Consensus on the treatment of vitiligo - Brazilian Society of Dermatology

Abstract Background: Vitiligo is a muco-cutaneous, autoimmune, localized, or disseminated disease, which manifests through hypochromic or achromic macules, with loss in quality of life. The prevalence of vitiligo in Brazil was determined to be 0.54%. There is no on-label medication for its treatment. To date, no Brazilian consensus on the treatment of vitiligo had been written. Objectives: The objective of this group of Brazilian dermatologists with experience in the treatment of this disease was to reach a consensus on the clinical and surgical treatment of vitiligo, based on articles with the best scientific evidence. Methods: Seven dermatologists were invited, and each was assigned two treatment modalities to review. Each treatment (topical, systemic, and phototherapy) was reviewed by three experts. Two experts reviewed the surgical treatment. Subsequently, the coordinator compiled the different versions and drafted a text about each type of treatment. The new version was returned to all experts, who expressed their opinions and made suggestions for clarity. The final text was written by the coordinator and sent to all participants to prepare the final consensus. Results/Conclusion: The experts defined the following as standard treatments of vitiligo: the use of topical corticosteroids and calcineurin inhibitors for localized and unstable cases; corticosteroid minipulse in progressive generalized vitiligo; narrowband UVB phototherapy for extensive forms of the disease. Surgical modalities should be indicated for segmental and stable generalized vitiligo. Topical and systemic anti-JAK drugs are being tested, with promising results.

Diagnosis and underdiagnosis of comorbidities in psoriasis patients - need for a multidisciplinary approach

Abstract BACKGROUND: Psoriasis is an immune-mediated disease that manifests predominantly in the skin, although systemic involvement may also occur. Although associated comorbidities have long been recognized and despite several studies indicating psoriasis as an independent risk factor for cardiovascular events, little has been done in general medical practice regardind screening. In the United States, less than 50% of clinicians are aware of these recommendations. OBJECTIVE: To identify the prevalence of these comorbidities in 296 patients followed up at a university dermatology clinic. METHODS: Systematically investigated comorbidity frequencies were compared with general practitioners' registry frequencies. Clinical features correlated with comorbidities were also investigated. RESULTS: High prevalences of systematically investigated comorbidities such as hypertension (30%) and dyslipidemia (26.5%) were documented. Conversely, data from general practitioners' records showed that 33% of dyslipidemia cases were undiagnosed and indicated possible underdiagnosis of some comorbidities. Furthermore, an association was found between: the number of comorbidities and psoriasis duration, age and high body mass index an association was found between the number of comorbidities and psoriasis duration, age, high body mass index, waist circumference or waist-to-hip ratio. (p<0.05). CONCLUSION: Disease duration, age and high body mass index, waist circumference or waist-to-hip ratio are possible criteria for choosing which patients should be screened for comorbidities. Underdiagnosis of comorbidities by general practitioners highlights the need for a multidisciplinary approach in psoriasis management.

Multicenter study for efficacy and safety evaluation of a fixeddose combination gel with adapalen 01% and benzoyl peroxide 2 5% (Epiduo® for the treatment of acne vulgaris in Brazilian population

Abstract: BACKGROUND: The current options for the treatment of acne vulgaris present many mechanisms of action. For several times, dermatologists try topical agents combinations, looking for better results. OBJECTIVES: To evaluate the efficacy, tolerability and safety of a topical, fixed-dose combination of adapalene 0.1% and benzoyl peroxide 2.5% gel for the treatment of acne vulgaris in the Brazilian population. METHODS: This is a multicenter, open-label and interventionist study. Patients applied 1.0 g of the fixed-dose combination of adapalene 0.1% and benzoyl peroxide 2.5% gel on the face, once daily at bedtime, during 12 weeks. Lesions were counted in all of the appointments, and the degree of acne severity, overall improvement, tolerability and safety were evaluated in each visit. RESULTS: From 79 recruited patients, 73 concluded the study. There was significant, fast and progressive reduction of non-inflammatory, inflammatory and total number of lesions. At the end of the study, 75.3% of patients had a reduction of >50% in non-inflammatory lesions, 69.9% in inflammatory lesions and 78.1% in total number of lesions. Of the 73 patients, 71.2% had good to excellent response and 87.6% had satisfactory to good response. In the first week of treatment, erythema, burning, scaling and dryness of the skin were frequent complaints, but, from second week on, these signals and symptoms have reduced. CONCLUSION: The fixed-dose combination of adapalene 0.1% and benzoyl peroxide 2.5% gel is effective, safe, well tolerated and apparently improves patient compliance with the treatment.

Anti-TNFα therapy in the management of psoriasis: experience of a state referral center

BACKGROUND: Psoriasis is a chronic immune-mediated disease, characterized by increased levels of TNFα. Anti-TNFα agents have revolutionized the treatment of severe psoriasis by targeting an important molecule involved in its pathogenesis. OBJECTIVES: We report the experience of a state referral center that uses anti-TNFα agents for psoriasis. METHODS: We conducted a retrospective case series. Seventy-four out of 120 patients met the inclusion criteria. Clinical and laboratory data was analyzed using the chi-squared, Wicoxon and McNemar's tests. Associations were considered statistically significant when p-value<0.05. RESULTS: Forty-one subjects (55.40%) were male, with a mean age of 47.69±14.99 years. Median disease duration and pre-treatment PASI were 14.0 months (IQR 9.0-20.0), and 13.55 points (IQR 8.5-20.32). Sixty patients (81.10%) had arthropathic psoriasis. Forty-six subjects (62.20%) had comorbidities; the most frequent was dyslipidemia (25.70%). In 55.40% of patients, insufficient response to conventional therapies was the principal indication for using anti-TNFα drugs. Clinical improvement occurred in 93.20% of cases, and the post-treatment PASI median was 0.0 points (IQR 0.0-0.0). Adverse effects occurred in 6.80% of patients. Infections and elevation of transaminases occurred in 28.40% and 8.10% of cases, respectively. CONCLUSION: Post-treatment reduction in PASI was satisfactory and the occurrence of adverse effects was minor, mostly mild infusion effects and local reactions at drug administration sites. .

Inherited epidermolysis bullosa: clinical and therapeutic aspects / Epidermólise bolhosa hereditária: aspectos clínicos e terapêuticos

Inherited epidermolysis bullosa (EB) is a heterogeneous group of genetic disorders that present with skin and, in some cases, mucosal fragility, predisposing patients to the development of blisters and/or erosions after minimal trauma or friction. Children with a recurrent history of these kinds of lesions or neonates that present them in the absence of another reasonable explanation should be investigated. Diagnosis must be based on clinical and histopathological findings. To date, management of inherited EB basically consists in avoiding traumas that trigger lesions, as well as preventing infection and facilitating healing of the wounds with the systematic use of bandages.

A epidermólise bolhosa hereditária (EBH) compreende um grupo heterogêneo de desordens genéticas que têm em comum a fragilidade cutânea e, em alguns casos mucosa, predispondo ao desenvolvimento de bolhas e/ou erosões após fricção ou trauma mínimo. Crianças com história recorrente deste tipo de lesão ou neonatos que as apresentem na ausência de outra explicação plausível devem ser investigados. O diagnóstico deve se basear em achados clínicos e histopatológicos. Até o presente momento, o manejo da EBH consiste basicamente em evitar os traumas desencadeadores das lesões, bem como evitar a infecção e facilitar a cicatrização das feridas com o uso sistemático de curativos.

Psoriasis and uveitis: a literature review / Psoríase e uveíte: uma revisão da literatura

Psoriasis is a systemic, chronic, immunologically mediated disease, with significant genetic and environmental influences. It affects from 1 to 3% of the world population. Recently, the relation between psoriasis and different comorbidities, particularly metabolic syndrome, has become extremely relevant. Uveitis is characterized by a process of intraocular inflammation resulting from various causes. Considering psoriasis and uveitis as immune-mediated diseases, this study aims to evaluate the possible association of psoriasis and/or psoriatic arthritis with uveitis and its subtypes. Few studies have evaluated the association of uveitis and psoriasis without joint involvement. It seems that psoriasis without arthropathy is not a risk factor for the development of uveitis. Uveitis tends to develop more frequently in patients with arthropathy or pustular psoriasis than in patients with other forms of psoriasis. Ophthalmic examination should be performed periodically in patients with psoriasis and uveitis. If ophthalmopathy is diagnosed, the patient should receive adequate treatment with anti-inflammatory drugs or immunomodulators to prevent vision loss.

Psoríase é uma doença sistêmica, crônica, imunologicamente mediada, com importante influência genética e ambiental, que afeta 1 à 3% da população mundial. Nos últimos anos, a relação da psoríase com diferentes comorbidades, em especial a síndrome metabólica, tornou-se extremamente relevante. A uveíte é caracterizada por um processo de inflamação intra-ocular resultante de várias causas. Considerando a psoríase e a uveíte como doenças imunologicamente mediadas, o presente trabalho visa avaliar a possível associação da psoríase e/ou artrite psoriática com a uveíte e seus subtipos. Poucos são os estudos que avaliam a associação de uveíte e psoríase sem comprometimento articular. Parece que a psoríase sem artropatia não seria um fator de risco para desenvolvimento de uveíte. A uveíte tende a desenvolver mais frequentemente em pacientes com artropatia ou psoríase pustulosa que em outras formas de psoríase. Avaliação oftalmológica deve ser feita periodicamente em pacientes com psoríase, proporcionando ao paciente um diagnóstico precoce da oftalmopatia e a instituição de tratamento adequado com anti-inflamatórios não hormonais ou drogas imunomoduladoras, no intuito de evitar a perda da visão nos pacientes com psoríase e uveíte.

Psoríase eritrodérmica refratária em criança com excelente resposta ao etanercepte / Refractory erythrodermic psoriasis in a child with an excellent outcome by using etanercept

A psoríase acomete 0,12 por cento a 0,71 por cento da população infantil, sendo que a forma eritrodérmica, grave e rara, ocorre em menos de 1,5 por cento dos casos. Os antagonistas do Fator de Necrose Tumoral-α (TNFα) constituem nova classe de drogas, utilizada para tratamento da psoríase grave a moderada, refratária às terapias convencionais. O Etanercepte é uma proteína de fusão do receptor do TNF-α, aprovada pelo Food and Drug Administration para tratamento da artrite reumatoide juvenil no grupo infantil. Apresentamos um caso de criança com 7 anos de idade, com psoríase em placa desde 8 meses de vida, que evoluiu para eritrodermia refratária a ciclosporina e metotrexato, com excelente resposta ao etanercepte, sem feitos adversos.

Psoriasis affects 0.12 percent to 0.71 percent of all children. Erythrodermic psoriasis is an uncommon but serious disorder, occurring in less than 1.5 percent of cases. Tumor necrosis factor-alpha blockers (TNF-α) are a new class of drugs used to treat moderate to severe psoriasis refractory to conventional therapies. Etanercept is a TNFα receptor fusion protein, approved by the FDA for treating juvenile rheumatoid arthritis. We present the case of a 7-year-old suffering from plaque psoriasis since 8 months old which evolved into erythroderma refractory to cyclosporine and methotrexate. Patient responded excellently to etanercept, with no adverse side effects.

Queratose liquenoide crônica: relato de caso / Keratosis lichenoides chronica: case report

A queratose liquenoide crônica ou doença de Nekam é uma dermatose mucocutânea rara da queratinização, com curso crônico e progressivo, que acomete geralmente indivíduos entre 20 e 40 anos. Existem, aproximadamente, 70 casos descritos na literatura. Devido à raridade desta dermatose e à ausência de tratamento efetivo, é uma doença de difícil manejo. No caso em questão, apresentamos um paciente de 42 anos com pápulas violáceas, hiperqueratósicas, algumas confluentes, com aspecto linear, rendilhado e em placas, localizadas no tronco e membros há cinco anos. Lesões aftoides na cavidade oral e úlceras rasas na genitália também faziam parte do quadro. O anatomopatológico foi bastante sugestivo de queratose liquenoide crônica. Introduziu-se tratamento com acitretina e dapsona, havendo melhora parcial do quadro.

Keratosis lichenoides chronica or Nekam's disease is a rare mucocutaneous dermatosis characterized by keratinization. It is chronic and progressive usually affecting individuals aged 20-40 years. Around 70 cases have been reported in the literature. Due to the rarity of this condition and the lack of effective treatment, it is a difficult disease to manage. In the case described below we present a 42-yearold patient with violaceous and hyperkeratotic papules in linear, reticular or plaque form, located on the trunk and limbs for five years. Aphthous lesions in the oral cavity and shallow ulcers on the genitalia also formed part of the clinical manifestation. Pathologic examination was suggestive of keratosis lichenoides chronica. Acitretin and dapsone was introduced and the lesions partially improved.

Psoríase pustulosa da infância: relato de caso / Childhood pustular psoriasis: case report

A psoríase pustulosa generalizada é tipo raro de psoríase descrita pela primeira vez, em 1910, por Von Zumbusch. A psoríase pustulosa generalizada na criança tende a ter um curso mais benigno que no adulto, entretanto, em sua forma grave, pode por em risco a vida do paciente. Talvez, pela raridade da doença, nenhuma opção terapêutica se mostrou consistentemente eficaz e segura até o momento. Relatos isolados sugerem ser o retinoide a droga de escolha nesses casos, porém trata-se de fármaco que, na primeira infância, traz mais dificuldades no manejo. Relata-se exemplo dessa rara doença em lactente, quadro extenso e grave, com resposta satisfatória ao uso de dapsona, com boa tolerabilidade.

Generalized pustulous psoriasis is a rare type of psoriasis first described in 1910 by Von Zumbusch. This disease tends to have a more benign development in children when compared to its development in adults. However, in its serious form it may represent a risk to the patient's life. Maybe, due to being such a rare disease no therapeutic option has so far consistently proved to be both effective and safe. Isolated reports suggest that retinoid is the best choice of drug in these cases. However, it is difficult to manage this drug in early infancy. The present report describes a serious case of this rare disease in an infant with a satisfactory response and good tolerance to the use of dapsone.

Psoríase e obesidade: revisão de literatura e recomendações no manejo: [revisão] / Psoriasis and obesity: literature review and recommendations for management: [review]

Estudos recentes demonstram uma relação entre obesidade e inflamação crônica, confirmada através da associação de níveis elevados de fator de necrose tumoral alfa (TNF-±), interleucina seis (IL-6) e proteína C reativa, com aumento do índice de massa corporal (IMC). O estado inflamatório, nos indivíduos obesos, poderia contribuir para o desenvolvimento ou agravamento da psoríase. Fenômenos análogos já foram descritos, em outras doenças inflamatórias crônicas, como a artrite reumatóide e doença de Chrõn. Estudos epidemiológicos mostram uma prevalência elevada de comorbidades cardiovasculares, secundárias às alterações metabólicas, associadas à psoríase e obesidade. Permanecem ainda não elucidados alguns aspectos desta associação, como: o impacto da obesidade (nas formas clínicas da dermatose, na associação com comorbidades e na resposta ao tratamento).

Recent studies have found a relationship between obesity and chronic inflammation, confirmed by the association of high levels of tumor necrosis factor (TNF-_), interleukin six (IL-6,) and reactive C-protein with an increase in body mass index (BMI). In obese individuals, this inflammatory condition could contribute to the development or aggravation of psoriasis. Analogous phenomena have already been described in other inflammatory chronic diseases, such as rheumatoid arthritis and Crohn's disease. Epidemiological studies have identified a high prevalence of cardiovascular comorbidities, secondary to the metabolic alterations associated with psoriasis and obesity. A few aspects of this association remain unclear, such as the impact of obesity in the clinical forms of dermatoses, in the response to treatment, and its relationship with comorbidities.

Infecção cutânea rara por Acinetobacter baumannii em imunocompetente: relato de um caso / Rare cutaneous infection by Acinetobacter baumannii in an immunocompetent patient: a case report

O Acinetobacter baumanni é patógeno oportunista antigamente considerado de baixa virulência. Atualmente está envolvido em processos infecciosos que acometem pacientes imunocomprometidos,grandes queimados e pacientes em unidades de terapia intensiva que fazem uso de ventilação mecânica. Esse relato de caso chama atenção para infecção cutânea rara por essa bactéria em paciente imunocompetente.

Acinetobacter baumannii is an oportunistic pathogen that used to be considered as having low virulence; however, it is currently known to be involved in infectious processes in patients with immunosuppression, large burns and those under mechanical ventilation in intensive care units. This case report emphasizes the possibility of cutaneous infection by A. baumanni in immunocompetent patients.

Post-kala-azar dermal leishmaniasis associated with AIDS

Post-kala-azar dermal leishmaniasis (PKDL) is rarely reported in South America. In spite of the fact that there are many reports about the association of visceral leishmaniasis and AIDS, PKDL is very uncommon in HIV-positive patients, and so far only four cases have been documented in the literature. We present another case with unusual clinicopathological aspects. The patient, a 28-year-old male, from Salvador, Bahia (an endemic area) presented with clinical manifestations of visceral leishmaniasis three years after the diagnosis of AIDS. During treatment for visceral leishmaniasis he developed disseminated miliary papules. Microscopically, the skin biopsy showed a "saw-tooth" pattern with a lichenoid mononuclear infiltrate simulating lichen planus. The histopathological diagnosis was achieved through the finding of amastigotes. The authors discuss the clinicopathological aspects of this case based on a review of the specific literature

Aspectos clínico-epidemiológicos e imunológicos da leishmaniose tegumentar / Clinical and epidemiological features of cutaneous leishmaniasis and immunological

A leishmaniose tegumentar americana (LTA) continua sendo um problema de Saúde Publica no mundo. No Brasil, no período de 1985 a 2001, a leishmaniose tegumentar americana (LTA) vem apresentando coeficientes de detecção que oscilam entre 10,45 a 21,23 por 100.000 habitantes. Em 1993, um surto de LTA foi detectado no povoado rural de Canoa, município de Santo Amaro, Bahia. Um estudo observacional prospectivo foi delineado, com objetivo de determinar as taxas de freqüência e caracterizar c1inicamente a doença. Foram acompanhados 555 indivíduos, registrando-se 29 casas de LTA. A prevalência de LTA na população avaliada no período de estudo foi de 5,2 por cento (29/555). A espécie de leishmania envolvida na área foi caracterizada como Leishmania braziliensis, sendo o flebotomíneo a Lutzomya intermedia. Foram detectados cães e eqüídeos infectados por leishmania. Esses dados foram publicados no trabalho1. No segundo trabalho os aspectos clínico-epidemiológicos e imunológicos dos 104 indivíduos documentados como portadores de infecção subclínica foram comparados com os 29 indivíduos que desenvolveram leishmaniose cutânea (LC). A infecção subclínica foi definida como indivíduo sadio, durante os 4 anos de acompanhamento clínico, sem lesão ativa cutâneo ou mucosa, sem cicatriz sugestiva de doença passada e que apresentaram o teste de hipersensibilidade tardia positivo. O grupo de indivíduos com doença clinicamente evidente (n=29) era mais jovem (19,4 ± 12,8 anos), apresentava reação cutânea com enduração significativamente maior (17,6±1,4 mm) assim como maior propor<;ao de sorologia positiva (16/29; 55,2 por cento) ao ser comparado (p<0,05) ao grupo de infecção subclínica (n=104; DTH: 9,4±4,5 mm; sorologia positiva: 48/104;46,2 por cento). Dosagem de IFN-y, TNF-a, e IL-5 foram avaliados em sobrenadantes de células mononucleares do sangue periférico (PBMC) através da técnica de ELISA em vinte pacientes com LC e em vinte indivíduos com a forma subclínica. Os resultados mostraram que 0 nível de IFN-y [353 ± 594 pg/ml (0-2,075)] e de TNF-a, [19 ± 144 (0-364) pg/ml] eram mais baixos nos casas de leishmaniose subclínica, quando comparados aos dos indivíduos com LC [1,546 ± 1,100 pg/ml (0¬3,321) e 258 ± 253 pg/ml (0-904) respectivamente]. A media do nível de IL- 5 nos indivíduos com infeCl;ao subclínica (105 ± 160 pg/ml; 0-679) foi Iigeiramente maior (p>0,05) que o observado nos casos de LC (26 ± 41 pg/ml; 0-85). Os dados sugerem que os indivíduos que não desenvolvem a doença talvez tenham a habilidade de modular melhor a resposta imune, prevenindo-os contra o danG tecidual e desenvolvimento de lesão cutânea. No terceiro trabalho foram estudados os dados da resposta imune de 56 militares voluntários e sadios à vacinação com antígeno de leishmania (Leishvacin®) com uma ou duas doses. A reação de Montenegro não foi utilizada para seleção para evitar possível sensibilização. Esse estudo...

Post-kala-azar dermal leishmaniasis associated with AIDS

Post-kala-azar dermal leishmaniasis (PKDL) is rarely reported in South America. In spite of the fact that there are many reports about the association of visceral leishmaniasis and AIDS, PKDL is very uncommon in HIV-positive patients, and so far only four cases have been documented in the literature. We present another case with unusual clinicopathological aspects. The patient, a 28-year-old male, from Salvador, Bahia (an endemic area) presented with clinical manifestations of visceral leishmaniasis three years after the diagnosis of AIDS. During treatment for visceral leishmaniasis he developed disseminated miliary papules. Microscopically, the skin biopsy showed a "saw-tooth" pattern with a lichenoid mononuclear infiltrate simulating lichen planus. The histopathological diagnosis was achieved through the finding of amastigotes. The authors discuss the clinicopathological aspects of this case based on a review of the specific literature.

Hanseníase associada a granuloma elastolítico / Leprosy combined wirh elastolytic granuloma

São descritos dois casos de Hanseníase combinados com granuloma elastolítico de células gigantes. Embora uma ocorrência concomitante não possa ser excluída, uma possível relação patogenética entre as duas condições é postulada. É possível que um mecanismo imunológico desempenhe um papel no processo elastolítico, que poderia também ser causado por dano actínico na pele alterada pela Hanseníase

Cutaneous protothecosis: report of the second Brazilian case

The present report describes a case of cutaneous protothecosis caused by Prototheca wickerhamii in a non-immunocompromised female from the state of Bahia, Brazil. This is the second case described in Brazil. Dermatological examination revealed diffusely infiltrated erythematous plaques on the flexor aspect of the right arm and forearm. The authors emphasize the pathological aspects that can lead to misdiagnosis this condition. The patient was successfully treated with fluconazole

Surto de leishmaniose tegumentar americana em Canoa, Santo Amaro, Bahia, Brasil / An outbreak of American cutaneous leishmaniasis in Canoa, Santo Amaro, Bahia, Brazil

Em 1993, um surto leishmaniose tegumentar americana (LTA) foi detectado no povoado rural de Canoa, município de Santo Amaro, Bahia. Um estudo observacional prospectivo delineou-se, com objetivo de determinar as taxas de freqüência e caracterizar clinicamente a doença. Foram acompanhados 555 indivíduos, registrando-se 29 casos de LTA, 11 casos sugestivos de LTA pregressa e 529 sadios. Desses 529 sadios, 65 apresentaram reaçäo de Montenegro positiva sem qualquer evidência presente ou passada de doença. A prevalência de LTA no período de estudo foi de 5,2 por cento (29/555). A leishmania envolvida foi caracterizada como Leishmania braziliensis e o vetor, Lutzomyia intermedia. Foram detectados cäes e equídeos infectados por leishmania. O acometimento de crianças menores de 10 anos, o acometimento igual entre os sexos e um componente de agregaçäo familiar sugerem um padräo de transmissäo peri ou intradomiciliar

Leishmaniose tegumentar disseminada: relato de caso / Disseminated cutaneous leishmaniasis: case report

A leishmaniose tegumentar disseminada é forma pouco freqüente de leishmaniose tegumentar que difere das formas anérgica difusa e cutânea clássica. Caracteriza-se clinicamente pela presença de grande número de lesöes papulosas e acneiformes, raramente com ulceraçöes e com freqüente comprometimento mucoso. O teste intradérmico é positivo, os títulos sorológicos, extremamente elevados, e o exame histopatológico costuma revelar granulomas e padräo folicular. A resposta linfoproliferativa costuma ser bastante variável, o que näo justifica chamar de reaçäo de hipersensibilidade ou "leishmanide" como era previamente conhecida no Velho Mundo. Os casos descritos nas Américas estäo associados à Leishmania amazonensis e à Leishmania braziliensis. Os autores relatam um caso dessa forma de leishmaniose, surgido no povoado rural de Canoa, Santo Amaro, Bahia, durante a vigência de um surto da doença, descrevendo as características clínico-laboratoriais do mesmo e comparando os dados com a literatura