Dexamethasone-cyclophosphamide pulse therapy outcomes comparing pemphigus vulgaris and pemphigus foliaceus groups in a Brazilian cohort study

Abstract Background Dexamethasone-cyclophosphamide pulse (DCP) and dexamethasone pulse (DP) have been successfully used to treat pemphigus, but DCP/DP outcomes comparing pemphigus vulgaris (PV) and pemphigus foliaceus (PF) are scarce. Objective To compare DCP/DP outcomes in a Brazilian cohort of PV and PF patients according to demographic and clinical data. Methods Retrospective analytical cohort study, reviewing medical charts of PV and PF patients (for DCP/DP Phases I‒IV consult Pasricha et al.16‒18). Results 37 PV and 41 PF patients non responsive to usual treatments were included similarly for DCP or DP therapy. Disease duration was longer among PF before DCP/DP prescription (p < 0.001); PF required a higher number of monthly pulses to acquire remission in Phase I (median 10 and 6 pulses, respectively; p = 0.005). DCP/DP outcomes were similar in both groups: remission in 37.8% of PV and 34.1% of PF after completed DCP/DP cycles following a median of 13 months (1-56 months follow-up); failure occurred in 13.5% of PV and 14.6% of PF in Phase I; relapse in 13.5% of PV and 12.2% of PF, and dropout in 27% of PV and 24.4% of PF in Phases II to IV. Mild side effects were documented. Study limitations The severity of PV and PF disease was not assessed by score indexes. Conclusions PV and PF patients presented similar DCP/DP outcomes. DCP/DP should be initiated earlier in PF patients due to the longer duration of their disease in order to decrease the number of pulses and the duration of Phase I to acquire remission.

Bullous pemphigoid and milia: prevalence and clinical laboratory findings in a Brazilian sample

Abstract Background: Bullous pemphigoid (BP) associated with milia lesions has been increasingly reported, but its prevalence has not been reported in a Brazilian BP population yet. Objectives: To describe the occurrence and clinical-laboratorial findings of BP-milia association in a southeastern Brazilian sample. Methods: A descriptive study based on the medical charts of 102 BP patients was accomplished. Clinical and laboratory data of BP-milia patients were compiled. Total serum IgE measurements, immunoblot assays based on basement membrane zone antigens, and HLA-DQ alleles typing were performed. Results: Milia was evident in 8 (7.8%) BP patients, five males, aged between 46 and 88 years. Increased total IgE levels were determined in 7 (87.5%) of the eight patients. In five of eight patients, immunoblotting showed IgG reactivity against the BP180-NC16a domain but not against collagen VII or laminin-332; it also revealed reactivity against the BP180 C-terminal domain or LAD-1, or both in four of them. The HLA-DQB1*03:01 and HLA-DQA1*05:05 alleles were identified in three of five BP-milia patients. Moreover, three of five cases presented the HLA-DQB1*06 allelic group. Study limitations: HLA determination was performed in five patients. Conclusions: Milia formation in BP patients seems to be less uncommon than previously admitted. Laboratory data revealed increased IgE; autoantibodies against the BP180 C-terminal domain or LAD-1, or both; and the HLA-DQB1*06 allelic group, described for the BP-milia association. Careful determination of antibodies against basement membrane zone molecules and HLA characterization in different populations may provide further insights into this association. © 2022 Sociedade Brasileira de Dermatologia. Published by Elsevier España, S.L.U. This is an open access article under the CC BY license (https://creativecommons.org/licenses/by/4.0/).

Epidermal necrolysis: SCORTEN performance in AIDS and non-AIDS patients

Abstract: Background: Stevens-Johnson syndrome and toxic epidermal necrolysis are life-threatening blistering drug reactions with high incidence of ocular sequela. The term 'Epidermal Necrolysis' has been recently used to better describe the full spectrum of the disease that includes Stevens-Johnson syndrome and toxic epidermal necrolysis at opposite ends, which differ by the extent of body surface area with epidermal detachment. SCORTEN is a mortality prognosis score for 'Epidermal Necrolysis' cases that still needed validation in acquired immunodeficiency syndrome. Objective: To evaluate the SCORTEN performance in acquired immunodeficiency syndrome, and the differences in outcomes between acquired immunodeficiency syndrome and non- acquired immunodeficiency syndrome cohorts. Methods: Retrospective cohort study of AIDS and non-AIDS 'Epidermal Necrolysis' cases admitted to a Brazilian reference center from 1990-2014. Results: Five deaths (16.7%) occurred as a consequence of EN in 30 AIDS patients, and seven (17.9%) in 39 non-AIDS patients, relative risk (RR) .92 (p=1.0). SCORTEN showed great performance, with an Area Under the Receiver Operating Curve (AUC) (ROC) of 0.90 with a 95% confidence interval ranging from .81 to .99. The performance of SCORTEN was better among non- AIDS patients than AIDS patients: AUC non- acquired immunodeficiency syndrome =0.99 (CI 05% 0.96-1.00), AUC acquired immunodeficiency syndrome = 0.74 (CI 95% 0.53-0.95), p=.02. Study Limitations: Heterogeneity of cases, wide variation of systemic corticosteroid doses when used. Conclusion: SCORTEN is valid for the Brazilian population, including among those patients with acquired immunodeficiency syndrome, and, as such, its use is recommended for aiding treatment choice in this subgroup of patients.

Clinico-immunological spectrum of American tegumentary leishmaniasis and leprosy coinfection: A case series in Southeastern Brazil

Abstract INTRODUCTION: American tegumentary leishmaniasis (ATL) and leprosy share common areas of prevalence, but reports of coinfection are scarce. METHODS: We report a series of 9 ATL-leprosy cases and discuss the association. An integrative diagram to analyze the clinico-immunological features of coinfection with both diseases. RESULTS: Nine patients with leishmaniasis (5 cutaneous, 3 mucocutaneous, 1 disseminated case) exhibited concurrent infection with distinct clinical forms of leprosy. Our diagram-based analysis evidenced a divergent clinico-immunological spectrum for each disease in 8 out of 9 cases. CONCLUSIONS: The spectrum of ATL-leprosy comorbidity suggests that the host has a specific immune response against each pathogen.

Anti-phospholipid syndrome in seven leprosy patients with thrombotic events on corticosteroid and/or thalidomide regimen: insights on genetic and laboratory profiles

Abstract INTRODUCTION Corticosteroids and/or thalidomides have been associated with thromboembolism events (TBE) in multibacillary (MB) leprosy. This report aimed to determine genetic and laboratory profiles associated with leprosy and TBE. METHODS Antiphospholipid antibodies (aPL), coagulation-related exams, prothrombin and Leiden's factor V mutations, and ß2-glycoprotein-I (ß2GPI) Val247Leu polymorphism were assessed. RESULTS Six out of seven patients with leprosy were treated with prednisone and/or thalidomide during TBE and presented at least one positive aPL. All patients presented ß2GPI polymorphism, and one showed prothrombin mutation. CONCLUSIONS Corticosteroid or thalidomide adverse effects and aPL and ß2GPI polymorphisms may cause TBE in patients with MB leprosy.

Polipose múltipla familiar: análise de 44 casos tratados no Hospital das Clínicas da FMRP-USP / Familiar adenomatosis polyposis: analysis of forty-four cases from the school of medicine of Riberão preto Hospital and Clinics

Os autores apresentam análise retrospectiva de 44 pacientes com Polipose Múltipla Familiar tratados no Hospital das Clínicas da Faculdade de Medicina de Ribeirão Preto,Universidade de São Paulo, entre janeiro de 1991 a julho de 2005. Foram estudados aspectos epidemiológicos (idade, sexo), genéticos, principais sintomas, antecedentes pessoais e aspectos envolvendo tratamento cirúrgico e complicações pós-operatórias, comparando os achados com os da literatura correlata. Dos pacientes, 31 são do sexo masculino e 13 do feminino com idade média de 32 anos (14 - 60 anos). Os sintomas prevalentes foram: sangramento intestinal (62,5 por cento), alteração do hábito intestinal (60 por cento), e com menor freqüência dor abdominal (45 por cento) e emagrecimento (30 por cento). Relataram casos de polipose familiar 67,5 por cento dos pacientes e 62,5 por cento referiram parentes com antecedente de neoplasias (intestinal e extra-intestinal). Cerca de 32,5 por cento dos pacientes já apresentavam neoplasia de cólon na época do diagnóstico da polipose,com idade média de 39 anos. O tratamento cirúrgico foi realizado em 95,4 por cento dos pacientes: 35,7 por cento foram submetidos à proctocolectomia total(9 casos com bolsa ileal em J e 6 casos com ileostomia definitiva) e 59,2 por cento a colectomia total com ileorretoanastomose. Atualmente 57 por cento dos pacientes avaliados ainda estão em seguimento com reavaliações periódicas, 7 por cento faleceram e 27 por cento abandonaram o tratamento.

The authors present a retrospective analysis of forty-four patients with familiar adenomatosis polyposis treated at the School of Medicine of Ribeirão Preto Hospital and Clinics - University of São Paulo, from January 1991 to July 2005. Epidemiologic (age and gender) and genetic aspects were investigated as well as main symptoms, personal history and surgical treatment outcome. Data obtained were compared to the available literature. Our results show that 31 patients were male and 13 female, with average age of 32 years-old (14 to 60 years-old). The main symptoms were intestinal bleeding (62.5 percent) and intestinal habit alteration (60 percent). Less frequently, patients showed abdominal pain (45 percent) and loss of weight (30 percent). Familiar adenomatosis polyposis was reported by 67.5 percent of the patients and relatives with neoplasms (intestinal and extra-intestinal) were referred by 62.5 percent of the patients. By the time of the polyposis diagnosis, 32.5 percent of the patients have already been diagnosed for colon cancer, with average age of 39 years old. Surgical treatment was performed in 95.4 percent of the patients, total proctocolectomy was performed in 35.7 percent (9 cases with ileal J- pouch and 6 with permanent ileostomy) and total colectomy with ileo rectal anasthomosis in 59.2 percent. From all patients evaluated, 57 percent are still under follow up, 7 percent died and 27 percent gave up treatment.