Abstract
Objective: To evaluate the comorbidities and associated conditions in patients with pemphigus vulgaris (PV) and pemphigus foliaceus (PF) treated at a dermatology referral center in northern Brazil. Methods: This is a descriptive, observational, single-center case series study carried out at a secondary care referral center for dermatological diseases. Results: Twenty-three patients diagnosed with pemphigus admitted to the service between August and October 2024 agreed to participate in this study. Of these, 18 were diagnosed with PV and 5 with PF. All diagnoses were based on clinical characteristics and confirmed by histopathological analysis of the lesions. Regarding comorbidities, osteoporosis/osteopenia, Cushing's syndrome, recurrent cutaneous candidiasis, and/or DM were observed more frequently, impairing the patients' quality of life. Conclusion: It is suggested that the information collected in this research agrees with the most recent literature, which indicates a trend of inversion of prevalence between PF and PV.
References
AMBIEL MV e ROSELINO AM. Prevalence of Metabolic Syndrome and its components in a Brazilian sample of pemphigus patients. An Bras Dermatol. 2014; 89(5): 752-6.
AYRES VMB. Estudo clínico e epidemiológico dos casos de pênfigo foliáceo endêmico e pênfigo vulgar em um centro de referência no estado de Minas Gerais, Brasil. Universidade Federal de Minas Gerais Faculdade de Medicina: Programa de Pós-Graduação em Ciências Aplicadas à Saúde do Adulto, Belo Horizonte. 2022; 1-91.
BAICAN A e CHIOREAN R, et al. of survival for patients with pemphigus vulgaris and pemphigus foliaceus: a retrospective cohort study. Orphanet J Rare Dis. 2015; 10: 48.
BARCELOS VM e VALE ECSD, et al. Epidemiological and clinical study of cases of endemic pemphigus foliaceus and pemphigus vulgaris in a reference center in the state of Minas Gerais, Brazil. An Bras Dermatol. 2024; 99(1): 43-52.
BEYZAEE AM e RAHMATPOUR ROKNI G, et al. Rituximab as the treatment of pemphigus vulgaris in the COVID-19 pandemic era: A narrative review. Dermatol Ther. 2021; 34(1): 14405.
BREDA BJ e MAGALHÃES RF, et al. Perfil epidemiológico dos pacientes do ambulatório de doenças bolhosas autoimunes do hospital de clínicas da Universidade Estadual de Campinas (UNICAMP). Revista dos Trabalhos de Iniciação Científica da UNICAMP, Campinas, SP, 2018; 26.
BYSTRYN JC e STEINMAN NM. The adjuvant therapy of pemphigus. An update. Arch Dermatol. 1996; 132(2): 203-12.
CUNHA JB. Avaliação do perfil dos pacientes portadores de pênfigo vulgar e foliáceo no serviço de dermatologia do hospital de clínicas da universidade federal do paraná. Dissertação (Especialização em Dermatologia) - Hospital de Clínicas da Universidade Federal do Paraná Setor de Ciências da Saúde, Curitiba, 2016; 2(1): 1-64.
DELVAUX C e BOHELAY G, et al. Activity of apremilast in a patient with severe pemphigus vulgaris: case report. Front Immunol. 2024; 15: 1404185.
FONSECA LAF e ALVES CAXM, et al. Pemphigus foliaceus as a differential diagnosis in vesicobullous lesions. Einstein (Sao Paulo). 2017; 15(2): 220-222.
HANS-FILHO G e AOKI V, et al. Fogo selvagem: endemic pemphigus foliaceus. An Bras Dermatol. 2018; 93(5): 638-650.
HSU DY e BRIEVA J, et al. Comorbidities and inpatient mortality for pemphigus in the U.S.A. Br J Dermatol. 2016; 174(6): 1290-8.
INGEN-HOUSZ-ORO S e VALEYRIE-ALLANORE L, et al. First-line treatment of pemphigus vulgaris with a combination of rituximab and high-potency topical corticosteroids. JAMA Dermatol. 2015; 151(2): 200-3.
KRIDIN K. Pemphigus group: overview, epidemiology, mortality, and comorbidities. Immunol Res. 2018; 66(2): 255-270.
LANGAN SM e SMEETH L, et al. Bullous pemphigoid and pemphigus vulgaris--incidence and mortality in the UK: population based cohort study. BMJ. 2008; 337(7662): 180.
MALIK AM e TUPCHONG S, et al. An Updated Review of Pemphigus Diseases. Medicina (Kaunas). 2021; 57(10): 1080.
MORAIS HGF e COSTA CSO, et al. Pemphigus vulgaris and mucous membrane pemphigoid: A systematic review of clinical manifestations, diagnosis, and treatment. J Stomatol Oral Maxillofac Surg. 2024; 125(5S2): 101960.
MUSTAFA MB e PORTER SR, et al. Oral mucosal manifestations of autoimmune skin diseases. Autoimmun Rev. 2015; 14(10): 930-51.
PARAMESWARAN A e ATTWOOD K, et al. Identification of a new disease cluster of pemphigus vulgaris with autoimmune thyroid disease, rheumatoid arthritis and type I diabetes. Br J Dermatol. 2015; 172(3): 729-38.
PORRO AM e HANS FILHO G, et al. Consensus on the treatment of autoimmune bullous dermatoses: pemphigus vulgaris and pemphigus foliaceus - Brazilian Society of Dermatology. An Bras Dermatol. 2019; 94(21): 20-32.
PORRO AM e SEQUE CA, et al. Pemphigus vulgaris. An Bras Dermatol. 2019; 94(3): 264-278.
TALLAB T e JOHARJI H, et al. The incidence of pemphigus in the southern region of Saudi Arabia. Int J Dermatol. 2001; 40(9): 570-2.
TAVAKOLPOUR S e MAHMOUDI H, et al. Sixteen-year history of rituximab therapy for 1085 pemphigus vulgaris patients: A systematic review. Int Immunopharmacol. 2018; 54: 131-138.
TAVAKOLPOUR S. Pemphigus trigger factors: special focus on pemphigus vulgaris and pemphigus foliaceus. Arch Dermatol Res. 2018; 310(2): 95-106.
TEE CT e LEE CS, et al. Characteristics and quality of life in pemphigus patients. Med J Malaysia. 2022; 77(3): 324-330.
TIMÓTEO RP e MARQUES LS, et al. Intervenção fisioterapêutica promove melhora na qualidade de vida de indivíduos com pênfigo [Physiotherapy intervention promotes better quality of life for individuals with pemphigus]. Rev Soc Bras Med Trop. 2010; 43(5): 580-3.
WIEDENMAYER N e HOGREFE K, et al. Case report: A novel high-dose intravenous immunoglobulin preparation for the treatment of severe pemphigus vulgaris failing standard therapy. J Dermatol. 2024; 51(12): 1665-1668.
