Marcelo Arnone

Generalized Pustular Psoriasis in Childhood

Abstract:  Generalized pustular psoriasis is a rare form of psoriasis consisting of a generalized eruption of sudden onset with erythema and sterile pustules. In children, generalized pustular psoriasis is even more uncommon and may present as a severe and potentially life‐threatening disorder. In this study, we present demographics, clinical aspects, treatment response, and follow‐up of seven children with generalized pustular psoriasis. Retrospective study reviewing the records of seven children with generalized pustular psoriasis including age, gender, age of onset, presence of scalp and nail involvement, family history, concomitant diseases, precipitating factors, treatment modalities, and outcome. Age of first symptoms ranged from 1 month to 11 years. All patients received systemic retinoids at one time of the follow‐up period. Other treatment modalities included immunosuppressive drugs, biologics, phototherapy, and sulfasalazine. Two patients presented with severe constitutional illness, secondary infection and septic shock, including one fatal outcome. All further cases have remained free of recurrences for a mean period of up to 3 years. In our study, generalized pustular psoriasis presented a wide clinical spectrum in children ranging from mild, asymptomatic outbreaks to more severe, life‐threatening episodes. One fatality was observed. Children generally responded well to systemic retinoids. Further studies and long‐term follow‐up periods are needed to define potential trigger factors, efficacy and safety of different treatment modalities in children with generalized pustular psoriasis.

Psoríase na infância e na adolescência

Psoriasis is a chronic, immunologically mediated, recurrent and universal inflammatory disorder. Approximately one third of adults refer onset before 16 years of age. The sooner the onset, the worse is the prognosis. In children, lesions may be physically disfiguring, leading to psychological impairment and evident loss of quality of life. Systemic therapy used in psoriasis, as well as phototherapy, has limited use in children due to accumulative effects of drugs, low acceptance, and risk of teratogenicity. In this section, we discuss the main clinical aspects of psoriasis in childhood and adolescence, differential diagnosis, therapeutic options, and prognosis.

Decrease in Mycobacterium tuberculosis specific immune responses in patients with untreated psoriasis living in a tuberculosis endemic area.

Tuberculosis has emerged as a major concern in patients with immuno-mediated diseases, including psoriasis, undergoing treatment with biologicals. However, it is not known whether the chronically activated immune system of psoriasis patients interferes with their Mycobacterium tuberculosis (Mtb)-specific immunity, especially in tuberculosis-endemic areas like Brazil. We evaluated T-cell responses to a Mtb lysate and to the recombinant Mtb proteins ESAT-6 and Ag85B of tuberculin skin test (TST) positive and TST negative patients with severe or mild/moderate, untreated psoriasis in three different assays: lymphocyte proliferation, enzyme immunoassay for interferon (IFN)-γ and interleukin (IL)-10 production by peripheral blood mononuclear cells and overnight enzyme immunospot (ELISpot) for enumerating IFN-γ-secreting cells. In our cohort, a low proportion (29%) of the severe psoriasis patients tested were TST-positive. IFN-γ and IL-10 secretion and T-cell proliferation to Mtb antigens were reduced in TST-negative but not in TST-positive patients with severe psoriasis when compared to healthy controls with the same TST status. Similarly, severe psoriasis patients had decreased cytokine secretion and proliferative response to phytohemagglutinin. However, most psoriasis patients and healthy controls showed detectable numbers of IFN-γ-secreting effector-memory T-cells in response to Mtb antigens by ELISpot. TST-negative, mild/moderate psoriasis patients had responses that were mostly intermediary between TST-negative controls and severe psoriasis patients. Thus, patients with severe psoriasis possess decreased anti-Mtb central memory T-cell responses, which may lead to false-negative results in the diagnosis of TB infection, but retain T-cell memory-effector activity against Mtb antigens. We hypothesize that the latter may confer some protection against tuberculosis reactivation.

Encephalocraniocutaneous Lipomatosis: Clinical Spectrum of Systemic Involvement

Abstract:  Encephalocraniocutaneous lipomatosis is a rare neurocutaneous syndrome with involvement of ectomesodermal tissues. Unilateral lipomatous hamartomas of the scalp and eyes are the hallmarks of this disorder. Associated findings are extremely variable, ranging from minor abnormalities to severe, debilitating disease. We report two unrelated Brazilian patients with encephalocraniocutaneous lipomatosis, one presenting with the full‐blown spectrum of cutaneous, ocular, and central nervous system manifestations, and the other with minor extracutaneous manifestations after a 5‐year follow‐up period.

Vasculite cutânea de pequenos vasos: etiologia, patogênese, classificação e critérios diagnósticos - Parte I

Vasculitis is an inflammation of vessel walls. It may range in severity from a self-limited disorder in one single organ to a life-threatening disease due to multiple-organ failure. It has many causes, although they result in only a few histological patterns of vascular inflammation. Vessels of any type in any organ can be affected, a fact that results in a broad variety of signs and symptoms. Different vasculitides with indistinguishable clinical presentations have very different prognosis and treatments. This condition presents many challenges to physicians in terms of classification, diagnosis, appropriate laboratory workup, treatment, and the need for careful follow-up. This article reviews the classification, etiology, pathology and diagnostic criteria of cutaneous vasculitis.

Encephalocraniocutaneous Lipomatosis: A New Case Report and Review of the Literature

Encephalocraniocutaneous lipomatosis is a rare neurocutaneous syndrome characterized by lipomatous hamartomas ranging in size from a few millimeters to several centimeters and affecting the head. Ocular anomalies and a variable degree of mental retardation with or without convulsions are usually observed. This disorder should be distinguished from other mosaic neurocutaneous phenotypes such as Proteus syndrome, oculocerebrocutaneous syndrome, and nevus sebaceous syndrome. We report the clinicopathologic findings of a 4‐year‐old Brazilian girl affected by this syndrome and review the literature. To our best knowledge, this is the first documented case of encephalocraniocutaneous lipomatosis occuring sporadically in South America.

Recommendations on the use of biosimilars by the Brazilian Society of Rheumatology, Brazilian Society of Dermatology, Brazilian Federation of Gastroenterology and Brazilian Study Group on Inflammatory Bowel Disease—Focus on clinical evaluation of monoclonal antibodies and fusion proteins used in the treatment of autoimmune diseases

The Brazilian Societies of Rheumatology (SBR) and Dermatology (SBD), the Brazilian Federation of Gastroenterology (FBG) and the Brazilian Study Group on Inflammatory Bowel Disease (GEDIIB) gathered a group of their respective specialists on the topic of interest to discuss the most relevant issues regarding the clinical use of biosimilar medicines in Brazil. The main aim of that meeting was to prepare a document with recommendations to guide medical specialists and to help the national regulatory and policy-making agencies as concerns the authorization for marketing biosimilars used in autoimmune diseases, such as rheumatoid arthritis, ankylosing spondylitis, psoriatic arthritis, Crohns disease, juvenile idiopathic arthritis and ulcerative colitis. In addition to considerations on the typical differences between innovator medicines and biosimilars, the specialists established a set of seven recommendations on regulatory advances related to clinical studies, indication extrapolation, nomenclature, interchangeability, automatic substitution and pharmacovigilance.

Emergence of fluoroquinolone-resistant Neisseria gonorrhoeae in São Paulo, Brazil

Continued monitoring of antimicrobial resistance patterns is essential in order for Sexually Transmitted Diseases (STD) treatment to be effective. Gonococci isolates from 65 patients in Sao Paulo were submitted to susceptibility testing, and a decreased susceptibility or resistance to ciprofloxacin was observed in 8.7% of these patients, indicating that Neisseria gonorrhoeae fluoroquinolone resistance is emerging in Brazil.

Donovanosis treated with thiamphenicol

Ten patients with donovanosis were treated with thiamphenicol for two weeks. In eight of them, included two HIV infected patients, lesions healed. The safety profile of thiamphenicol makes it a useful and cost-effective agent in the management of donovanosis. Randomized controlled trials should be conducted with these treatment options.

Perfil epidemiológico de mulheres com vaginose bacteriana, atendidas em um ambulatório de doenças sexualmente transmissíveis, em São Paulo, SP

BACKGROUND- Bacterial vaginosis is an important disease on account of its high prevalence as well as the obstetrical and gynecological complications. OBJECTIVE- To present an epidemiological profile of patients diagnosed with bacterial vaginosis seen at an outpatient clinic in the city of Sao Paulo, Brazil, described according to socio-demographic and clinical variables. METHODS- A cross-sectional descriptive study was performed by collecting data from the medical records of 658 females, seen from January, 1999 to December, 2004. Our study took into account age, ethnicity, marital status, schooling, sexual preference, number of partners and associated sexual diseases. RESULTS- The prevalence observed was 29%. Regarding the profile of women with bacterial vaginosis, the highest ratio of cases occurred in adolescents aged 10 to 19 years old (40%), black women (37.1%), widows (62.5%), women who have not graduated from high school (39.5%), heterosexual women (29.5%), women with two or more sexual partners in the last 30 days (50%) and in the last five years (35.3%). The concomitant association with other sexually transmitted diseases was found in 35% of cases. CONCLUSION- The distribution of bacterial vaginosis in patients according to age, ethnicity, number of sexual partners and associated sexual diseases was similar to that described in the literature. The observed ratio was within values of other studies (10 to 36%).