNF-alpha inhibitors in patients treated for inflammatory bowel disease, such as Crohn's, should be carefully evaluated for potential side effects, including oral PV.
Assuntos
Doença de Crohn , Herpes Simples , Fatores Imunológicos , Infliximab , Pênfigo , Humanos , Pênfigo/tratamento farmacológico , Pênfigo/complicações , Doença de Crohn/complicações , Doença de Crohn/tratamento farmacológico , Feminino , Herpes Simples/complicações , Herpes Simples/tratamento farmacológico , Fatores Imunológicos/efeitos adversos , Fatores Imunológicos/uso terapêutico , Infliximab/uso terapêutico , Infliximab/efeitos adversos , Adulto , Fator de Necrose Tumoral alfa/antagonistas & inibidores , Doenças da Boca/tratamento farmacológico , Doenças da Boca/complicaçõesRESUMO
We present two middle-aged patients with pruritic, crusted scalp erosions. Skin biopsy showed epidermal acantholysis with IgG and C3 intercellular deposits on direct immunofluorescence, leading to the diagnosis of localized pemphigus vulgaris. Resolution of the lesions without relapse occurred after low doses of oral prednisone and intralesional triamcinolone acetonide.
Assuntos
Pênfigo , Dermatoses do Couro Cabeludo , Humanos , Pênfigo/patologia , Pênfigo/diagnóstico , Pênfigo/tratamento farmacológico , Dermatoses do Couro Cabeludo/patologia , Dermatoses do Couro Cabeludo/tratamento farmacológico , Dermatoses do Couro Cabeludo/diagnóstico , Pessoa de Meia-Idade , Masculino , Triancinolona Acetonida/uso terapêutico , Triancinolona Acetonida/administração & dosagem , Feminino , Prednisona/uso terapêutico , Glucocorticoides/uso terapêutico , Couro Cabeludo/patologia , Acantólise/patologia , Acantólise/diagnósticoRESUMO
Introduction: Although the treatment for pemphigus vulgaris (PV) has been revolutionized by the use of rituximab combined with corticosteroids, new effective therapies with a better safety profile are needed. Observation: A 67-year-old woman was diagnosed with severe mucosal PV, which was initially misdiagnosed as atypical Behçet's disease. Following an unsuccessful colchicine treatment, significant improvement was observed upon the introduction of apremilast: reduced pain, fewer lesions, and a stabilized weight. The discontinuation of apremilast led to a rapid relapse. Retrospective analysis through anti-Dsg3 ELISA indicated a gradual decrease in antibody levels during the apremilast treatment. Discussion: Apremilast, a phosphodiesterase 4 inhibitor approved for psoriasis and Behçet's disease's related oral ulcers treatment, demonstrated its efficacy in this PV case. This is the second case report highlighting the effectiveness of apremilast for PV treatment. Apremilast's ability to upregulate cyclic adenosine monophosphate (cAMP) levels appears to contribute to the stabilization of keratinocyte adhesion. Conclusion: Apremilast may be a promising therapeutic option for the treatment of pemphigus, with an innovative mechanism of action, no induced immunosuppression, and good tolerance. It could be a good alternative to steroids, in the treatment regimen of steroids combined with rituximab.
Assuntos
Pênfigo , Talidomida , Humanos , Pênfigo/tratamento farmacológico , Pênfigo/diagnóstico , Feminino , Talidomida/análogos & derivados , Talidomida/uso terapêutico , Idoso , Resultado do Tratamento , Anti-Inflamatórios não Esteroides/uso terapêutico , Inibidores da Fosfodiesterase 4/uso terapêuticoRESUMO
BACKGROUND: Pemphigus vulgaris (PV) is characterized by autoantibodies targeting keratinocyte adhesion proteins desmoglein (Dsg) 1 and 3, and by the human leukocyte antigen (HLA) predisposition allele HLA-DRB1*0402. Treatment using rituximab (RTX) combined with short-term corticosteroids (CS) allows disease control and long-lasting remission. OBJECTIVES: The principal aim of this study was to evaluate the impact of RTX on the circulating subpopulations of Dsg3-specific T lymphocytes that specifically regulate B-cell responses: follicular helper T (Tfh) and follicular regulatory T (Tfr) lymphocytes. METHODS: Using the HLA-DRB1*0402 tetramer loaded with the Dsg3 immunodominant peptide, we used flow cytometry to analyse the frequency, polarization and activation status of blood Dsg3-specific follicular T-cell populations at baseline, month (M) 6 and long-term follow-up (M60-90) from patients with PV. RESULTS: At baseline, we observed a predominance of Tfh1* and Tfh17 subsets and an underrepresentation of the Tfh2 subset among autoreactive Dsg3-specific Tfh cells compared with nonautoreactive Tfh cells. RTX treatment induced a decrease of autoreactive Tfh cells with no effect on their polarization during follow-up. In parallel, we observed the emergence of a Dsg3-specific Tfr subpopulation with a significant overexpression of the surface activation markers PD1, ICOS and CD25 that was not observed at the surface of autoreactive Tfh and nonautoreactive Tfr cells of the same patients with PV. In contrast, very few Dsg3-specific Tfr cells were observed in patients with PV who were treated with CS alone. CONCLUSIONS: Here we show that the emergence of circulating autoreactive Dsg3-specific Tfr cells is associated with the long-term efficacy of RTX in patients with PV.
Pemphigus vulgaris (PV) is an autoimmune disease mediated by pathogenic antibodies directed against the body's own tissues (called autoantibodies). In this condition, autoantibodies bind to and inhibit a protein called desmoglein 3 (Dsg3), which is responsible for the adhesion of keratinocytes (cells that make up the superficial layers of the skin and mucous membranes). As a result, PV causes painful blistering. People with PV are commonly treated with low doses of oral corticosteroids (prednisone) and rituximab (administered via infusions), which have significantly improved patient outcomes. Rituximab temporarily (for 69 months) depletes B lymphocytes, which are immune cells that differentiate into cells producing anti-Dsg3 antibodies. While autoimmune diseases can be lifelong, this study looked at why some people achieve long-lasting remissions without ongoing treatment, even after the return of B lymphocytes. We analysed anti-Dsg3 follicular T cells, which regulate B-cell differentiation. Among these, we observed the emergence of regulatory (or inhibiting) T cells over time following treatment, accompanied by a decrease in conventional (or activating) follicular T cells. We also found that these autoimmune follicular regulatory T cells were more activated compared with conventional follicular T cells or other T cells not directed against the Dsg3 protein. Overall, our findings suggest that the emergence of these follicular regulatory T cells may be responsible for the sustained remission observed in some people with PV and could be promoted by the use of rituximab.
Assuntos
Autoanticorpos , Desmogleína 3 , Pênfigo , Rituximab , Linfócitos T Reguladores , Pênfigo/imunologia , Pênfigo/tratamento farmacológico , Pênfigo/sangue , Humanos , Desmogleína 3/imunologia , Linfócitos T Reguladores/imunologia , Linfócitos T Reguladores/efeitos dos fármacos , Rituximab/uso terapêutico , Rituximab/farmacologia , Masculino , Feminino , Pessoa de Meia-Idade , Autoanticorpos/imunologia , Autoanticorpos/sangue , Resultado do Tratamento , Adulto , Idoso , Fatores Imunológicos , Linfócitos T Auxiliares-Indutores/imunologia , Linfócitos T Auxiliares-Indutores/efeitos dos fármacos , Cadeias HLA-DRB1/genética , Cadeias HLA-DRB1/imunologia , Ativação Linfocitária/efeitos dos fármacos , Ativação Linfocitária/imunologiaAssuntos
Fatores Imunológicos , Pênfigo , Rituximab , Pênfigo/tratamento farmacológico , Pênfigo/patologia , Humanos , Rituximab/administração & dosagem , Rituximab/uso terapêutico , Resultado do Tratamento , Fatores Imunológicos/administração & dosagem , Fatores Imunológicos/uso terapêutico , Feminino , Masculino , Pessoa de Meia-Idade , AdultoRESUMO
Rituximab is a monoclonal antibody that targets CD20 antigen in B cells. For pemphigus, rituximab has been highly effective in steroid-sparing therapy for moderate to severe cases. Originator rituximab has demonstrated favorable treatment effects in patients with pemphigus, but its high cost remains a challenge. Biosimilar rituximab is expected to offer a potential solution. However, it is required for the comparative study of efficacy and safety between biosimilar and originator because all biosimilars may not be identical to the originator. In this study, we compared the treatment effects and safety of biosimilar (Truxima) and originator (MabThera) rituximab in patients with pemphigus. A final cohort of 52 patients in the MabThera group and 72 patients in the Truxima group was enrolled. Except for the intravenous immunoglobulin administration rate, there were no differences in baseline characteristics between the two groups, and for the purpose of comparing efficacy, investigations into time to complete remission, total steroid intake to complete remission, and total steroid intake for 6 months following rituximab treatment revealed no significant differences between the two groups. Truxima can be considered a relatively affordable alternative treatment option for pemphigus, offering cost-effectiveness to patients who are indicated for the treatment with MabThera.
Assuntos
Medicamentos Biossimilares , Pênfigo , Rituximab , Humanos , Rituximab/efeitos adversos , Rituximab/uso terapêutico , Rituximab/administração & dosagem , Pênfigo/tratamento farmacológico , Pênfigo/imunologia , Medicamentos Biossimilares/efeitos adversos , Medicamentos Biossimilares/uso terapêutico , Medicamentos Biossimilares/administração & dosagem , Medicamentos Biossimilares/economia , Masculino , Feminino , Pessoa de Meia-Idade , Adulto , Resultado do Tratamento , Idoso , Fatores Imunológicos/uso terapêutico , Fatores Imunológicos/efeitos adversos , Fatores Imunológicos/administração & dosagem , Indução de Remissão/métodos , Estudos RetrospectivosAssuntos
Linfócitos B , Depleção Linfocítica , Pênfigo , Rituximab , Humanos , Pênfigo/imunologia , Pênfigo/tratamento farmacológico , Pênfigo/diagnóstico , Rituximab/administração & dosagem , Rituximab/efeitos adversos , Linfócitos B/imunologia , Linfócitos B/efeitos dos fármacos , Fatores Imunológicos/administração & dosagem , Fatores Imunológicos/efeitos adversos , Feminino , Masculino , Pessoa de Meia-IdadeAssuntos
Pênfigo , Talidomida , Humanos , Talidomida/análogos & derivados , Talidomida/uso terapêutico , Pênfigo/tratamento farmacológico , Feminino , Masculino , Pessoa de Meia-Idade , Anti-Inflamatórios não Esteroides/uso terapêutico , Índice de Gravidade de Doença , Idoso , Adulto , Inibidores da Fosfodiesterase 4/uso terapêuticoAssuntos
Tirosina Quinase da Agamaglobulinemia , Pênfigo , Inibidores de Proteínas Quinases , Pênfigo/tratamento farmacológico , Humanos , Tirosina Quinase da Agamaglobulinemia/antagonistas & inibidores , Inibidores de Proteínas Quinases/uso terapêutico , Inibidores de Proteínas Quinases/farmacologia , Proteínas Tirosina Quinases/antagonistas & inibidores , Proteínas Tirosina Quinases/metabolismoRESUMO
Despite an increase in global research on the subject of Pemphigus, which seriously affects patient health and quality of life, there is no bibliometric research on this subject in literature to date. The aim of this study was to conduct a holistic analysis of scientific articles published on Pemphigus, using bibliometric methods. Articles published on the subject of Pemphigus between 1980 and 2021 were downloaded from the web of science (WoS) database and analyzed using various statistical methods. To determine trend subjects, collaboration between countries, and the most effective studies with citation analyses, visual network maps were obtained with bibliometric analyses. A total of 3034 articles were analyzed. The 3 countries making the greatest contribution to literature were the USA (n:831, 27.3%), Japan (n:402, 13.2%), and Germany (n:221, 7.2%). The 3 most active institutions were Keio University (n:163, 5.3%), Kurume University (n:130, 4.2%) and Tel Aviv University (n:107, 3.5%). The 3 journals publishing the most articles were the British Journal of Dermatology (n: 88), Journal of the American Academy of Dermatology (n:171) and the Journal of Investigative Dermatology (n:143). The 3 leading journals according to the mean number of citations (NC) per article (citation count: CC) were the New England Journal of Medicine (CC:246), the Lancet (CC:143) and the Journal of Cell Biology (CC:133). The author with the most articles published was Hashimoto Takashi (n.168, 5.5%). As a result of cluster analysis, it was seen that 9 different main clusters had been studied on Pemphigus subjects to date (1: desmoglein, 2: paraneoplastic Pemphigus (PNP) - Pemphigus types-desmosome, 3: desmoglein 1 ve 3-autoimmunity, 4: treatment-rituximab, 5: acantholysis-apoptosis, 6: quality of life-remission-relapse, 7: autoantibodies, 8: epidemiology-mortality, 9: corticosteroids). The most commonly studied subjects were determined to be pemphigus vulgaris (PV), pemphigus foliaceus (PF), autoimmunity, rituximab, PNP, desmoglein (desmoglein3-desmoglein1), autoantibodies, acantholysis, autoantibody, treatment, autoimmune disease, desmosome, ELISA, and immunofluorescence. The primary trending topic was rituximab drug, which is used in the treatment of Pemphigus. The other most studied trend topics were azathioprine drug used in treatment, intravenous immunoglobulin treatment, quality of life, mortality rates, Pemphigus herpetiformis, and wound healing.
Assuntos
Bibliometria , Pênfigo , Pênfigo/tratamento farmacológico , Humanos , Publicações Periódicas como Assunto/estatística & dados numéricos , Pesquisa Biomédica/tendências , EficiênciaRESUMO
INTRODUCTION: Pemphigus, an uncommon autoimmune blistering disorder affecting the skin and mucous membranes, currently with mortality primarily attributed to adverse reactions resulting from treatment protocols. Additionally, the existing treatments exhibit a notable recurrence rate. The high incidence of relapse and the considerable adverse effects associated with treatment underscore the imperative to explore safer and more effective therapeutic approaches. Numerous potential therapeutic targets have demonstrated promising outcomes in trials or preliminary research stages. These encompass anti-CD-20 agents, anti-CD-25 agents, TNF-α inhibition, FAS Ligand Inhibition, FcRn inhibition, BAFF inhibition, Bruton's tyrosine kinase (BTK) inhibition, CAAR T Cells, JAK inhibition, mTOR inhibition, abatacept, IL-4 inhibition, IL-17 inhibition, IL-6 inhibition, polyclonal Regulatory T Cells, and autologous hematopoietic stem cell transplantation. AREAS COVERED: The most significant studies regarding the impact and efficacy of the mentioned treatments on pemphigus were meticulously curated through a comprehensive search conducted on the PubMed database. Moreover, the investigations of interest cited in these studies were also integrated. EXPERT OPINION: The efficacy and safety profiles of the other treatments under discussion do not exhibit the same level of robustness as anti-CD20 therapy, which is anticipated to endure as a critical element in pemphigus treatment well into the foreseeable future.
Assuntos
Pênfigo , Pênfigo/tratamento farmacológico , Pênfigo/terapia , Humanos , Animais , Recidiva , Terapia de Alvo MolecularAssuntos
Pênfigo , Rituximab , Humanos , Pênfigo/tratamento farmacológico , Pênfigo/diagnóstico , Rituximab/administração & dosagem , Rituximab/uso terapêutico , Rituximab/efeitos adversos , Feminino , Pessoa de Meia-Idade , Masculino , Adulto , Idoso , Fatores Imunológicos/administração & dosagem , Fatores Imunológicos/efeitos adversos , Coração/efeitos dos fármacos , Coração/fisiopatologiaRESUMO
BACKGROUND: The prevalence and outcomes of coronavirus 2019 (COVID-19) among patients using glucocorticoids and immunosuppressants remain controversial. AIM: The study aims to investigate the impact of immunosuppressants especially glucocorticoids on patients in the Autoimmune Bullous Diseases Cohort of West China Hospital (AIBDWCH) during COVID-19. METHODS: We conducted a cross-sectional survey from December 7, 2022, to February 8, 2023, using questionnaires administered either face-to-face or by phone. COVID-19 cases were classified as confirmed, probable, or suspected according to World Health Organization criteria. Patients were divided into Group A (confirmed and probable cases) and Group B (suspected and other cases). The impact of glucocorticoids and immunosuppressive agents on COVID-19 disease and progression was evaluated with logistic regression models. RESULTS: This study included 111 patients with pemphigus. Overweight patients had a reduced risk of confirmed COVID-19 (odds ratio [OR] 0.35 [95 % CI 0.13-0.97], p = 0.045). Patients treated with a medium dose of prednisone during the pandemic had a lower incidence of COVID-19 compared to those on low doses, though the difference was not statistically significant. No independent effects of age, sex, comorbidities, and therapies were observed. No significant differences were found in COVID-19 symptoms among different therapy groups. CONCLUSIONS: Treatment with immunosuppressants, particularly glucocorticoids at low-to-medium doses, did not elevate COVID-19 risk in pemphigus patients. Consistent outcomes across treatments confirm the safety of these therapies during the pandemic.
Assuntos
COVID-19 , Glucocorticoides , Imunossupressores , Pênfigo , Humanos , Pênfigo/tratamento farmacológico , Pênfigo/epidemiologia , COVID-19/epidemiologia , Masculino , Feminino , Pessoa de Meia-Idade , Imunossupressores/uso terapêutico , Imunossupressores/efeitos adversos , Estudos Transversais , Adulto , Idoso , Glucocorticoides/uso terapêutico , Glucocorticoides/efeitos adversos , China/epidemiologia , SARS-CoV-2 , Inquéritos e Questionários , Fatores de RiscoRESUMO
Paraneoplastic pemphigus is a rare, life-threatening autoimmune disease that is clinically characterized by mostly extensive and refractory mucosal erosions and polymorphous skin lesions. We report here on a 16-year-old girl with isolated oral erosions, in whom mucosal pemphigoid was initially suspected and after treatment with prednisolone and dapsone marked improvement was achieved. However, a few months later the patient developed massive respiratory insufficiency as a result of bronchiolitis obliterans, so that a lung transplant was planned. As part of the preparatory diagnostic workup, unicentric, abdominally localized Castleman's disease was diagnosed, which ultimately led to the diagnosis of paraneoplastic pemphigus as evidenced by envoplakin autoantibodies. Tumor resection and subsequent lung transplantation achieved good results with sustained mucocutaneous remission.