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.

Abstract: Background: Urticarias are frequent diseases, with 15% to 20% of the population presenting at least one acute episode in their lifetime. Urticaria are classified in acute ( ≤ 6 weeks) or chronic (> 6 weeks). They may be induced or spontaneous. Objectives: To verify the diagnostic and therapeutic recommendations in chronic spontaneous urticaria (CSU), according to the experience of Brazilian experts, regarding the available guidelines (international and US). Methods: A questionnaire was sent to Brazilian experts, with questions concerning diagnostic and therapeutic recommendations for CSU in adults. Results: Sixteen Brazilian experts answered the questionnaire related to diagnosis and therapy of CSU in adults and data were analyzed. Final text was written, considering the available guidelines (International and US), adapted to the medical practices in Brazil. Diagnostic work up in CSU is rarely necessary. Biopsy of skin lesion and histopathology may be indicated to rule out other diseases, such as, urticarial vasculitis. Other laboratory tests, such as complete blood count, CRP, ESR and thyroid screening. Treatment of CSU includes second-generation anti-histamines (sgAH) at licensed doses, sgAH two, three to fourfold doses (non-licensed) and omalizumab. Other drugs, such as, cyclosporine, immunomodulatory drugs and immunosuppressants may be indicated (non-licensed and with limited scientific evidence). Conclusions: Most of the Brazilian experts in this study partially agreed with the diagnostic and therapeutic recommendations of the International and US guidelines. They agreed with the use of sgAH at licensed doses. Increase in the dose to fourfold of sgAH may be suggested with restrictions, due to its non-licensed dose. Sedating anti-histamines, as suggested by the US guideline, are indicated by some of the Brazilian experts, due to its availability. Adaptations are mandatory in the treatment of CSU, due to scarce or lack of other therapeutic resources in the public health system in Brazil, such as omalizumab or cyclosporine.

Alopecia areata is a non-scarring form of alopecia that can be localized or widespread. Its etiology is unknown, but immunological factors are implicated in its pathogenesis. With the more frequent use of anti TNFα biologic drugs, some alopecia areata cases during their use have been described. We report a case of universal alopecia in a patient with rheumatoid arthritis while using adalimumab and leflunomide.

Há controvérsias se parapsoríase pode evoluir para linfoma Cutâneo de Células T. Neste trabalho avaliou-se eficácia da fototerapia no tratamento da parapsoríase e sua relação com linfoma Cutâneo de Células T. Selecionaram-se pacientes com parapsoríase tratados com PUVA ou UVB-NB. Após um-oito anos do tratamento, pacientes foram contatatos para avaliar a evolução da doença. Em 62 pacientes, a taxa de cura foi de 79,3%;17,2% apresentaram melhora de lesões. Apenas dois pacientes evoluíram para linfoma Cutâneo de Células T. A fototerapia mostrou-se eficaz no tratamento da parapsoríase, independente do tipo de fototerapia utilizada. Nos casos estudados, parapsóríase não mostrou tendência a se desenvolver em linfoma Cutâneo de Células T.

Whether parapsoriasis represents an early stage of T-cell cutaneous lymphoma is still the subject of controversy. We evaluated the efficacy of phototherapy in the treatment of parapsoriasis and its relation with TCCL. Patients diagnosed with parapsoriasis and treated with phototherapy PUVA or UVB-NB were selected. Between 1 to 8 years following treatment the evolution of their disease was evaluated. In 62 patients the cure rate was 79.3% and 17.2% showed improvement of the lesions. Only two patients developed full blown T-cell cutaneous lymphoma. Phototherapy is an excellent treatment for parapsoriasis, with high cure rates, regardless of the type of phototherapy employed. Of the 62 patients under study, parapsoriasis showed no general tendency to progress to T-cell cutaneous lymphoma.

FUNDAMENTOS: A esclerodermia é uma doença autoimune caracterizada pela esclerose progressiva do tecido conjuntivo e alterações da microcirculação. A forma cutânea é considerada uma doença autolimitada. No entanto, em alguns casos, ocorrem lesões atróficas, deformantes, que dificultam o desenvolvimento normal. Relatos da literatura apontam a fototerapia como uma modalidade terapêutica com resposta favorável nas formas cutâneas da esclerodermia. OBJETIVOS: Este trabalho teve como objetivo avaliar o tratamento da esclerodermia cutânea com fototerapia. MÉTODOS: Foram selecionados pacientes com diagnóstico de esclerodermia cutânea para o tratamento com fototerapia, os quais foram classificados de acordo com o tipo clínico e o estágio evolutivo das lesões. Utilizou-se o exame clínico e a ultrassonografia da pele como metodologia para demonstrar os resultados obtidos com o tratamento proposto. RESULTADOS: Foi observado o início da melhora clínica das lesões com média de 10 sessões de fototerapia. A palpação clínica mostrou amolecimento em todas as lesões estudadas, com redução nos escores de avaliação estabelecidos. No exame de ultrassom, a maioria das lesões avaliadas mostrou diminuição da espessura da derme, e apenas cinco mantiveram sua medida. Não se observou diferença na resposta ao tratamento de acordo com o tipo de fototerapia instituída. CONCLUSÕES: O tratamento proposto foi efetivo em todas as lesões, independentemente do tipo de fototerapia realizada. A melhora foi observada em todas as lesões tratadas e comprovada pela avaliação clínica e pelo exame de ultrassom da pele.

BACKGROUND: Scleroderma is a chronic autoimmune disease characterized by progressive connective tissue sclerosis and microcirculatory changes. Localized scleroderma is considered a limited disease. However, in some cases atrophic and deforming lesions may be observed that hinder the normal development. Literature reports indicate phototherapy as a therapeutic modality with favorable response in cutaneous forms of scleroderma. OBJECTIVES: This study had the purpose of assessing the phototherapy treatment for localized scleroderma. METHODS: Patients with localized scleroderma were selected for phototherapy treatment. They were classified according to the type of localized scleroderma and evolutive stage of the lesions. Clinical examination and skin ultrasound were used to demonstrate the results thus obtained. RESULTS: Some clinical improvement was observed after an average of 10 phototherapeutic sessions. All skin lesions were softer at clinical palpation with scores reduction upon pre and post treatment comparison. The ultrasound showed that most of the assessed lesions presented a decrease in dermal thickness, and only five maintained their previous measure. Treatment response was similar regardless of the type of phototherapeutic treatment employed. CONCLUSIONS: The proposed treatment was effective for all lesions, regardless of the phototherapeutic modality employed. The improvement was observed in all treated skin lesions and confirmed by clinical evaluation and skin ultrasound.

FUNDAMENTOS: A hipomelanose macular progressiva é uma dermatose comum em diferentes continentes. Sua causa é desconhecida e os tratamentos propostos são pouco eficazes. OBJETIVOS: Determinar aspectos epidemiológicos da hipomelanose macular progressiva em pacientes atendidos num setor de fototerapia, no período de 1997 a 2008, e avaliar a resposta terapêutica com PUVA ou UVBNB. MÉTODOS: Foram avaliados 84 pacientes com Hipomelanose Macular Progressiva. Após 16 sessões de fototerapia, a resposta terapêutica foi definida: I=inalterado, MD=melhora discreta(< 50% de repigmentação), MM=melhora moderada(50-79%), MI=melhora intensa(80-99%) e C=cura(100%). Após um tempo mínimo de três meses, pacientes com cura ou MI foram contatados por telefone para avaliar a manutenção do resultado terapêutico. RESULTADOS: Predominaram mulheres (79%) e a cor branca. A idade mínima de aparecimento da Hipomelanose Macular Progressiva foi de 13 anos e a máxima de 36 anos. Fototerapia com PUVA foi indicada em 27 pacientes e UVBNB em 57. Estatisticamente, não houve diferença significante entre o tratamento com PUVA e UVBNB (teste de Fisher P>0.05). A maioria (81%) dos pacientes obteve 50% ou mais de repigmentação e 65% tiveram cura ou MI. Entretanto, 72% apresentaram recorrência das lesões. CONCLUSÃO: A ausência de pacientes, com mais de 40 anos, sugere que a Hipomelanose Macular Progressiva seja uma doença autolimitada. Tanto PUVA como UVB NB são opções terapêuticas, porém não impedem a recidiva da doença.

BACKGROUND: Progressive macular hypomelanosis is a common dermatosis in various continents. Its cause is unknown and proposed treatments have had little effect. OBJECTIVES: To determine epidemiological aspects of progressive macular hypomelanosis in patients referred to a phototherapy clinic between 1997 and 2008 and to evaluate therapeutic response to PUVA (psoralen + UVA) photochemotherapy or narrowband UVB phototherapy. METHODS: Eighty-four patients with progressive macular hypomelanosis were evaluated. After 16 phototherapy sessions, therapeutic response was classified as: unchanged, slightly improved (<50% of repigmentation), moderately improved (50-79% of repigmentation), much improved (80-99%) or cured (100%). After a minimum of three months, patients whose response was classified as cured or much improved were contacted by telephone to evaluate the persistence of the therapeutic response. RESULTS: Most of the patients were women (79%) and white (85%). Age at onset of progressive macular hypomelanosis ranged from 13 to 36 years. PUVA was prescribed for 27 patients and narrowband UVB phototherapy for 57. No significant difference was found between the outcomes obtained with PUVA and those obtained with narrowband UVB phototherapy (Fisher's exact test; p<0.05). The majority of patients (81%) had 50% or more repigmentation, with 65% being classified as cured or much improved. Nevertheless, there was a recurrence of the lesions in 72% of patients. CONCLUSIONS: The fact that no patients were over 40 years of age suggests that progressive macular hypomelanosis is a self-limiting disease. Both PUVA and narrowband UVB are effective therapeutic options; however, they do not prevent recurrence of the disease.

FUNDAMENTOS: Formas moderada e grave de psoríase requerem fototerapia e/ou medicações sistêmicas. Tanto UVB banda estreita quanto fototerapia UVA com psoralênicos (PUVA) podem ser utilizadas no tratamento dessas formas de psoríase, sendo comprovada a efetividade de ambas as terapias. OBJETIVOS: Avaliar as indicações de dois tipos de fototerapia no tratamento da psoríase refratária à terapia tópica: UVB banda estreita e PUVA. MÉTODOS: Entre janeiro de 2006 e dezembro de 2007, os pacientes encaminhados a dois serviços de fototerapia foram incluídos neste estudo. Dados sobre os casos e tipos de prescrição foram coletados de maneira retrospectiva. RESULTADOS: Dentre os 67 pacientes estudados, 51 (76%) foram tratados com UVB banda estreita. As razões para sua indicação foram presença de psoríase em gotas (22%), presença de finas placas (15%), uso de drogas fotossensibilizantes (15%), idade abaixo de 20 anos (9%), fototipo I (9%) e doença hepática (6%). Os 16 (24%) restantes foram tratados com PUVA. A principal indicação dessa terapia foi gravidade da doença (15%), seguida de fototipo IV (9%). CONCLUSÕES: As prescrições de UVB banda estreita excederam as de PUVA devido ao menor número de contraindicações, menor possibilidade de efeitos colaterais, e ainda por ser uma opção mais prática.

BACKGROUND: Moderate and severe forms of psoriasis require phototherapy and / or systemic medications. Both UVA and UVB can be used to treat cases of moderate and severe psoriasis, and the effectiveness of both has been proven. OBJECTIVE: to access the prescription behavior relating to two types of phototherapy for treating psoriasis refractory to topical treatment: narrowband UVB (NB-UVB) or psoralen plus UVA phototherapy (PUVA). METHODS: Between January 2006 and December 2007, patients referred to two phototherapy services were included in this study. Data on the cases and on the type of prescription were collected retrospectively. RESULTS: Among the 67 studied patients, 51 (76%) were treated with narrowband UVB. The reasons for the indication were the presence of the guttate type of psoriasis (22%), the presence of thin plaques (15%), the use of drugs that affected photosensitivity (15%), age less than 20 years (9%), skin type I (9%), and liver disease (6%). The remaining 16 (24%) were treated with PUVA. The main indication for this therapy was the severity of the disease (15%), followed by the presence of skin type IV (9%). CONCLUSIONS: Prescriptions of narrowband UVB exceeded those of PUVA because of fewer contraindications and fewer possible side effects, and because it was a more practical option.

FUNDAMENTOS: A doença enxerto contra hospedeiro é um dos obstáculos ao sucesso do transplante de medula óssea, e o envolvimento cutâneo é freqüente. A fototerapia é utilizada devido à intensa atividade imunomoduladora local, sendo opção terapêutica adjuvante para as lesões cutâneas resistentes à terapia convencional. OBJETIVO: Realizar análise descritiva do tratamento da doença enxerto contra hospedeiro com fototerapia (Puva ou UVB de faixa estreita). MÉTODOS: Foram atendidos nove pacientes com manifestação cutânea da doença enxerto contra hospedeiro aguda ou crônica. Seis foram tratados com Puva, terapia de primeira escolha, e três com UVB de faixa estreita. As sessões foram realizadas três vezes por semana, e a resposta terapêutica avaliada após 12 sessões. RESULTADOS: Todos os pacientes com doença enxerto contra hospedeiro aguda mostraram melhora, com desaparecimento do eritema e do edema. Naqueles com doença crônica, observaram-se involução das lesões liquenóides e melhora da mobilidade daqueles com a forma esclerodermiforme. Dois pacientes apresentaram doença de evolução grave e foram a óbito. CONCLUSÃO: A fototerapia mostrou-se efetiva no tratamento das manifestações cutâneas da doença enxerto contra hospedeiro aguda e crônica. A Puva permite o controle da doença, podendo a UVB de faixa estreita ser opção para pacientes impossibilitados de usar medicação sistêmica.

BACKGROUND: Graft versus host disease is one of the obstacles to successful bone marrow transplantation. It often affects the skin. Phototherapy has been used because of its strong local immunomodulatory activity and it is an option for adjuvant therapy for skin lesions of graft versus host disease resistant to conventional therapy. OBJECTIVE: To make a descriptive analysis of treating graft versus host disease with phototherapy (PUVA or narrowband UVB). Methods - Nine patients with cutaneous manifestation of acute or chronic graft versus host disease were studied. The first choice therapy was PUVA, applied in six patients, and three were treated with narrowband UVB. The sessions were held three times a week and therapeutic response was evaluated after 12 sessions. RESULTS: All patients with acute graft versus host disease showed improvement, with the disappearance of erythema and edema. In those with chronic graft versus host disease, there was good response to therapy with regression of lichenoid lesions and better mobility of patients with the sclerodermoid form. Two patients had severe progression and died. CONCLUSION: Phototherapy showed to be effective in treating skin manifestations of acute and chronic graft versus host disease. PUVA allows control of the disease. The narrowband UVB is an option for patients who cannot take systemic medications.

Paquidermoperiostose é genodermatose autossômica dominante rara, que afeta pele e ossos, porém sua prevalência e incidência permanecem desconhecidas. Ocorre predominantemente em homens, que também mostram fenótipo mais grave da doença. Descrevese o caso de paciente do sexo masculino, de 60 anos.

Pachydermoperiostosis is a rare autosomal dominant genodermatosis characterized by bone and cutaneous involvement, even though its prevalence and incidence are ignored. It occurs predominantly in men, which present a more serious clinical presentation. We report a case of a 60-year-old man with this disease.

FUNDAMENTOS: A fotoquimioterapia com PUVA é indicada para tratamento da micose fungóide, empregada como monoterapia em estágios precoces ou combinada a outras drogas nos estágios mais avançados da doença. OBJETIVOS: Avaliação da resposta terapêutica à fotoquimioterapia PUVA em pacientes com micose fungóide. MÉTODOS: Entre janeiro de 1996 e novembro de 2003 avaliaram-se 17 pacientes com micose fungóide no setor de Fototerapia da Clínica Dermatológica da Santa Casa de São Paulo. A terapia com PUVA foi realizada como monoterapia nos estádios iniciais ou como coadjuvante nos estádios avançados da doença. Avaliou-se o resultado do tratamento quanto ao aspecto clínico das lesões e parâmetros histológicos após tratamento. RESULTADOS: Quatorze de 16 pacientes responderam à fotoquimioterapia. Relacionando o estadiamento da doença à resposta terapêutica obteve-se o seguinte: cinco pacientes (um em estágio IA e quatro em IB) com controle total (cura das lesões); quatro (todos IB) com melhora intensa (controle de 70-99%); dois (IIB e IVA) com melhora moderada (de 50 a 69%); três (IA, IB, IIA) com melhora discreta (menos 50%); dois (IB, IIB) inalterados (sem resposta). Um paciente teve de descontinuar o tratamento por apresentar intenso ardor. CONCLUSÃO: Houve resposta à terapia PUVA em 87% dos pacientes, com controle total ou melhora intensa da doença em 56% dos casos. Sua efetividade permitiu regressão das lesões cutâneas, principalmente nos casos precoces. A fotoquimioterapia com PUVA mostrou ser tratamento seguro e efetivo, devendo ser considerado em pacientes com micose fungóide.

BACKGROUND: PUVA photochemotherapy is indicated to treat mycosis fungoides, either as monotherapy in the earlier stages of the disease or in combination with other drugs in more advanced stages of evolution. OBJECTIVES: To evaluate PUVA photochemotherapy response in patients with mycosis fungoides. METHODS: From January 1996 to November 2003, 17 patients with a diagnosis of mycosis fungoides were seen in the Dermatological Phototherapy Division of Santa Casa de Sao Paulo, Brazil. PUVA treatment was carried out as monotherapy at early stages of evolution and in combination with other treatments in more advanced cases of mycosis fungoides. The treatment response was evaluated regarding cutaneous clinical and histological improvement. RESULTS: Fourteen of 16 patients improved after PUVA. The rate of improvement in skin after treatment related to the initial stage of disease presented as follows: five patients (one in stage IA and four in IB) had total control (cure of lesions); four (all IB) had major regression (improvement of 70%-99%); two (IIB and IVA) had moderated improvement (50%-69%); three (IA, IB, IIA) had mild regression (less than 50%); two (IB, IIB) were unaltered. Only one patient had to discontinue treatment due to intense burning. CONCLUSION: Eighty-seven percent patients responded to PUVA therapy, and 56% presented total control or significant improvement of lesions. The effectiveness of treatment resulted in regression of lesions mainly in early stage cases. PUVA photochemotherapy was a successful and safe treatment, making it a good choice for patients with mycosis fungoides.

Fototerapia é utilizada para tratar uma grande variedade de dermatoses. Desde o século passado a fototerapia tem sido utilizada em várias modalidades, com irradiação UVA ou UVB. Está indicada para todos os tipo de dermatoses inflamatórias e com período crônico de evolução, como vitiligo, pasoríase, parapsoríase, linfomas cutâneos de células T, eczemas crônicos, demonstrando bons resultados terapêuticos. Pode ser utilizada como monoterapia ou associada a outras drogas, como retinóides, metotrexate, ciclosporina, com objetivo de diminuir o tempo de tratamento e as doses das medicações mencionadas. Como os demais tipos de tratamento, a fototerapia apresenta algumas limitações, como a necessidade de equipamentos específicos, a adesão do paciente, a possibilidade de indicação ao paciente e a dose cumulativa de irradiação UV. A fototerapia deve ser conduzida com seguimento criterioso para a obtenção de resposta efetiva com poucos efeitos colaterais.

Phototherapy has been used to treat a large variety of dermatoses since the past century. It is classified according to the type of irradiation (UVA or UVB). Phototherapy is indicated for all types of inflammatory and chronic skin diseases, such as vitiligo, psoriasis, parapsoriasis, cutaneous T-cell lymphomas and chronic eczemas, with good therapeutic results. It can be used as monotherapy or associated with others drugs, such as retinoids, methotrexate and cyclosporine, aiming to reduce length of treatment and doses. Like other treatments, phototherapy has some limitations - it requires specific equipment, patient's compliance, has restricted indications and leads to cumulative UV doses. The therapy must be performed with strict follow-up to obtain effective therapeutic response and few adverse effects.