Discoid lupus in antiphospholipid syndrome: case description and literature review.

OBJECTIVE: The aim of the study was to report about a patient with discoid lupus erythematosus (DLE) who developed antiphospholipid syndrome (APS) 12 years after DLE diagnosis and review related literature. PATIENTS AND METHODS: This is a case report of a 34-year-old woman with DLE who developed APS. A review of articles published in the PubMed/MEDLINE, LILACS, and SciELO databases from 1966 to October 2020 was conducted using the following search terms: "antiphospholipid syndrome," "antiphospholipid antibodies," and "discoid lupus erythematosus" No language limitation was applied. RESULTS: Besides the present case, 5 case reports were identified. One case-control and two cross-sectional studies on antiphospholipid antibodies with or without APS in DLE were also reviewed. These studies revealed that APS can develop even 37 years after DLE was diagnosed. The case-control study found that patients with DLE have more anticardiolipin antibodies than controls. In contrast, one cross-sectional study showed a low prevalence of antiphospholipid antibodies in their group of patients, which was similar to findings in the general population. CONCLUSIONS: This study reviewed previous articles on DLE cases associated with antiphospholipid antibodies and/or APS, adding a new case description.

Allergic contact dermatitis by ophthalmological medications in Brazil: experience of a dermatology department.

SUMMARY: Topical treatments in ophthalmologic therapy are significant for the development of allergic contact dermatitis (ACD) in the periorbital region. Preservatives, antibiotics, glucocorticoids, and beta-blocker eye drops are defined as drugs with the highest sensitizing potential. The unavailability of patch test batteries containing substances of ophthalmological use makes it difficult for this diagnosis. In the present report, we describe six patients who developed ACD induced by different agents presenting in eye drops, confirmed with the cutaneous patch tests. The ACD diagnosis due to ophthalmic medications can be challenging, since many different agents can cause it, and the sensitivity of these cutaneous tests is low. Thus, early diagnosis is essential to avoid the complications of ACD on the skin and ocular disorders related to chronic periorbital eczema.

Ungual warts: comparison of treatment with intralesional bleomycin and electroporation in terms of efficacy and safety.

BACKGROUND: Ungual warts are considered the most common benign nail tumour, and they are caused by the human papillomavirus. Despite the numerous treatments reported in the medical literature, ungual warts are considered frustrating, with high relapse rates and a potential risk of nail dystrophy. Bleomycin is a therapeutic option showing a good safety profile and high cure rates. OBJECTIVE: To evaluate the efficacy of electrochemotherapy using intralesional bleomycin for the treatment of ungual warts in comparison with intralesional bleomycin alone and describe the side-effects related to the use of both techniques. METHODS: This was a prospective, randomized, double-blind, controlled clinical trial. Forty-four 18- to 60-year-old female and male patients with ungual warts of only one finger were included. The patients were divided into two treatment groups: GA - intralesional bleomycin; and GB - electroporation and intralesional bleomycin. Following a single application, the patients were followed up for 180 days. RESULTS: The patients' mean age was 36 years for GA and 37 years for GB. Most patients were female (68%). Of 22 patients in GA completing the study, 11 (50%) achieved the cure, while 18 (85.7%) of 21 patients completing the study in GB showed cure. A significant association of patients with or without cure after the GA and GB treatments (P = 0.022) was observed. None of the patients in either group had systemic side-effects. Independent of the technique used, all the participants considered the adverse effects tolerable. CONCLUSION: The intralesional use of bleomycin associated with electroporation for the treatment of ungual warts (both periungual and subungual) showed a statistically superior cure when compared with intralesional bleomycin alone. Side-effects were more frequently observed in the electrochemotherapy with bleomycin group than in the bleomycin monotherapy group.

Skin cancer prevention campaign in childhood: survey based on 3676 children in Brazil.

BACKGROUND AND OBJECTIVES: Primary skin cancer prevention campaigns are essential and more effective among children, not only because of the importance of sun exposure effects during this period, but also because this age is when individuals are developing behaviours. The Brazilian Society of Dermatology - Regional State of Sao Paulo developed and conducted the programme named 'The Sun, Friend of Childhood', a school health education and disease prevention project for children and parents. Our objective was to evaluate the cognitive and behavioural effects of the children and parents before and after an education model-based intervention of sun protection. METHODS: We carried out a study on a school population of Social Service of Industry - Regional State of São Paulo, from the first to the fifth years of the regular course (6-10 years). Our educational project was planned to be based on two children's learning tools (comic magazine and a DVD cartoon). Questionnaires in relation to habits and knowledge in sun exposure were applied to the children (3776) before and (2748) after the intervention. A questionnaire was applied to 3663 parents regarding personal details and habits of their children. RESULTS: According to the McNemar's statistical test, all changes in the children in acquiring new knowledge about good practices for sun exposure were statistically significant. CONCLUSIONS: Educative sun exposure programmes in childhood are a relevant tool to modify the history of life for next generations, to concern the skin cancer and good health practices.

Urticarial vasculitis in the childhood with C2 hypocomplementenemia: a rare case.

We report a first case of hypocomplementemic urticarial vasculitis of C2 fraction in a child, with cutaneous manifestation only, with no reports in scientific literature.

Polymorphisms on IFNG, IL12B and IL12RB1 genes and paracoccidioidomycosis in the Brazilian population.

Paracoccidioidomycosis (PCM) is a systemic chronic mycosis, endemic in Latin America, especially Brazil, and is the eighth leading cause of death among chronic and recurrent infectious diseases. PCM infection is characterized by the presence of Th1 immune response; the acute form, by a mixed Th2/Th9, while the chronic form is characterized by Th17/Th22 profiles. The occurrence and severity of human PCM may also be associated with genetic factors such as single nucleotide polymorphisms (SNP) on cytokines encoding genes. We investigated the association between these polymorphisms and the different clinical forms of PCM. We included 156 patients with PCM (40 with the acute form, 99 with the chronic multifocal and 17 with the chronic unifocal form) and assayed their DNA samples for IFNG +874 T/A SNP by PCR-ARMS (Amplification Refractory Mutational System), IL12B +1188 A/C SNP on 3' UTR and IL12RB1 641 A/G SNP on exon 7 by PCR-RFLP (Restriction Fragment Length Polymorphism). We found similar genotypic and allelic frequencies of the investigated SNPs among the clinical forms of PCM. Considering male patients, the IL12RB1 641 AA genotype was more frequent in the chronic multifocal form while heterozygosis was in the chronic unifocal form of PCM (p=0.048). Although our data suggest that the AA genotype (IL12RB1) may be associated with the more disseminated chronic disease, more patients of the chronic unifocal PCM group need to be analyzed as well as the secretion patterns of IFN-γ combined with the IL-12Rß1 expression for a better comprehension of this association.

Lipoprotein(a) and livedoid vasculopathy: A new thrombophilic factor?

Livedoid vasculopathy is a chronic disorder characterised by recurrent reticulated purpura on lower extremities, associated with painful purpuric or necrotic macules and ulcerations. Current knowledge indicates LV to be a thrombo-occlusive vasculopathy of cutaneous blood vessels; exact pathogenesis is yet to be understood. Elevated levels of lipoprotein(a) have been found in LV patients. To date, elevated plasma levels of lipoprotein(a) are considered an independent and causal genetic risk factor for the development of cardiovascular disease, as well as a relevant factor in hypercoagulable states. Because of its structural homology with plasminogen, Lp(a) might have important anti-fibrinolytic properties. Altered endothelial function and participation in immune and autoimmune processes, such as antiphospholipid syndrome, are also potential mechanisms of Lp(a) involvement in LV pathogenesis. Lp(a) is part of the wound healing process; the possibility of Lp(a) serum elevation to reflect an acute-phase reagent in LV scenario is also considered. The objective of this review is to examine the possible association of lipoprotein(a) with LV pathogenesis, based on its effects on thrombogenesis, fibrinolysis and autoimmunity.

Drug reaction with eosinophilia and systemic symptoms/drug-induced hypersensitivity syndrome: clinical features of 27 patients.

BACKGROUND: Drug reaction with eosinophilia and systemic symptoms (DRESS) [also called drug-induced hypersensitivity syndrome (DIHS)] includes severe reactions to drugs that need to be promptly recognized by physicians. AIM: To explore heterogeneity in the clinical presentation of DRESS/DIHS at a large academic hospital in Latin America, using the criteria defined by the European Registry of Severe Cutaneous Adverse Reactions (RegiSCAR) scoring system. METHODS: A retrospective medical record review of 60 patients with diagnostic suspicion of DRESS/DIHS admitted to our hospital between July 2008 and April 2012 was performed, including demographic data, clinical features, laboratory findings and treatment. RESULTS: Of the 60 patients, 27 fulfilled the criteria for DRESS/DIHS. Maculopapular exanthema (85.1%), fever (96.2%) and hepatic involvement (85.1%) were the most common features. Anticonvulsants were the most common causal drugs (77.7%); Phenytoin was the most common individual drug (44.4%), followed by carbamazepine (29.6%). All patients were treated initially with prednisone 1 mg/kg/day. Mortality rate was 4%. CONCLUSION: The major findings of this study (to our knowledge the largest collection of data on DRESS/DIHS in Latin America) include a positive statistical association between presence of atypical lymphocytes and higher levels of alanine aminotransferase (P < 0.001) and reinforce the importance of anticonvulsants in the pathogenesis of this severe reaction.

Dermal dendrocytes FXIIIA+ phagocytizing extruded mast cell granules in drug-induced acute urticaria.

BACKGROUND: Few authors have been attempting between mast cells and dermal dendrocytes interactions on urticaria. OBJECTIVE: To describe the extruded mast cell granules and dermal dendrocytes in drug-induced acute urticaria. METHODS: Seven patients with drug-induced acute urticaria were enrolled in the study. We token skin biopsies of urticaria lesion and perilesional skin. The 14 fragments collected were processed to immunogold electron microscopy using single stains to tryptase and FXIIIa, besides double immunogold labeling with both. RESULTS: Some sections demonstrated mast cells in degranulation process, both in anaphylactic and piecemeal degranulation types. After double immunogold staining, 10 nm (FXIIIa) and 15 nm (Tryptase) gold particles were present together over the granules in mast cells indicating that tryptase and FXIIIa are each localized within the granules of these cells. Interestingly, we found a strong evidence of than the exocytosed mast cell granules contents both FXIIIa and tryptase immunolabeled are phagocytized by dermal dendrocytes. CONCLUSIONS: The current observations provide morphological evidence that the exocytosis-phagocytosis mechanisms of mast cell granules represents one pathophysiological example of mast cells-dermal dendrocytes interactions in urticaria.

Frequency of thrombophilia determinant factors in patients with livedoid vasculopathy and treatment with anticoagulant drugs--a prospective study.

BACKGROUND: Livedoid vasculopathy (LV) is a chronic idiopathic disease characterized by painful purpuric macules on lower extremities. Its exact aetiology remains uncertain, but thrombotic and microcirculatory phenomena have been implicated as possible pathogenic factors. OBJECTIVES: To assess prospectively the frequency of thrombophilia and to verify the effectiveness of anticoagulant therapy among LV patients. METHODS: Thirty-four LV patients were tested for prothrombin time, activated partial thromboplastin time, antithrombin activity, protein C and S activity, anticardiolipin antibodies, lupus anticoagulant, prothrombin gene mutation, factor V Leiden mutation, methylenetetrahydrofolate reductase mutation, plasma homocysteine and fibrinogen. Thirteen of these patients were treated with anticoagulant drugs (either warfarin or heparin). RESULTS: Of 34 patients, 18 (52%) presented laboratory abnormalities of procoagulant conditions. Positive treatment response to anticoagulant therapy was observed in 11 patients. Improvement of pain was obtained in 1-3 weeks, an average of 1.8 week. Complete healing of the lesions was observed in about 2.3 months. Remission was sustained even after treatment interruption and lasted an average 7.8 months. No severe adverse effects were noticed. CONCLUSION: The authors suggest all patients with diagnosis of LV to be investigated for thrombophilic status. Anticoagulant drugs were well tolerated and seemed to be effective in treating not only LV symptoms but also its ulcerations.