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1.
J Dermatolog Treat ; 35(1): 2329784, 2024 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-38508226

RESUMO

BACKGROUND: There is a lack of real-life safety data on treatment options for chronic urticaria in the presence of comedication and comorbidities. METHODS: We present a single-center UCARE pilot study of 212 outpatients with chronic urticaria. Patients were divided into three groups according to different CU therapies according to international guidelines. RESULTS: Of 212 patients, 108 (mean age 48.9 years, 71.3% female) had 59 comorbidities, including cardiovascular, autoimmune and malignant diseases. Patients were followed for a mean of 24.6 months (SD ± 21.3). Urticaria therapies were divided into three groups: A: 105 (97.2%) with omalizumab and 2nd generation antihistamines), B: 16 patients (14.8%): dual therapy with antihistamines and cyclosporine in 10 (9.3%), montelukast in five (4. 6%), dapsone in four (3.7%), hydroxychloroquine in one patient (0.9%), C: 12 (11.1%) patients received a third drug for 4.9 months (SD ± 3.2) and one quadruple therapy (2.1 months). 10 out of 12 (83.3%) patients received montelukast, two (16.7%) cyclosporine, two (16.7%) dapsone and one (8.3%) hydroxychloroquine as a third drug for chronic urticaria. CONCLUSIONS: Combining treatment modalities for chronic urticaria and comorbidities are available and feasible with a good safety profile.


Assuntos
Acetatos , Antialérgicos , Urticária Crônica , Ciclopropanos , Quinolinas , Sulfetos , Urticária , Humanos , Feminino , Pessoa de Meia-Idade , Masculino , Hidroxicloroquina/uso terapêutico , Projetos Piloto , Doença Crônica , Urticária Crônica/tratamento farmacológico , Urticária/tratamento farmacológico , Omalizumab/uso terapêutico , Antagonistas dos Receptores Histamínicos H1/uso terapêutico , Ciclosporina/uso terapêutico , Dapsona/uso terapêutico , Antialérgicos/uso terapêutico
3.
Sao Paulo Med J ; 142(4): e2023151, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38422241

RESUMO

BACKGROUND: Loxosceles spp are arthropods found worldwide. Its bite may produce cutaneous loxoscelism (necrotic or edematous) or cutaneous-visceral loxoscelism. Depending on their severity and location, cutaneous forms are managed with local cold application and systemic administration of antihistamines, corticosteroids, antibiotics, polymorphonuclear inhibitors, and analgesics. OBJECTIVE: This study aimed to report a case of cutaneous loxoscelism and to identify the main dermatological manifestations associated with the Loxosceles spp bite. DESIGN AND SETTING: This case report and literature review was conducted in a Mexican university. METHODS: A detailed report on the medical management of a patient with cutaneous loxoscelism treated at the emergency department of a public hospital was published. Scopus, PubMed, Web of Science, and Google Scholar databases were searched to identify articles reporting cutaneous loxoscelism. The following keywords were used during the database search: "loxoscelism" OR "spider bite," OR "loxosceles" OR "loxosceles species" OR "loxosceles venom" OR "loxoscelism case report" AND "cutaneous" OR "dermonecrotic arachnidism." RESULTS: A 62-year-old female patient with cutaneous loxoscelism was treated with systemic dapsone and local heparin spray. Eighteen studies with 22 clinical cases were included in this systematic review. Of the 22 patients, 12 (54.5%) were men. L. rufescens was the predominant spider species. CONCLUSIONS: The administration of dapsone and heparin for the management of cutaneous loxoscelism demonstrated success in this case, with no sequelae observed. In general, the literature review indicated favorable outcomes in patients treated with antimicrobials and corticosteroids, with continuous healing of skin lesions. SYSTEMATIC REVIEW REGISTRATION: PROSPERO ID CRD42023422424 (https://www.crd.york.ac.uk/prospero/display_record.php?ID=CRD42023422424).


Assuntos
Dapsona , Picaduras de Aranhas , Feminino , Masculino , Humanos , Pessoa de Meia-Idade , Dapsona/uso terapêutico , Picaduras de Aranhas/tratamento farmacológico , Hemoglobinas , Heparina , Corticosteroides , Regeneração
6.
Am J Case Rep ; 25: e942048, 2024 Feb 14.
Artigo em Inglês | MEDLINE | ID: mdl-38351602

RESUMO

BACKGROUND Leprosy, also known as Hansen's disease, is a neglected tropical disease with low prevalence in the United States. The disease's long incubation period can cause delayed presentation, and most affected individuals have a history of travel or work in leprosy-endemic regions. The immune response to Mycobacterium leprae determines the clinical characteristics of leprosy, with tuberculoid leprosy being characterized by well-defined granulomas and involvement of peripheral nerves. The recommended treatment is a combination of dapsone and rifampin for 12 months. CASE REPORT A 78-year-old man with a history of extensive travel to Africa and Asia 50 years ago, presented with a non-tender, non-pruritic, and hypopigmented skin lesion on his left knee. Biopsy results confirmed granulomatous inflammation and the presence of Mycobacterium leprae, leading to a diagnosis of tuberculoid/paucibacillary leprosy. The patient received dapsone and rifampin treatment, which resulted in symptom improvement. CONCLUSIONS The patient's long incubation period of 50 years between exposure and symptom onset is remarkable and possibly one of the longest reported for tuberculoid leprosy. It emphasizes the importance of considering leprosy in cases with an extensive travel history and long incubation periods. Our patient's case presented contradictory staining results, suggesting potential sampling variation or a rare mixed leprosy form. Based on his clinical findings, he was diagnosed with tuberculoid leprosy. Early diagnosis and treatment are crucial to prevent irreversible nerve damage and improve patient outcomes. Healthcare providers should be vigilant in acquiring a detailed travel history to facilitate early diagnosis and appropriate management of leprosy cases.


Assuntos
Hanseníase Tuberculoide , Hanseníase , Masculino , Humanos , Idoso , Hanseníase Tuberculoide/diagnóstico , Hanseníase Tuberculoide/tratamento farmacológico , Hanseníase Tuberculoide/patologia , Rifampina/uso terapêutico , Período de Incubação de Doenças Infecciosas , Hanseníase/diagnóstico , Hanseníase/tratamento farmacológico , Hanseníase/patologia , Mycobacterium leprae , Dapsona/uso terapêutico
7.
Br J Haematol ; 204(3): 1024-1028, 2024 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-38220217

RESUMO

Immune thrombocytopenia (ITP) resolves in most children within 3-12 months of diagnosis. Chronic ITP affects 10%-20% of patients, some of whom require treatment. Several second-line agents are efficacious in this group of patients. This paper describes our experience of using dapsone as a single second-line agent in children with chronic ITP. One hundred and three children with chronic ITP were seen at our centre from January 2012 to December 2016. Forty-five children met the inclusion criteria and received dapsone; 17 (37.8%) were boys; and 28 (62.2%) were girls. Early response to dapsone was seen in 37.8% of patients. The median duration of long-term follow-up was 50 months, and at least a partial response was seen in 64.4% of the patients. Dapsone offers good initial response rates and sustained remission in paediatric chronic ITP, comparable to other therapeutic agents available.


Assuntos
Púrpura Trombocitopênica Idiopática , Trombocitopenia , Masculino , Feminino , Humanos , Criança , Púrpura Trombocitopênica Idiopática/tratamento farmacológico , Contagem de Plaquetas , Dapsona/uso terapêutico , Estudos Retrospectivos , Trombocitopenia/tratamento farmacológico
8.
Acta Derm Venereol ; 104: adv11917, 2024 Jan 25.
Artigo em Inglês | MEDLINE | ID: mdl-38270257

RESUMO

Epidermolysis bullosa acquisita (EBA) rarely develops in childhood. This study retrospectively recruited paediatric patients with EBA (age ≤ 16 years), diagnosed by clinical and histopathological features and results of immunofluorescence, immunoblotting and enzyme-linked immunosorbent assay (ELISA), and reviews their clinical manifestations, histopathology, immunological features, and responses to various treatments. All 7 included patients presented with inflammatory EBA. Among them, 3 had a bullous pemphigoid-like phenotype. Pathologically, in addition to dermal-epidermal blistering, in all patients, the distribution of neutrophils was superficial perivascular or interstitial, or in the dermal papilla. Mixed neutrophils and eosinophils were detected in 2 of the 3 patients with bullous pemphigoid-like phenotypes. In addition to treatment with glucocorticoids, dapsone was administered in 4 patients, while thalidomide and sulfasalazine were administered in 1 patient. All patients responded to the these therapies. Relapse was mainly due to reduction and cessation of glucocorticoids. In conclusion, EBA in childhood may be unique, and thus distinct from its adult counterpart. Specific treatment and follow-up protocols are required for therapy of this rare autoimmune skin disease in children.


Assuntos
Doenças Autoimunes , Epidermólise Bolhosa Adquirida , Penfigoide Bolhoso , Adulto , Humanos , Criança , Adolescente , Epidermólise Bolhosa Adquirida/diagnóstico , Epidermólise Bolhosa Adquirida/tratamento farmacológico , Estudos Retrospectivos , Dapsona/uso terapêutico , Glucocorticoides/uso terapêutico
9.
J Dtsch Dermatol Ges ; 22(3): 400-421, 2024 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-38259085

RESUMO

Non-biologic immunosuppressive drugs, such as azathioprine, dapsone or methotrexate are fundamental treatment options for a wide range of autoimmune and chronic inflammatory skin diseases. Some of these drugs were initially used for malignancies (e.g., azathioprine or methotrexate) or infectious diseases (e.g., hydroxychloroquine or dapsone) but are nowadays mostly used for their immunosuppressive/immunomodulating action. Although dermatologists have years of clinical experience with these drugs, some of the mechanisms of action are not fully understood and are the subject of research. Although these drugs are commonly used, lack of experience or knowledge regarding their safety profiles and management leads to skepticism among physicians. Here, we summarize the mechanism of action and detailed management of adverse effects of the most commonly used immunosuppressive drugs for skin diseases. Furthermore, we discuss the management of these drugs during pregnancy and breastfeeding, as well as their interaction and handling during vaccination.


Assuntos
Doenças Autoimunes , Dermatopatias , Gravidez , Feminino , Humanos , Azatioprina/uso terapêutico , Metotrexato/uso terapêutico , Imunossupressores/uso terapêutico , Dapsona/uso terapêutico , Doenças Autoimunes/tratamento farmacológico
10.
Am J Trop Med Hyg ; 109(6): 1260-1265, 2023 12 06.
Artigo em Inglês | MEDLINE | ID: mdl-37931307

RESUMO

Since the introduction of multidrug therapy (MDT), various disabilities/morbidities due to leprosy have been prevented. However, there is a subset of patients in whom the skin lesions do not resolve completely or remain unchanged despite a full course of MDT, which is a great source of anxiety to the patient and their family members. Hence, we tried to ascertain the putative causes and risk factors of persistent skin lesions (PSLs) by analyzing the clinical, histopathological, bacteriological, and drug resistance patterns. This is a retrospective, cohort study wherein 35 patients who had PSLs after completion of MDT were included. The majority of the patients were 18 to 30 years of age, with males predominating. Borderline tuberculoid leprosy was the most common clinical spectrum observed (71.4%). The majority had PSLs distributed predominantly over photo-exposed sites (upper limbs > trunk > face). Eight patients (22.8%) had a history of contact with leprosy patients in their family, and six patients (17.1%) had associated comorbidities. Improvement in histopathological parameters such as a decrease in granuloma fraction was observed in 22 patients (62.8%) with PSLs after release from treatment in comparison with baseline. Four patients (11.4%) were noted to have drug resistance (three to rifampicin and one to dapsone). Thus, our study emphasizes that leprosy patients with PSLs after completion of MDT should undergo histopathological evaluation and drug resistance studies.


Assuntos
Hanseníase , Dermatopatias , Masculino , Humanos , Hansenostáticos , Estudos Retrospectivos , Quimioterapia Combinada , Estudos de Coortes , Hanseníase/complicações , Hanseníase/tratamento farmacológico , Hanseníase/epidemiologia , Dapsona/uso terapêutico , Dapsona/efeitos adversos , Dermatopatias/tratamento farmacológico
11.
Clin Oral Investig ; 27(12): 7091-7114, 2023 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-37921879

RESUMO

OBJECTIVE: The aim of this scoping review was to evaluate the efficacy and safety of the use of systemic nonsteroidal immunomodulators (SNSI) for oral lichen planus (OLP) treatment. MATERIALS AND METHODS: This review was conducted according to PRISMA-ScR guidelines and registered at PROSPERO (CRD42021243524). Consulted databases were Pubmed, Embase, Scopus, and Web of Science. The inclusion criteria was as follows: clinical trials, case series, prospective, and retrospective studies conducted with participants presenting OLP of any sex and age. RESULTS: Thirty-two studies were selected, assessing 9 different SNSI: methotrexate, dapsone, levamisole, hydroxychloroquine, thalidomide, metronidazole, azathioprine, mycophenolate mofetil, and colchicine. Methotrexate and dapsone were the drugs with the best evidence among the options included, regarding number and quality of studies. Methotrexate resulted in significant improvement in the clinical condition and remission of symptoms, ranging between 63 and 93% of cases. Dapsone presented a similar effect to the use of topical corticosteroids and tacrolimus CONCLUSION: Among SNSI therapeutic options, methotrexate, and dapsone showed promising efficacy and safety. However, large-scale randomized clinical trials are still needed. CLINICAL RELEVANCE: SNSI have been used in the treatment of recalcitrant OLP; however, so far, it is not clear which are the best options. This scoping review highlights the potential use of methotrexate and dapsone.


Assuntos
Líquen Plano Bucal , Humanos , Líquen Plano Bucal/tratamento farmacológico , Metotrexato/uso terapêutico , Estudos Prospectivos , Estudos Retrospectivos , Fatores Imunológicos/uso terapêutico , Adjuvantes Imunológicos , Dapsona/uso terapêutico
12.
J Glob Antimicrob Resist ; 35: 262-267, 2023 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-37852372

RESUMO

OBJECTIVES: Drug resistance in leprosy is an emerging concern, leading to treatment failures, recurrences, and potential spread of resistant Mycobacterium leprae in the community. In this study, we aimed to assess drug resistance prevalence and patterns amongst leprosy patients at a tertiary care referral hospital in India. METHODS: Mutations in drug resistance determining regions for dapsone, rifampicin, and ofloxacin of the M. leprae genome in DNA extracted from skin biopsies of 136 leprosy patients (treatment-naive = 67, with persistent skin lesions = 35, with recurrence = 34) were analysed by polymerase chain reaction followed by Sanger sequencing. Wild-type strain (Thai-53) was used as a reference strain. RESULTS: Resistance mutations were identified in a total of 23 patients, constituting 16.9% of the cohort. Within this subset of 23 cases, resistance to ofloxacin was observed in 17 individuals (12.5%), while resistance to both dapsone and rifampicin was detected in three patients each (2.2% for both). The occurrence of ofloxacin resistance showed minimal disparity between recurrent and treatment-naive cases, at 17.6% and 16.4%, respectively. Dapsone resistance emerged in two treatment-naive cases and one case with persistent skin lesions. Notably, none of the treatment-naive cases or those with recurrence/relapse exhibited rifampicin resistance. Subsequently, no statistically significant correlation was identified between other clinical variables and the presence of antimicrobial resistance. CONCLUSIONS: The occurrence of resistance to the current multidrug therapy regimen (specifically dapsone and rifampicin) and to ofloxacin, a secondary antileprosy medication in M. leprae, represents a concerning scenario. This calls for an expansion towards bactericidal drug options and the establishment of robust surveillance for drug resistance in countries burdened with high leprosy rates. Moreover, the introduction of stringent antimicrobial stewardship initiatives is imperative. As a single centre study, it represents a limited, cross-sectional view of the real situation in the field.


Assuntos
Hanseníase , Mycobacterium leprae , Humanos , Mycobacterium leprae/genética , Rifampina/farmacologia , Rifampina/uso terapêutico , Hansenostáticos/farmacologia , Hansenostáticos/uso terapêutico , Ofloxacino/farmacologia , Quimioterapia Combinada , Estudos Transversais , Farmacorresistência Bacteriana/genética , Hanseníase/tratamento farmacológico , Hanseníase/epidemiologia , Dapsona/farmacologia , Dapsona/uso terapêutico , Índia/epidemiologia
13.
Cochrane Database Syst Rev ; 8: CD002292, 2023 08 11.
Artigo em Inglês | MEDLINE | ID: mdl-37572360

RESUMO

BACKGROUND: Bullous pemphigoid (BP) is the most common autoimmune blistering disease. Oral steroids are the standard treatment. We have updated this review, which was first published in 2002, because several new treatments have since been tried. OBJECTIVES: To assess the effects of treatments for bullous pemphigoid. SEARCH METHODS: We updated searches of the following databases to November 2021: Cochrane Skin Specialised Register, CENTRAL, MEDLINE, and Embase. We searched five trial databases to January 2022, and checked the reference lists of included studies for further references to relevant randomised controlled trials (RCTs). SELECTION CRITERIA: RCTs of treatments for immunofluorescence-confirmed bullous pemphigoid. DATA COLLECTION AND ANALYSIS: At least two review authors, working independently, evaluated the studies against the review's inclusion criteria and extracted data from included studies. Using GRADE methodology, we assessed the certainty of the evidence for each outcome in each comparison. Our primary outcomes were healing of skin lesions and mortality. MAIN RESULTS: We identified 14 RCTs (1442 participants). The main treatment modalities assessed were oral steroids, topical steroids, and the oral anti-inflammatory antibiotic doxycycline. Most studies reported mortality but adverse events and quality of life were not well reported. We decided to look at the primary outcomes 'disease control' and 'mortality'. Almost all studies investigated different comparisons; two studies were placebo-controlled. The results are therefore based on a single study for each comparison except azathioprine. Most studies involved only small numbers of participants. We assessed the risk of bias for all key outcomes as having 'some concerns' or high risk, due to missing data, inappropriate analysis, or insufficient information. Clobetasol propionate cream versus oral prednisone Compared to oral prednisone, clobetasol propionate cream applied over the whole body probably increases skin healing at day 21 (risk ratio (RR 1.08, 95% confidence interval (CI) 1.03 to 1.13; 1 study, 341 participants; moderate-certainty evidence). Skin healing at 21 days was seen in 99.8% of participants assigned to clobetasol and 92.4% of participants assigned to prednisone. Clobetasol propionate cream applied over the whole body compared to oral prednisone may reduce mortality at one year (RR 0.73, 95% CI 0.53 to 1.01; 1 study, 341 participants; low-certainty evidence). Death occurred in 26.5% (45/170) of participants assigned to clobetasol and 36.3% (62/171) of participants assigned to oral prednisone. This study did not measure quality of life. Clobetasol propionate cream may reduce risk of severe complications by day 21 compared with oral prednisone (RR 0.65, 95% CI 0.50 to 0.86; 1 study, 341 participants; low-certainty evidence). Mild clobetasol propionate cream regimen (10 to 30 g/day) versus standard clobetasol propionate cream regimen (40 g/day) A mild regimen of topical clobetasol propionate applied over the whole body compared to the standard regimen probably does not change skin healing at day 21 (RR 1.00, 95% CI 0.97 to 1.03; 1 study, 312 participants; moderate-certainty evidence). Both groups showed complete healing of lesions at day 21 in 98% participants. A mild regimen of topical clobetasol propionate applied over the whole body compared to the standard regimen may not change mortality at one year (RR 1.00, 95% CI 0.75 to 1.32; 1 study, 312 participants; low-certainty evidence), which occurred in 118/312 (37.9%) participants. This study did not measure quality of life. A mild regimen of topical clobetasol propionate applied over the whole body compared to the standard regimen may not change adverse events at one year (RR 0.94, 95% CI 0.78 to 1.14; 1 study, 309 participants; low-certainty evidence). Doxycycline versus prednisolone Compared to prednisolone (0.5 mg/kg/day), doxycycline (200 mg/day) induces less skin healing at six weeks (RR 0.81, 95% CI 0.72 to 0.92; 1 study, 213 participants; high-certainty evidence). Complete skin healing was reported in 73.8% of participants assigned to doxycycline and 91.1% assigned to prednisolone. Doxycycline compared to prednisolone probably decreases mortality at one year (RR 0.25, 95% CI 0.07 to 0.89; number needed to treat for an additional beneficial outcome (NNTB) = 14; 1 study, 234 participants; moderate-certainty evidence). Mortality occurred in 2.4% (3/132) of participants with doxycycline and 9.7% (11/121) with prednisolone. Compared to prednisolone, doxycycline improved quality of life at one year (mean difference 1.8 points lower, which is more favourable on the Dermatology Life Quality Index, 95% CI 1.02 to 2.58 lower; 1 study, 234 participants; high-certainty evidence). Doxycycline compared to prednisolone probably reduces severe or life-threatening treatment-related adverse events at one year (RR 0.59, 95% CI 0.35 to 0.99; 1 study, 234 participants; moderate-certainty evidence). Prednisone plus azathioprine versus prednisone It is unclear whether azathioprine plus prednisone compared to prednisone alone affects skin healing or mortality because there was only very low-certainty evidence from two trials (98 participants). These studies did not measure quality of life. Adverse events were reported in a total of 20/48 (42%) participants assigned to azathioprine plus prednisone and 15/44 (34%) participants assigned to prednisone. Nicotinamide plus tetracycline versus prednisone It is unclear whether nicotinamide plus tetracycline compared to prednisone affects skin healing or mortality because there was only very low-certainty evidence from one trial (18 participants). This study did not measure quality of life. Fewer adverse events were reported in the nicotinamide group. Methylprednisolone plus azathioprine versus methylprednisolone plus dapsone It is unclear whether azathioprine plus methylprednisolone compared to dapsone plus methylprednisolone affects skin healing or mortality because there was only very low-certainty evidence from one trial (54 participants). This study did not measure quality of life. A total of 18 adverse events were reported in the azathioprine group and 13 in the dapsone group. AUTHORS' CONCLUSIONS: Clobetasol propionate cream applied over the whole body is probably similarly effective as, and may cause less mortality than, oral prednisone for treating bullous pemphigoid. Lower-dose clobetasol propionate cream applied over the whole body is probably similarly effective as standard-dose clobetasol propionate cream and has similar mortality. Doxycycline is less effective but causes less mortality than prednisolone for treating bullous pemphigoid. Other treatments need further investigation.


Assuntos
Azatioprina , Penfigoide Bolhoso , Humanos , Azatioprina/uso terapêutico , Prednisona/uso terapêutico , Clobetasol/uso terapêutico , Penfigoide Bolhoso/tratamento farmacológico , Doxiciclina/uso terapêutico , Metilprednisolona/uso terapêutico , Dapsona/uso terapêutico , Niacinamida/uso terapêutico
15.
BMJ Case Rep ; 16(8)2023 Aug 09.
Artigo em Inglês | MEDLINE | ID: mdl-37558274

RESUMO

We report a case of refractory paediatric pemphigus vulgaris with sepsis, treated successfully with intravenous immunoglobulin (IVIG) and amniotic membrane dressing. The patient was initially started on oral prednisolone (1 mg/kg/day) and dapsone 50 mg once daily. Azathioprine 50 mg orally was then used in place of dapsone due to rapid disease progression with extensive skin involvement. However, the patient developed sepsis and azathioprine had to be discontinued. Because of rapidly progressive disease and sepsis, the patient was put on IVIG at a dose of 2 g/kg in divided doses over 3 days along with amniotic membrane dressing. There was marked improvement after 2 weeks of follow-up.


Assuntos
Pênfigo , Sepse , Humanos , Criança , Pênfigo/complicações , Pênfigo/tratamento farmacológico , Imunoglobulinas Intravenosas/uso terapêutico , Azatioprina/uso terapêutico , Curativos Biológicos , Sepse/complicações , Sepse/tratamento farmacológico , Dapsona/uso terapêutico
16.
Front Immunol ; 14: 1214011, 2023.
Artigo em Inglês | MEDLINE | ID: mdl-37503352

RESUMO

Epidermolysis bullosa acquisita (EBA) is a chronic, recurrent autoimmune subepidermal bullous disease characterized by the presence of autoantibodies targeting type VII collagen -- basement membrane zone antigen. Standard therapy for EBA includes a combination of systemic corticosteroids and dapsone; however, severe cases may require advanced treatment. The current article reports on four EBA cases in which biologics: infliximab, rituximab (Rtx), and intravenous immunoglobulin (IVIG) were applied. All patients fulfilled the clinical and immunological criteria of EBA: they presented tense blisters healing with atrophic scars on the skin on traumatized areas and in mucous membranes. The diagnosis of EBA was established using numerous techniques: direct and indirect immunofluorescence, salt split skin, ELISA, Fluorescence Overlay Antigen Mapping using Laser Scanning Confocal Microscopy. Since all the patients did not achieve long-term remission on standard treatment (prednisone, dapsone) due to ineffectiveness or side effects of drugs, they eventually were treated with biologics leading to extraordinary skin improvement and stopping the disease for 1-3 years. Biologics in all patients were tolerated very well. No side effects were observed during application as well as multi-month follow-up. The presented cases provide a premise that biological drugs can be a valuable component of EBA therapy.


Assuntos
Produtos Biológicos , Epidermólise Bolhosa Adquirida , Humanos , Epidermólise Bolhosa Adquirida/diagnóstico , Epidermólise Bolhosa Adquirida/tratamento farmacológico , Vesícula , Autoanticorpos , Dapsona/uso terapêutico , Produtos Biológicos/uso terapêutico
17.
Bol Med Hosp Infant Mex ; 80(Supl 1): 77-81, 2023.
Artigo em Inglês | MEDLINE | ID: mdl-37490686

RESUMO

BACKGROUND: Acquired epidermolysis bullosa is a rare and chronic autoimmune subepidermal bullous disease characterized by the formation of autoantibodies against type VII collagen. Presentation in childhood is rare and with several manifestations. CASE REPORT: We report the case of a 12-year-old female patient who presented bullous and polymorphic lesions on the chest and extremities of several months of evolution. Due to the characteristics of the skin lesions, a histopathological and direct immunofluorescence study was conducted, confirming the diagnosis of acquired epidermolysis bullosa. Subsequently, corticosteroid and dapsone treatment was administered, with favorable clinical response during follow-up. CONCLUSIONS: Acquired epidermolysis bullosa is unusual in pediatric age, so it should be considered in the differential diagnosis of other congenital and acquired bullous diseases of childhood. The definitive diagnosis is performed through an immunofluorescence, study, which allows for rapid and effective treatment to control the disease and avoid permanent sequelae.


INTRODUCCIÓN: La epidermólisis bullosa adquirida es una enfermedad ampollar subepidérmica autoinmune, rara y crónica caracterizada por la formación de autoanticuerpos contra colágeno tipo VII. La presentación en la infancia es poco frecuente y con manifestaciones variables. CASO CLÍNICO: Se describe el caso de una paciente de sexo femenino de 12 años de edad que presentó lesiones ampollares y polimórficas en tórax y extremidades de varios meses de evolución. Por las características de las lesiones cutáneas, se realizó un estudio histopatológico y de inmunofluorescencia directa que confirmó el diagnóstico de epidermólisis bullosa adquirida, por lo que se administró tratamiento con corticoide y dapsona, con una respuesta clínica favorable durante el seguimiento. CONCLUSIONES: La epidermólisis bullosa adquirida es inusual en la edad pediátrica. Por tanto, debe considerarse en el diagnóstico diferencial de otras enfermedades ampollares congénitas y adquiridas de la infancia. El diagnóstico definitivo se realiza a través del estudio de inmunofluorescencia, lo que permite instaurar rápidamente un tratamiento rápido y eficaz para controlar la enfermedad y evitar secuelas permanentes.


Assuntos
Epidermólise Bolhosa Adquirida , Humanos , Feminino , Criança , Epidermólise Bolhosa Adquirida/diagnóstico , Epidermólise Bolhosa Adquirida/tratamento farmacológico , Epidermólise Bolhosa Adquirida/patologia , Diagnóstico Diferencial , Dapsona/uso terapêutico
18.
Cornea ; 42(5): 565-571, 2023 May 01.
Artigo em Inglês | MEDLINE | ID: mdl-37000702

RESUMO

PURPOSE: Ocular mucous membrane pemphigoid (OcMMP) is a rare and potentially blinding condition for which consensus treatment guidelines do not exist. The purpose of this study was to assess the effectiveness and safety of various immunomodulatory agents in the treatment of OcMMP in a private practice setting. METHODS: We conducted a 10-year retrospective chart review of patients managed with OcMMP (n = 22). The median age at diagnosis was 73 (range: 35-91) years, and 59% (13/22) of patients were female. Visual acuity, Foster stage, and adverse effects (AEs) were documented. Treatment outcomes for each treatment episode were qualified at 3 months as complete response (CR), response (R), or failure (F). After 3 months, CR was then further subqualified as sustained CR, reactivation after initial CR, or AE after initial CR. The Fisher exact test P values were calculated for each outcome in comparison with mycophenolate. RESULTS: Twenty patients were treated with an immunomodulatory agent for a total of 55 treatment episodes. In comparison to dapsone, mycophenolate was more likely to achieve sustained CR (50% vs. 0%, P = 0.022) and R (100% vs. 50%, P = 0.007), and less likely to fail (0% vs. 50%, P = 0.007). Dapsone was also more likely to be discontinued because of AEs than mycophenolate (40% vs. 6%, P = 0.041). CONCLUSIONS: Mycophenolate is a superior first-line agent to dapsone in the treatment of OcMMP. Although not statistically significant, mycophenolate trends toward superiority over methotrexate as well. Mycophenolate is very effective when used in combination with rituximab. Azathioprine remains a reasonable second-line agent.


Assuntos
Penfigoide Mucomembranoso Benigno , Penfigoide Bolhoso , Humanos , Feminino , Masculino , Estudos Retrospectivos , Penfigoide Bolhoso/induzido quimicamente , Imunossupressores/uso terapêutico , Penfigoide Mucomembranoso Benigno/diagnóstico , Penfigoide Mucomembranoso Benigno/tratamento farmacológico , Mucosa , Dapsona/uso terapêutico
19.
Ned Tijdschr Geneeskd ; 1672023 03 29.
Artigo em Holandês | MEDLINE | ID: mdl-36988914

RESUMO

BACKGROUND: Recurrent blisters in the face can be a sign of linear IgA disease, a rare blistering disease with cutaneous as well as mucosal involvement. CASE DESCRIPTION: A 75-year-old male patient visited our Dermatology outpatient department with complaints of recurring blisters in his face. Erosions and crusts, but no blisters, were seen during physical examination. Histopathologic examination showed subepithelial vesicles. Eventually, direct immunofluorescence showed IgA deposition at the basement membrane zone which confirmed the diagnosis of linear IgA disease. The patient was successfully treated with dapsone. Due to possible mucosal involvement, the patient was also referred to the ENT-specialist and the ophthalmologist who could not find any abnormalities. CONCLUSION: With a story of recurrent blisters in the face, one should be aware of the blister disease called linear IgA disease. Clinical presentation may be subtle. Physicians should rule out mucosal involvement as this can lead to great morbidity.


Assuntos
Dapsona , Pele , Humanos , Masculino , Idoso , Pele/patologia , Dapsona/uso terapêutico , Vesícula/diagnóstico , Vesícula/etiologia , Administração Cutânea , Imunoglobulina A
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