Micose fungoide hipocromiante na infância: avaliação de um caso clínico / Hypopigmented mycosis fungoides in childhood: evaluation of a clinical case

Micose fungoide é uma neoplasia dos linfócitos T helper que afeta a pele e geralmente não apresenta manifestações extracutâneas por muitos anos. Já a micose fungoide hipocromiante (MFH) corresponde a um subtipo daquela com melhor prognóstico e que se apresenta com lesões hipocrômicas a acrômicas, com distribuição mais comum em tronco, regiões proximais de extremidades e cintura pélvica. O diagnóstico é firmado pela apresentação clínica aliada à histopatologia, que evidencia epidermotropismo com linfócitos atípicos. A doença geralmente apresenta boa resposta ao tratamento, o qual pode ser realizado com fototerapia com raios ultravioleta A (UVA), ultravioleta B (UVB), mostarda nitrogenada tópica, carmustina tópica e irradiação cutânea com elétrons. Descreve-se o caso de uma criança com máculas hipocrômicas que surgiram inicialmente no abdome, posteriormente se estendendo para raiz de coxas e região inguinal. As lesões apresentavam descamação fina, porém sem eritema ou prurido e ausência de linfonodomegalia palpável. A princípio o caso foi conduzido como eczemátide hipocromiante, mas o paciente apresentou agravamento importante das lesões em poucos meses apesar do tratamento, sendo assim, aventada a hipótese diagnóstica de MFH. Duas biópsias de pele foram realizadas, e os exames anatomopatológico e imunohistoquímica evidenciaram certo epidermotropismo, mas sem atipia de linfócitos. Foi orientado helioterapia domiciliar, com exposição corporal de 10 minutos no período da manhã, além do uso de emolientes e cuidados gerais com a pele, com relativa boa resposta. O acompanhamento é semestral e caso haja modificação do quadro, a biópsia com estudo histopatológico e a imunohistoquímica serão realizados novamente. (AU)

Mycosis fungoides is a type of cancer of the T helper lymphocytes that affects the skin and generally has no extracutaneous manifestations for many years. Otherwise, hypopigmented mycosis fungoides corresponds to a subtype with better prognosis and presents as hypopigmented or achromatic lesions, with more widespread distribution in the trunk, proximal parts of extremities and pelvic girdle. The diagnosis is confirmed by clinical presentation and histopathology, which shows epidermotropism and atypical lymphocytes. The disease usually show good response to treatment, which is performed with phototherapy with ultraviolet A (UVA), ultraviolet B (UVB), topical nitrogen mustard, topical carmustine and cutaneous irradiation with electrons. We describe the case of a child with hypochromic taint that initially appeared in the abdomen, later extending to root thighs and groin. The lesions had fine scaling, absence of erythema or itching, without the presence of palpable lymph node enlargement. At first the case was conducted as pytiriasis alba, but the patient had significant aggravation of injuries in a few months in spite of the treatment, thus mooted diagnosis of hypopigmented mycosis fungoides. Two skin biopsies were performed, and the histological and immunohistochemical tests showed epidermotropism but no atypia of lymphocytes. It was guided home heliotherapy, with body exposure of 10 minutes in the morning, besides the use of emollients and general skin care, with a relatively good response. The follow-up is bi-annually and if there is a modification in the condition, the biopsy with histopathological study and immunohistochemistry will be performed again. (AU)

Prevalence study of dermatoses referred to the phototherapy unit at the Dermatology Service of the Clinics Hospital of Porto Alegre, RS, Brazil / Estudo de prevalência das dermatoses encaminhadas ao setor de fototerapia do ambulatório de dermatologia do Hospital de Clínicas de Porto Alegre, RS, Brasil

BACKGROUND: Phototherapy consists of exposure to ultraviolet radiation for therapeutic reasons. Radiation is already used in dermatological practice, and many studies have already proved the beneficial effect of UV light treatment for chronic inflammatory or lymphoproliferative skin diseases. The Dermatology Service of the Clinics Hospital of Porto Alegre (Hospital de Clínicas de Porto Alegre) has been using phototherapy for a long time, and no official data have been described so far. OBJECTIVES: To study the prevalence of dermatoses referred to the phototherapy unit at the Clinics Hospital of Porto Alegre and describe the total number of patients who have already been referred to this sector and their phototype. METHODS: This is a descriptive cross-sectional study. Data were collected through a review of the phototherapy patients' records (secondary data), which are available on a database of the Dermatology Service of the Clinics Hospital of Porto Alegre, from August 1997 to July 2011. RESULTS: A total of 653 records were analyzed. Phototype 3 was the most prevalent (n=313). Distribution of the prevalence of dermatoses referred to the phototherapy unit was as follows: vitiligo (279), psoriasis (255), cutaneous T-cell lymphoma/mycosis fungoides (29), graftversus-host disease (15), scleroderma (11), atopic dermatitis (10), alopecia areata (6), parapsoriasis (5), eczema (4), granuloma annulare (4), and others (35). As vitiligo and psoriasis were the two most prevalent dermatoses, they were analyzed separately, with no statistical difference in prevalence between them (P=0,177). CONCLUSIONS: Our findings are in accordance with the literature, showing that although phototherapy is still mostly indicated to treat psoriasis, it ...

FUNDAMENTOS: Fototerapia é exposição à radiação ultravioleta para uso terapêutico. O uso dessas radiações já é utilizado na prática dermatológica, e estudos já provaram o efeito benéfico do tratamento UV em doenças inflamatórias crônicas ou linfoproliferativas. O Serviço de Dermatologia do Hospital de Clínicas de Porto Alegre tem disponibilizado a fototerapia para seus pacientes há um longo tempo, sem dados oficiais descritos até então. OBJETIVO: Estudar a prevalência das dermatoses encaminhadas à fototerapia do Hospital de Clínicas de Porto Alegre e descrever o número total de pacientes já encaminhados a esse setor e seu fototipo. MÉTODOS: Estudo transversal descritivo. A coleta de dados foi feita por revisão da totalidade das fichas de cadastro dos pacientes de fototerapia (dados secundários), as quais se encontram em um banco de dados no Serviço de Dermatologia do Hospital de Clínicas de Porto Alegre, de agosto de 1997 a julho de 2011. RESULTADOS: Ao todo 653 pacientes foram analisados. O fototipo 3 foi o mais prevalente (n=313). A distribuição da prevalência das dermatoses foi: vitiligo (279), psoríase (255), linfoma cutâneo de células T/ micose fungóide (29), doença do enxerto versus hospedeiro (15), esclerodermia (11), dermatite atópica (10), alopécia areata (6), parapsoríase (5), eczema (4), granuloma anular (4) e outros (35). Visto que vitiligo e psoríase foram as dermatoses mais prevalentes, eles foram analisados separadamente. Não houve diferença estatística entre as prevalências ...

Características clínico-epidemiológicas de pacientes en fototerapia por psoriasis en el Hospital Hernán Henríquez Aravena entre los años 2007 y 2009 / Clinical-epidemiological characteristics of phototherapy psoriasis patients in Hernan Henriquez Aravena Hospital between 2007-2009

La psoriasis es un trastorno crónico común de la piel, se caracteriza por pápulas eritematosas y escamas blancas, aunque el diagnóstico diferencial de la psoriasis es amplio, en la gran mayoría de los casos sólo se necesita la anamnesis y el examen físico. En el tratamiento, la luz ultravioleta (UV) ha sido reconocida como beneficiosa a través de sus efectos antiproliferativos y anti- inflamatorios. El propósito nuestro trabajo es describir las características clínicas y epidemiológicas de esta patología en pacientes sometidos a fototerapia en el Hospital Hernán Henríquez Aravena de Temuco entre septiembre de 2007 y diciembre 2009.Se realizó un estudio retrospectivo en el que se revisaron fichas clínicas y informes de fototerapia de 60 pacientes portadores de psoriasis. El tipo clínico más común fue la psoriasis en placa con un 90 por ciento de los casos, seguida de psoriasis eritrodérmica con un 5 por ciento, el tipo de piel más común fue el Tipo 2 con un 87,7 por ciento de los casos. En cuanto a la severidad, la psoriasis moderada fue el 46,8 por ciento de los casos, con un índice de severidad y área de psoriasis (PASI) promedio del grupo estudiado de 11,61 siendo el tipo de psoriasis con mayor gravedad el tipo eritrodérmica (p=0,008). La asociación entre severidad y tipo de piel fue significativa siendo los tipos de piel 1 el que presentó mayor severidad (p=0,012). Nuestro estudio demostró algunas asociaciones significativas entre severidad y tipo de psoriasis, y con tipo de piel. Además de una frecuencia similar en ambos sexos coincidiendo con lo reportado en la literatura.

Psoriasis is a common chronic disorder of the skin which is generally characterized by red papules and white scales. Differential diagnosis of Psoriasis is wide, but it is possible to reach a proper diagnosis with the clinical history and physical examination. For the treatment Ultraviolet light (UV) has been recognized as beneficial, through both antoproliferative and antiinflamatory effects. The purpose of our work is to describe the clinical and epidemiologic features of this pathology in patients with diagnosis of psoriasis submitted for phototherapy in Hospital Hernan Henríquez Aravena of Temuco, between September 2007 and December 2009. A retrospective study was conducted in which there were review medical records and phototherapy protocols of 60 patients with diagnosis of psoriasis. The most common clinical type was the Plaque Psoriasis with 90 percent of the cases, followed by Erythrodermic Psoriasis with 5 percent. The most common skin type was type 2 with 87.7 percent of the cases. Regarding severity, moderate psoriasis presented in 46.8 percent of the cases, with an average Psoriasis area ans severity index (PASI) score of the studied group of 11.61, the type of mayor severity was the Erythrodermic psoriasis (p=0,008). The association between severity and type of skin was significant, being the patients with skin type 1 the ones that presented an increased disease severity (p = 0.012). Our study showed some significant associations between severity and type of psoriasis, and type of skin. Besides the similarity of frequence between genders coincides with previous literature reports.

Qual é o tipo de fototerapia mais comumente indicada no tratamento da psoríase?: UVB banda estreita e PUVA: comportamento da prescrição / What is the most common phototherapy prescription for psoriasis: NB-UVB or PUVA? Prescription behavior

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 por cento) foram tratados com UVB banda estreita. As razões para sua indicação foram presença de psoríase em gotas (22 por cento), presença de finas placas (15 por cento), uso de drogas fotossensibilizantes (15 por cento), idade abaixo de 20 anos (9 por cento), fototipo I (9 por cento) e doença hepática (6 por cento). Os 16 (24 por cento) restantes foram tratados com PUVA. A principal indicação dessa terapia foi gravidade da doença (15 por cento), seguida de fototipo IV (9 por cento). 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 percent) were treated with narrowband UVB. The reasons for the indication were the presence of the guttate type of psoriasis (22 percent), the presence of thin plaques (15 percent), the use of drugs that affected photosensitivity (15 percent), age less than 20 years (9 percent), skin type I (9 percent), and liver disease (6 percent). The remaining 16 (24 percent) were treated with PUVA. The main indication for this therapy was the severity of the disease (15 percent), followed by the presence of skin type IV (9 percent). 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.

Ten-year experience of phototherapy in Yonsei Medical Center

Phototherapy with PUVA or UVB has been used to treat a wide variety of diseases such as psoriasis, vitiligo, atopic dermatitis and mycosis fungoides, etc. The present study was performed to investigate the pattern of phototherapy in the phototherapy clinic of Yonsei Medical Center. One thousand six hundred ninety two patients who received PUVA or UVB phototherapy were included in this study. We analyzed the protocols for phototherapy between 1985 and 1994. The number of phototherapy per year increased sharply until 1991 and thereafter it has remained relatively constant. The most common age group at the start of phototherapy was the third decade. The most common indications for PUVA and UVB phototherapy were vitiligo and psoriasis, respectively. Most patients had received less than 50 treatments of PUVA and less than 200 J/cm2 of cumulative UVA. Most patients had received less than 50 treatments with UVB and cumulative UVB doses were variable. We had not found any malignancy in the skin. Since the maximum safe cumulative doses of UVA or UVB have not yet been established, it is difficult to decide when phototherapy should b discontinued. The data presented in this study needs to be further analyzed in correlation with photoaging and cancer development for the safe usage of phototherapy.